Pediatric Urology

Pediatric Urology: Surgical Complications and Management

Edited by Duncan T. Wilcox, Prasad P. Godbole and Martin A. Koyle

© 2008 Blackwell Publishing Ltd. ISBN: 978-1-405-16268-5

Pediatric Urology

Surgical Complications and

Management

EDITED BY

Duncan T. Wilcox mbbs md feapu

Associate Professor, Pediatric Urology

Rose Mary Haggar Professorship in Urology

University of Texas Southwestern

Dallas, TX, USA

Prasad P. Godbole frcs frcs (paed)

Consultant Paediatric Urologist

Sheffield Children's NHS Trust

Sheffield, UK

Martin A. Koyle md faap facs

Professor of Urology and Pediatrics

University of Washington;

Chief, Division of Urology

Children's Hospital and Regional Medical Center

Seattle, WA, USA

A John Wiley & Sons, Ltd., Publication

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Library of Congress Cataloguing-in-Publication Data

Pediatric urology: surgical complications and management/edited by Duncan

T. Wilcox, Prasad P. Godbole, Martin A. Koyle.

p.;cm

Includes bibliographical references and index.

ISBN:978-1-4051-6268-5 (alk.paper)

1. Genitourinary organs-Surgery-Complications. 2. Pediatric urology. I. Wilcox, Duncan T. II.

Godbole, Prasad III, Koyle, Martin A. [DNLM: 1. Urologic Diseases-surgery. 5. Infant.

6. Intraoperative Complications-prevention & control. 7. Male Urogenital Diseases-surgery.

8. Postoperative Complications-prevention & control. WS 320 P3756 2008]

RD571.P45 2008

617.4601-dc22

ISBN: 978-1-4051-6268-5 2007050631

A catalogue record for this book is available from the British Library.

Set in 9.25/11 Minion by Charon Tec Ltd (A Macmillan Company), Chennai, India

Printed and bound in Singapore by Markono Print Media Pte Ltd

1 2008

v

Contents

Contributors, viii

Foreword, xii

Preface, xiv

Part I Principles of Surgical Audit

1 How to Set Up a Prospective Surgical Audit, 3

Andrew Sinclair and Ben Bridgewater

2 Evaluating Personal Surgical Audit and What to Do If Your

Results Are Outside the "Mean", 8

Andrew Sinclair and Ben Bridgewater

3 The Implications of a Poor Surgical Outcome, 12

Robert Wheeler

Part II General Principles

4 The Metabolic and Endocrine Response to Surgery I, 21

Laura Coates and Joe I. Curry

5 The Metabolic and Endocrine Response to Surgery II: Management, 29

Benjamin P. Wisner, Douglas Ford and Martin A. Koyle

6 Perioperative Anesthetic and Analgesic Risks and Complications, 36

Philippa Evans and Mark Thomas

Part III Open Surgery of the Upper Urinary Tract

7 Nephrectomy, 47

Paul Crow and Mark Woodward

8 Partial Nephrectomy, 52

Marc-David Leclair and Yves Héloury

9 Ureteropelvic Junction Obstruction, 58

Jenny Yiee and Duncan T. Wilcox

10 Ureteral Reimplant Surgery, 67

Laurence S. Baskin and Gerald Mingin

11 Ureteroureterostomy, 73

Job K. Chacko and Martin A. Koyle

Part IV Surgery of the Bladder

12 Epispadias-Exstrophy Complex, 83

Ahmad A. Elderwy and Richard Grady

13 Umbilical and Urachal Anomalies, 92

Paul F. Austin

Part V Endoscopic Surgery of the Urinary Tract

14 Cystoscopy and Cystoscopic Interventions, 101

Divyesh Y. Desai

15 Vesicoureteric Reflux, 111

Christian Radmayr

16 Interventional Procedures, 117

Korgun Koral

17 Minimally Invasive Interventions for Stone Disease, 125

H. Serkan Dogan and Serdar Tekgül

18 General Laparoscopy, 132

Chris Kimber and Neil McMullin

19 Laparoscopy for the Upper Urinary Tract, 138

J.S. Valla

20 Robotics in Pediatric Urology: Pyeloplasty, 145

L. Henning Olsen and Yazan F. Rawashdeh

21 Lower Urinary Tract Laparoscopy in Pediatric Patients, 152

Rakesh P. Patel, Benjamin M. Brucker and Pasquale Casale

Part VI Genitalia

22 Hernia and Hydrocele Repair, 163

Henrik Steinbrecher

23 Orchidopexy and Orchidectomy, 170

Kim A.R. Hutton and Indranil Sau

24 Laparoscopic Orchidopexy, 183

Derek J. Matoka, Michael C. Ost, Marc C. Smaldone and Steven G. Docimo

25 Varicocele, 193

Ramnath Subramaniam and Eva Macharia

26 Hypospadias Urethroplasty, 201

Warren T. Snodgrass

27 Phalloplasty for the Biological Male, 212

Piet Hoebeke, Nicolaas Lumen and Stan Monstrey

28 Female Genital Reconstruction I, 218

Sarah M. Creighton

29 Female Genital Reconstruction II, 224

Jeffrey A. Leslie and Richard C. Rink

30 Persistent Cloaca, 232

Stephanie Warne and Duncan T. Wilcox

vi Contents

Part VII Renal Impairment Surgery

31 Hemodialysis and Peritoneal Dialysis, 241

Alun Williams

32 Kidney Transplantation, 247

Alun Williams

Part VIII Urogenital Tumors

33 Wilms Tumor and Other Renal Tumors, 257

Michael Ritchey and Sarah Conley

34 Rhabdomyosarcoma, 262

Barbara Ercole, Michael Isakoff and Fernando A. Ferrer

35 Testicular Tumors, 269

Jonathan H. Ross

36 Adrenal Tumors, 278

Bruce Broecker and James Elmore

Part IX Trauma

37 Genital Trauma, 289

Vijaya Vemulakonda and Richard W. Grady

38 Urinary Tract Trauma, 296

Ashok Rijhwani, W. Robert DeFoor, Jr, and Eugene Minevich

Part X Surgery for Urinary and Fecal Incontinence

39 Augmentation Cystoplasty, 307

Prasad P. Godbole

40 Appendicovesicostomy and Ileovesicostomy, 315

Martin Kaefer

41 Surgical Management of the Sphincter Mechanism, 324

Juan C. Prieto and Linda A. Baker

42 Surgery for Fecal Incontinence, 337

W. Robert DeFoor, Jr, Eugene Minevich, Curtis A. Sheldon and Martin A. Koyle

Index, 344

Contents vii

viii

Paul F. Austin MD, FAAP

Director of Pediatric Urology Research

Associate Professor of Urologic Surgery

St. Louis Children's Hospital

Washington University School of Medicine

St. Louis, MO, USA

Linda A. Baker MD

Director of Pediatric Urology Research

Associate Professor of Urology

University of Texas Southwestern Medical Center at Dallas;

Pediatric Urologist

Children's Medical Center at Dallas

Dallas, TX, USA

Laurence S. Baskin MD

Professor of Urology and Pediatrics

Chief of Pediatric Urology

UCSF Children's Hospital

University of California

San Francisco, CA, USA

Ben Bridgewater MBBS, PhD, FRCS (CTh)

Consultant Cardiac Surgeon

Clinical Director and Director of Clinical Audit

University Hospital of South Manchester NHS Foundation Trust

Manchester, UK

Bruce Broecker MD

Clinical Associate Professor of Pediatric Urology

Emory University School of Medicine

Atlanta, GA, USA

Benjamin M. Brucker MD

Children's Hospital of Philadelphia

Hospital of the University of Pennsylvania

Philadelphia, PA, USA

Pasquale Casale MD

Division of Pediatric Urology

Children's Hospital of Philadelphia

Philadelphia, PA, USA

Job K. Chacko MD

Fellow, Pediatric Urology

A.I. duPont Hospital for Children

Thomas Jefferson University

Wilmington, DE, USA

Laura Coates MBBS, MRCS

Paediatric Surgery Registrar

Department of Paediatric Surgery

Bristol Royal Hospital for Children

Bristol, UK

Sarah Conley MD

Resident, Urology

Mayo Clinic College of Medicine

Phoenix, AZ, USA

Sarah M. Creighton MD, FRCOG

Consultant Gynaecologist

Elizabeth Garrett Anderson Hospital

University College Hospital

London, UK

Paul Crow MbChb, MD, FRCS (Urol)

Specialist Registrar in Paediatric Urology

Department of Paediatric Urology

Bristol Royal Hospital for Children

Bristol, UK

Joe I. Curry MBBS, FRCS (Eng), FRCS (Paed Surg)

Consultant Neonatal and Paediatric Surgeon

The Hospital for Sick Children

Great Ormond Street

London, UK

W. Robert DeFoor, Jr, MD, MPH

Assistant Professor

Division of Pediatric Urology

Cincinnati Children's Hospital

Cincinnati, OH, USA

Divyesh Desai MB MS, MChir (Urology), FEAPU

Paediatric Urologist and Director, Urodynamics Unit

Great Ormond Street Hospital for Children NHS Trust;

Honorary Lecturer, Institute of Child Health

London, UK

Steven G. Docimo MD

Vice President of Medical Affairs

Professor and Director, Pediatric Urology

The Children's Hospital of Pittsburgh of UPMC;

Vice-Chairman, Department of Urology

The University of Pittsburgh Medical Center

Pittsburgh, PA, USA

Contributors

H. Serkan Dogan MD

Division of Pediatric Urology

Department of Urology

Uludag University

Bursa, Turkey

Ahmad A. Elderwy MD

Visiting Fellow, Pediatric Urology

Children's Hospital and Regional Medical Center

Seattle, WA, USA;

Assiut University Hospital

Assiut, Egypt

James Elmore MD

Clinical Assistant Professor of Pediatric Urology

Emory University School of Medicine

Atlanta, GA, USA

Barbara Ercole MD

University of Connecticut

Hartford, CT, USA

Philippa Evans BA, MBBS, MRCP, FRCA

SpR Anaesthetics

Great Ormond Street Hospital

London, UK

Fernando A. Ferrer MD

Associate Professor

Pediatric Surgery (Urology) and Oncology

Connecticut Children's Medical Center

University of Connecticut

Hartford, CT, USA

Douglas Ford MD

Professor of Pediatrics

Section of Pediatric Nephrology

University of Colorado at Denver and Health Sciences Center

The Children's Hospital

Aurora, CO, USA

Prasad P. Godbole FRCS, FRCS (Paed)

Consultant Paediatric Urologist

Sheffield Children's NHS Trust

Sheffield, UK

Richard W. Grady MD

Associate Professor of Urology

Department of Urology

The University of Washington School of Medicine

Children's Hospital and Regional Medical Center

Seattle, WA, USA

Yves Héloury MD, FEAPU

Professor of Pediatric Surgery

Head of Department

Hôpital Mère-Enfant

Nantes, France

Piet Hoebeke MD, PhD

Department of Pediatric Urology and Urogenital Reconstruction

Ghent University Hospital

Gent, Belgium

Kim A.R. Hutton MBChB, ChM, FRCS (Paed)

Consultant Paediatric Surgeon and Urologist

University Hospital of Wales

Cardiff, Wales, UK

Michael Isakoff MD

Assistant Professor

Hematology and Oncology

Connecticut Children's Medical Center

University of Connecticut

Hartford, CT, USA

Martin Kaefer MD

Associate Professor of Urology

Department of Pediatric Urology

Indiana University School of Medicine

Indianapolis, IN, USA

Chris Kimber FRACS, FRCS, MAICD

Head of Paediatric Surgery and Urology

Southern Health;

Consultant Paediatric Urologist

Royal Children's Hospital

Melbourne, Australia

Korgun Koral MD

Associate Professor of Radiology

University of Texas Southwestern Medical Center at Dallas;

Children's Medical Center

Dallas, TX, USA

Martin A. Koyle MD, FAAP, FACS

Professor of Urology and Pediatrics

University of Washington;

Chief, Division of Urology

Children's Hospital and Regional Medical Center

Seattle, WA, USA

Marc-David Leclair MD, FEAPU

Consultant in Pediatric Urology

Pediatric Surgery Department

Hôpital Mère-Enfant

Nantes, France

Jeffrey A. Leslie MD

Fellow, Pediatric Urology

James Whitcomb Riley Hospital for Children

Indiana University Department of Urology

Indianapolis, IN, USA

Nicolaas Lumen MD

Department of Pediatric Urology and Urogenital Reconstruction

Ghent University Hospital

Gent, Belgium

Contributors ix

Eva Macharia BA, MA (Oxon), MBBChir (Cantab)

Surgical Trainee

Department of Paediatric Urology

St. James University Hospital

Leeds, UK

Derek J. Matoka MD

Division of Pediatric Urology

Children's Hospital of Pittsburgh of UPMC

The University of Pittsburgh Medical Center

Pittsburgh, PA, USA

Neil McMullin MBBS, FRACS, FFin, MAICD

Director of Urology

Royal Children's Hospital;

Consultant Paediatric Urologist

Southern Health

Melbourne, Australia

Eugene Minevich MD

Associate Professor

Division of Pediatric Urology

Cincinnati Children's Hospital Medical Center

Cincinnati, OH, USA

Gerald Mingin MD

Assistant Professor of Surgery

The University of Vermont;

Attending Pediatric Urologist

Vermont Children's Hospital

Burlington, VT, USA

Stan Monstrey MD, PhD

Department of Plastic Surgery

Ghent University Hospital

Gent, Belgium

L. Henning Olsen MD, FEBU, FEAPU

Consultant Urological Surgeon

Pediatric Urologist

Clinical Associate Professor in Urology

Department of Urology, Section of Pediatric Urology

Aarhus University Hospital, Skejby;

Institute of Clinical Medicine

University of Aarhus

Aarhus, Denmark

Michael C. Ost MD

Assistant Professor of Pediatric Urology

Division of Pediatric Urology

Children's Hospital of Pittsburgh of UPMC

The University of Pittsburgh Medical Center

Pittsburgh, PA, USA

Rakesh P. Patel MD, MS, FRCS

Children's Hospital of Philadelphia

Hospital of the University of Pennsylvania

Philadelphia, PA, USA

Juan Carlos Prieto MD

Fellow, Pediatric Urology

University of Texas Southwestern Medical Center at Dallas;

Children's Medical Center at Dallas

Dallas, TX, USA

Christian Radmayr MD, PhD, FEAPU

Professor of Urology

Head, Department of Pediatric Urology

Medical University Innsbruck

Innsbruck, Austria

Yazan F. Rawashdeh MD, PhD

Department of Urology, Section of Pediatric Urology

Aarhus University Hospital, Skejby;

Institute of Clinical Medicine

University of Aarhus

Aarhus, Denmark

Ashok Rijhwani MS, MCh, FRCS, DNB

Consultant Pediatric Surgeon, Pediatric Urologist and

Transplant Surgeon

Columbia Asia Hospital

Bangalore, India

Richard C. Rink MD

Robert A. Garrett Professor of Pediatric Urology

Chief, Pediatric Urology

James Whitcomb Riley Hospital for Children

Indiana University School of Medicine

Indianapolis, IN, USA

Michael Ritchey MD

Professor of Urology

Mayo Clinic College of Medicine

Scottsdale, AZ, USA

Jonathan H. Ross MD

Head, Section of Pediatric Urology

Glickman Urological and Kidney Institute

The Children's Hospital at Cleveland Clinic;

Associate Professor of Surgery

Cleveland Clinic Lerner College of Medicine

Case Western Reserve University

Cleveland, OH, USA

Indranil Sau MBBS, MS, MRCS

Registrar in Paediatric Surgery

University Hospital of Wales

Cardiff, Wales, UK

Curtis A. Sheldon MD, FACS, FAAP

Professor and Director

Division of Pediatric Urology

Cincinnati Children's Hospital

Cincinnati, OH, USA

x Contributors

Andrew Sinclair FRCS (Urol)

Urology Registrar

Northwest England Regional Rotation

Cheshire, UK

Marc C. Smaldone MD

Division of Pediatric Urology

Children's Hospital of Pittsburgh of UPMC

The University of Pittsburgh Medical Center

Pittsburgh, PA, USA

Warren T. Snodgrass MD

Professor of Urology

Department of Urology, Pediatric Urology Section

Children's Medical Center and the University of Texas Southwestern

Medical Center

Dallas, TX, USA

Henrik Steinbrecher BSc (Hons), MBBS, MS, FRCS,

FRCS (Paed)

Consultant Pediatric Urologist

Southampton General Hospital

Southampton, UK

Ramnath Subramaniam MBBS, FRCS (Paed), FEAPU

Consultant Pediatric Urologist

St. James University Hospital

Leeds, UK

Serdar Tekgül MD

Division of Pediatric Urology

Department of Urology

Hacettepe University

Ankara, Turkey

Mark Thomas BSc, MBBChir, FRCA

Consultant Paediatric Anaesthetist

Great Ormond Street Hospital

London, UK

J.S. Valla MD, FRCS, FEAPU

Chirurgie Pédiatrique

Fondation Lenval

Nice, France

Vijaya M. Vemulakonda MD, JD

Fellow, Pediatric Urology

The University of Washington School of Medicine

Children's Hospital and Regional Medical Center

Seattle, WA, USA

Stephanie Warne MD, MB BCh, FRCS

Paediatric Surgical Registrar

Royal Hospital for Sick Children

Edinburgh, UK

Robert Wheeler FRCS, MS, FRCPCH, LLB (Hons),

LLM

Consultant Paediatric & Neonatal Surgeon

Honorary Senior Lecturer in Medical Law

Wessex Regional Centre for Paediatric Surgery

Southampton University Hospitals Trust

Southampton, UK

Duncan T. Wilcox MBBS, MD, FEAPU

Associate Professor, Pediatric Urology

Rose Mary Haggar Professorship in Urology

University of Texas Southwestern

Dallas, TX, USA

Alun Williams FRCS (Paed)

Consultant Paediatric Urologist

Nottingham University Hospitals NHS Trust

Nottingham, UK

Benjamin Wisner MD

Senior Resident, Division of Urology/Department of Surgery

The Children's Hospital

University of Colorado at Denver and Health Sciences Center

Aurora, CO, USA

Mark Woodward MD, FRCS (Paed)

Consultant Paediatric Urologist

Department of Paediatric Urology

Bristol Royal Hospital for Children

Bristol, UK

Jenny Yiee MD

Department of Urology

University of California Los Angeles

Los Angeles, CA, USA

Contributors xi

xii

The readers of any book devoted to surgical complications

will inevitably be looking for insights into how to

avoid these complications in the first place. In this outstanding

new textbook they will not be disappointed.

Duncan Wilcox, Prasad Godbole and Martin Koyle and

their impressive international team of contributors have

extended their remit beyond a simple account of complications

to produce a comprehensive and authoritative

overview of what constitutes good practice in the specialty

of pediatric urology.

As surgeons we tend to focus on factors such as

technical error, inexperience and inadequate training.

Nevertheless, it is important to recognise that many

adverse outcomes are multifactorial in origin, reflecting

system failures rather than the failings of an individual

surgeon. Indeed, weak teamwork, poor communication

and substandard overall care can conspire to undo

the work of the most experienced and talented of surgical

craftsmen. Decision making and clinical judgement

are also fundamental to good surgical practice and no

amount of technical mastery will guarantee success

when an operation has been performed unnecessarily

or for the wrong indications. For this reason the editors

and contributors have also examined the broader factors

contributing to poor surgical outcomes.

Surgeons in all specialties are rapidly having to adapt

to the demands of greater accountability and the chapter

on personal audit provides welcome guidance on this

subject.

Post operative death, the most measurable of adverse

outcomes, is thankfully a rare event in pediatric urological

practice. But whilst many non lethal complications

can be readily documented and compared between surgeons

(hypospadias revision rates and testicular atrophy

following orchidopexy being obvious examples) deriving

meaningful and reproducible measures of the success of

other procedures, such as continence rates after lower

tract reconstruction, can be more problematic. In the

face of these difficulties the editors and contributors have

nevertheless succeeded in providing pediatric urologists

Foreword

with the most comprehensive and authoritative overview

of results and complications published to date.

Surgical advances and innovations inevitably bring

risks as well as benefits. The chapters on minimally invasive

treatments of stone disease, endoscopic correction

of reflux and laparoscopic pediatric urology will be read

with particular interest by those surgeons who are still

on their personal learning curves with these new interventional

procedures.

Despite the surgeon's best endeavors, almost every

operation carries some risk of unavoidable complications

and virtually no pediatric urological procedure can guarantee

an outcome which will always meet the patient's

(more often parents') expectations. One of the more

undesirable aspects of modern surgical practice is the

prevalence of malpractice lawyers ready to exploit these

situations. In fact, many aggrieved parents may simply be

seeking an explanation and a possible apology rather than

punitive financial redress. It is to be hoped that that this

excellent book may help to forestall some misguided litigation

by enabling pediatric urologists to put occasional

individual adverse outcomes into context - by reference

to the results reported by other surgeons and within the

speciality as a whole.

Surgical training has traditionally relied upon the

apprenticeship model in which young surgeons benefited

from the experience of their seniors, which in

many instances included hard lessons learned "on the

job." This approach to specialist training is now both

unacceptable and outdated. Unacceptable because of a

regulatory climate which dictates that experience should

no longer be gained at the expense of patients. Outdated

because the old apprenticeship model is increasingly difficult

to reconcile with the reduction in trainees' working

hours and their legitimate expectation of a better work/

life balance.

As we move to a far more structured model of training

the emphasis will shift to learning about complications,

not at first or second hand, but through a collective

experience disseminated via meetings, journals and the

authoritative multi author format exemplified by this

textbook. Duncan Wilcox, Prasad Godbole and Martin

Koyle and their contributors are to be congratulated on

this outstanding contribution to the training and continuing

education of pediatric urologists at every stage

in their careers. The role of this textbook in promoting

higher standards of surgical practice is also destined to

make an invaluable contribution to the future well being

of pediatric urological patients and their families.

Professor David F.M. Thomas

Department of Paediatric Urology

Leeds Teaching Hospitals

Leeds, UK

Foreword xiii

In recent years there has been an increasing emphasis on

evidenced-based medicine. To improve surgical-based

specialties, it is essential to understand that complications

occur and that honest reporting of complications

is vital if we are to continue to develop. In the field of

pediatric urology, there are a number of textbooks that

concentrate on the diagnosis and treatment of conditions.

The aim of this book, however, is to focus on surgical

outcomes and hence complications in pediatric

urology. In addition, ways to avoid and treat complications

are discussed.

We are very grateful to our authors, who have drawn

on their experience and the literature to give an honest

account of the surgical outcomes and complications of

pediatric urological procedures and are giving advice on

how to avoid and treat such problems. Where possible

Preface

xiv

this book has relied on evidenced-based information but

where this is not available then surgical experience has

been highlighted. As you will see there is plenty of room

left in the field of pediatric urology for new evidencedbased

practice.

We would like to thank Elisabeth Dodds of Wiley-

Blackwell for all her advice with this project and trying

to ensure we remained on schedule, and to the authors

of the chapters who were understanding and timely with

their submissions.

Most importantly, we wish to thank our families for

all their support and patience with this project.

Duncan T. Wilcox

Prasad P. Godbole

Martin A. Koyle

I Principles of

Surgical Audit

Pediatric Urology: Surgical Complications and Management

Edited by Duncan T. Wilcox, Prasad P. Godbole and Martin A. Koyle

© 2008 Blackwell Publishing Ltd. ISBN: 978-1-405-16268-5

3

How to Set Up a Prospective

Surgical Audit

Andrew Sinclair and Ben Bridgewater

Introduction

Clinical audit is one of the "keystones" of clinical governance.

A surgical department that subjects itself to regular

and comprehensive audit should be able to provide

data to current and prospective patients about the quality

of the services it provides, as well as reassurance to

those who pay for and regulate health care. Well-organized

audit should also enable the clinicians providing

services to continually improve the quality of care they

deliver.

There are many similarities between audit and

research, but historically audit has often been seen as the

"poor relation." For audit to be meaningful and useful, it

must, like research, be methodologically robust and have

sufficient "power" to make useful observations; it would

be easy to gain false reassurance about the quality of care

by looking at outcomes in a small or "cherry-picked"

group of straightforward cases. Audit can be conducted

retrospectively or prospectively and, again like research,

prospective audit has the potential to provide the most

useful data, and routine prospective audit provides excellent

opportunities for patient benefit [1-4].

Much of the experience we draw on comes from cardiac

surgery, where there is a long history of structured data

collection, both in the United States and in the United

Kingdom. This was initially driven by clinicians [1-3,5],

but more recently has been influenced by politicians and

the media [6,7]. Cardiac surgery is regarded as an easy

specialty to audit in view of the high volume and proportion

of a single operation coronary artery bypass

graft (CABG) in most surgeons practice set against a

small but significant hard measurement end point of

mortality (which is typically approximately 2%).

Why conduct prospective audit?

There are a number of reasons why clinicians might

decide to conduct a clinical audit as given in Table 1.1.

Key points

• Clinical audit can be prospective and/or

retrospective.

• Audit information can be obtained from

national, hospital, and surgeon-specific data.

• A clinical department benefits from a clear audit

plan.

• Clinical audit improves patient outcome.

1

Table 1.1 Possible reasons for conducting clinical audit.

As a result of local clinical interests

As a result of clinical incident reporting

To comply with regional or national initiatives

To inform patients about surgical results

To drive continuous quality improvement

Pediatric Urology: Surgical Complications and Management For health care regulation

Edited by Duncan T. Wilcox, Prasad P. Godbole and Martin A. Koyle

© 2008 Blackwell Publishing Ltd. ISBN: 978-1-405-16268-5

4 Part I Principles of Surgical Audit

As a result of local clinical interests

Historically, many audit projects have been undertaken

as a result of local clinical interests. This may reflect

interest in a particular procedure by an individual or a

group, or may reflect concern about specific outcomes

for a particular operation.

As a result of clinical incident reporting

The major "disciplines" that ensure high quality care and

patient safety are clinical risk management and audit.

Most health care organizations should have sophisticated

systems in place to report and learn from adverse incidents

and near misses [8]. Reporting is usually voluntary

and investigated according to a "fair and just culture" but

it is unlikely that all incidents that occur are reported. If

an adverse incident is recorded, this identifies that it has

occurred, but gives no indication of how often it has happened

previously, and only limited indication of the likelihood

of recurrence. A mature organization should have

clear links between risk reporting and audit, and choose

topics for the latter based on data from the former.

To comply with regional or national

initiatives

Increasingly, audits have been driven by organizations

that exist outside a hospital. These may include audit led

by professional societies, regulatory bodies, or regional/

national quality improvement initiatives.

To inform patients about surgical

results

Across the world, health care is becoming more patientfocussed.

The modern health care consumer will sometimes

look to choose their health care provider on the basis

of that hospital or surgeon's outcomes and, even if patients

are not choosing between different hospitals, recent data

from the United Kingdom suggests that patients are interested

in outcomes of surgery by their doctors [9]. Patients'

views should inform decisions about what to audit,

and they may be interested in many areas which will be

dependent on the planned operation but may include data

on mortality, success rates, length of stay, and the incidence

of postoperative infection and other complications.

To drive continuous quality improvement

It has been shown quite clearly from cardiac surgery

that structured data collection, analysis, and feedback

to clinicians improve the quality of outcomes. This has

been detected when data is anonymous [2,3] and where

named surgeon and hospital outcomes have been published

[1,4]. The magnitude of this effect is large; in the

United Kingdom a system of national reporting for surgical

outcomes was introduced in 2001 and has led to a

40% reduction in risk-adjusted mortality [4]. The introduction

of any drug showing a similar benefit would be

heralded as a major breakthrough, but routine national

audit has not been embraced by most surgical specialties.

Simply collecting and reviewing data seems to drive

improvement, but is likely that the magnitude of the

benefits derived and the speed at which improvements

are seen can be maximized by developing a clear understanding

of what data to collect and using optimal managerial

structures and techniques to deliver better care.

There is some debate about whether publicly disclosing

health care outcomes encourages clinicians to avoid taking

on high-risk cases [1,4,7,10,11].

For health care regulation

Health care regulators have a responsibility to ensure

that hospitals, and the clinicians working in them, are

performing to a satisfactory standard. While some assurance

can be gained from examining the systems and

processes in place within an organization, the "proof of

the pudding is in the eating" and demonstrating satisfactory

clinical results is important and can only come

from analyzing benchmarked outcomes data. Regulators

of individual clinicians, such as the American Boards in

the United States and the General Medical Council in the

United Kingdom, are changing their emphasis so that it

is becoming more important for clinicians to prove they

are doing a good job, rather than this being assumed.

Routine use of structured outcomes data is included in

draft proposals for recertification by the American Board

of Thoracic Surgery and the Society for Cardiothoracic

Surgery of Great Britain and Ireland and will follow to

other specialties in time [12].

What data can be used for audit?

Routine hospital data

Most health care systems are rich in data and poor in

information. Medicare data in the United States and

Hospital Episodes Statistics in the United Kingdom

contain data on patient demographics, diagnoses, procedure,

mortality, length of stay, day cases rates, and

readmissions. These information systems are developed

for administration or financial purposes rather than

clinical ones, but may potentially contain much useful

Chapter 1 How to Set Up a Prospective Surgical Audit 5

clinical data and will often have the capacity to provide

some degree of adjustment for casemix. In the United

Kingdom, this data has historically not been trusted by

clinicians, but recently there has been increasing engagement

between doctors and the data which is improving

clinical data quality and increasing confidence. Many

UK hospitals now have systems to benchmark their

outcomes against national or other peer groups, flag up

areas of good practice, detect outlying performance, and

engage in quality improvement [13].

Ideally, hospitals should have clearly defined systems in

place to use the data: for example, they should regularly

compare their outcomes for chosen procedures against an

appropriately selected group of other hospitals. Significant

"good" practice should be celebrated and shared with

others inside and outside the organization, and bad outcomes

should be investigated. It is not infrequent that

high mortality or other clinical indictor rates may have a

clear explanation other than that of "bad" clinical practice.

The data may be incorrect, or there may be issues about

classification or attribution that explain away an apparent

alert, but structured investigation should improve the

organization's and the clinician's knowledge about their

data systems and may lead to impressions that necessitate

improvements in patient care.

Specialty-specific multicenter data

A number of surgical disciplines in the United States and

the United Kingdom have embarked upon national programs

to collect prospective disease- or operation-specific

datasets. These are usually clinically driven and have benefits

above routine hospital data in that a more useful

dataset can be designed for specific purposes and in particular

can look in more detail at subtleties of casemix and

specific clinical outcomes in a way that is more robust and

sensitive than that derived from routine hospital administration

systems. Contemporary cardiac surgical datasets

collect variables on preoperative patient characteristics,

precise operative data and postoperative mortality, ICU

stay, hospital stay, reexplorations, infection, renal failure,

tracheostomy, blood usage, stroke rate, and intraaortic

balloon pump use. The preoperative and operative data

allow outcomes to be adjusted for case complexity to prevent

comparison of "apples and oranges" by various algorithms

such as the EuroSCORE [14].

Setting up specialty-specific multicenter audit raises

a number of challenges including defining clarity of

purpose, gaining consensus, agreeing a dataset, securing

resource, overcoming information technology issues,

and clarifying ownership of data, information policies,

and governance arrangements. In cardiac surgery, there

is now increasing international dialogue between professional

organizations to move toward the collection of

standardized data to allow widespread comparisons.

Locally derived data

Individual hospital departments will often decide to

audit a specific theme that may be chosen because of

clinical risk management issues, subspecialist interest,

or other concerns. In the UK National Health Service

(NHS), dedicated resource for audit was historically "top

sliced" from the purchasers of health care to generate a

culture of clinical quality improvement, but commentators

are divided about whether significant benefits have

been realized from this approach [9]. In the early stages,

large amounts of audit activity were undertaken, but

there were significant failures in subsequently delivering

appropriate change. To maximize the chances of improving

care as a result of audit, the following should be considered.

Will the sample size be big enough to be useful?

What dataset is needed? Will that data be accessible from

existing hospital casenotes or will prospective data collection

be necessary? Is there an existing robust benchmark

to which the results of the audit can be compared? How

will the "significance" of the results be analyzed? Does

conducting the audit have financial implications? Will the

potential results of the audit have financial implications?

Are all stakeholders who may need to change their behavior

as a result of the audit involved in the process?

Techniques of data collection

Historically, the majority of audit activity was conducted

from retrospective examination of casenotes,

which was labour intensive and relied on the accuracy

and completeness of previously recorded data. There has

subsequently been increasing use of prospective data collection,

much of which has been based on paper forms.

This obviously improves the quality of data, but again

requires time and effort from clinical or administrative

staff for completion. The development of care pathways

whereby multidisciplinary teams manage clinical conditions

in predefined ways are thought to improve patient

outcomes and will generate structured data that is readily

amenable to audit. The use of modern information

technology to support care pathways is the "holy grail" of

effective audit - all data is generated for clinical use and

the relevant subset of that data can then be examined for

any relevant purpose. The care pathway can be adapted

6 Part I Principles of Surgical Audit

to include new or alternative variables as required. All

data collection can be networked and wireless, assuming

issues about data access, confidentiality, and security are

resolved. Variations on this theme are now available in

many hospitals and it is these principles that underpin

a major IT investment in the UK NHS [15]. Maximizing

benefits from this approach raises a number of challenges

including producing major changes in clinical

practice and medical culture.

Good practice in audit

A clinical department should benefit from a clear forward

plan about its audit activity that should be developed

by the multidisciplinary team in conjunction

with patients and their carers. The audit activity should

include an appropriate mix of national, local, and risk

management driven issues, and the specifics should

depend on the configuration of services and local preferences.

The plan should include thoughts about dissemination

of results to users and potential users of

the services. The multidisciplinary team should include

doctors, professionals allied to medicine, and administration

staff. Adherence to the audit plan should be

monitored through the departmental operational management

structures. For the department to be successful

in improving care as a result of audit, there should

be clear understanding of effective techniques of change

management.

Arguments against audit

In the United Kingdom, audit has been an essential part

of all doctors' job plans for a number of years, but audit

activity remains sporadic. In some high-profile specialties

such as cardiac surgery, comprehensive audit has

been led by clinicians and driven by politicians and the

media. In other areas there has been little or no coordinated

national audit activity. This may be due to a perceived

lack of benefits from audit from clinicians along

with failure to meet challenges in gaining consensus or

difficulties in securing adequate resource. The experience

from cardiac surgery is that structured national

audit improves the quality of mortality outcomes [1-4].

It is likely that other issues such as complication rates are

also reduced with associated costs savings, and as such

effective audit may well pay for itself.

Summary

In modern health care, patients are increasingly looking

to be reassured about the quality of care they receive and

doctors are being driven toward demonstrating their

competence, rather than this being assumed. Hospital

departments should have a robust clinical governance

strategy that should include "joined-up" clinical risk

management and audit activity. There are strong arguments

that structured audit activity improves the quality

of outcomes and for these benefits to be maximized

there should be involvement of multidisciplinary teams

supported by high-quality operational management.

References

1 Hannan EL, Kilburn H, Jr., Racz M, Shields E, Chassin MR.

Improving the outcomes of coronary artery bypass surgery

in New York State. JAMA 1994;271:761-6.

2 Grover FL, Shroyer LW, Hammermeister K, Edwards FH,

Ferguson TB, Dziuban SW et al. A decade of experience

with quality improvement in cardiac surgery using the

Veterans Affairs and Society of Thoracic Surgeons national

databases. Ann Surg 2001;234:464-74.

3 Hammermeister KE, Johnson R, Marshall G, Grover FL.

Continuous assessment and improvement in quality of care.

A model from the Department of Veterans Affairs Cardiac

Surgery. Ann Surg 1994;219:281-90.

4 Bridgewater B, Grayson AD, Brooks N, Grotte G, Fabri B,

Au J et al. Has the publication of cardiac surgery outcome

data been associated with changes in practice in Northwest

England: An analysis of 25,730 patients undergoing CABG

surgery under 30 surgeons over 8 years. Heart 2007; January

19; [Epub ahead of print]. PMID: 17237128.

5 Keogh BE, Kinsman R. Fifth national adult cardiac surgical

database report 2003.

6 Available at http://society.guardian.co.uk/nhsperformance/

story/0,,1439210,00.html accessed on 25.01.2008 .

7 Marshal M, Sheklle P, Brook R, Leatherman S. Dying to

know: Public release of information about quality of healthcare.

Nuffield Trust and Rand 2000.

8 An organisation with a memory. Report of an expert group

on learning from adverse events in the NHS chaired by the

Chief Medical Officer accessed on 25.01.2008. http://www.

dh.gov.uk/en/Publicationsandstatistics/Publications/Publica

tionsPolicyAndGuidance/DH_4065083.

9 Good Doctors, safer patients. Proposals to strengthen the

system to assure and improve the performance of doctors

and to protect the safety of patients. A report by the Chief

Medical Officer accessed on 25.01.2008. http://www.dh.gov.

uk/en/Publicationsandstatistics/Publications/PublicationsP

olicyAndGuidance/DH_4065083.

Chapter 1 How to Set Up a Prospective Surgical Audit 7

10 Chassin MR, Hannan EL, DeBuono BA. Benefits and hazards

of reporting medical outcomes publicly. N Engl J Med

1996;334:394-8.

11 Dranove D, Kessler D, McCellan M, Satterthwaite M. Is

more information better? The effects of report cards on

healthcare providers. J Polit Econ 2003;111:555-88.

12 Trust, assurance and safety. The regulation of health professionals

in the 21st Century. The Stationary Office: London,

February 2007. http://www.dh.gov.uk/en/Publicationsandst

atistics/Publications/PublicationsPolicyAndGuidance/DH_

065946.

13 Available at www.drfoster.co.uk. accessed on 25.01.2008 .

14 Roques F, Nashef SA, Michel P et al. Risk factors and

outcome in European cardiac surgery; analysis of the

EuroSCORE multinational database of 19,030 patients. Eur

J Cardiothorac Surg 1999;15:816-23.

15 Available at www.connectingforhealth.nhs.uk/. accessed on

25.01.2008.

8

Evaluating Personal Surgical

Audit and What to Do If Your

Results Are Outside the "Mean"

Andrew Sinclair and Ben Bridgewater

Introduction

Any well-conducted audit should give information

about systems and outcomes related to patient care.

Data collection that generates new information about

patient outcomes should be classified as research and

to be regarded as audit, results need to be compared

against a previously defined and accepted standard.

Often an audit will demonstrate satisfactory outcomes

and this in itself may be a useful finding which should

be of interest to patients, clinicians, managers, commissioners,

and regulators of health care. It is hoped that

structured and regular audit data collection will lead

to ongoing improvements in quality as described in

the previous chapter. On occasions audit results will be

unacceptable and it is essential that this is recognized

and acted upon.

Presentation and analysis of data

Effective audit requires clarity of purpose. When an audit is

conceived the clinical question should be clearly stated and

the data required to generate an answer should be defined.

It is also important to be sure to what outcomes you will

compare yourself, and there may be a number of options.

Data on mortality or complication rates may be available

from pooled national or regional registries [1-4]. Results

of specific series of cases may be published through peer

review journals for individual hospitals or individuals,

but these outcomes may often be better than the "norm"

because of submission and publication bias. False reassurance

may be gained from comparing outcomes with outdated

historical results; in cardiac surgery in the United

Kingdom a widely accepted risk-adjustment algorithm, the

EuroSCORE [5], has been used to benchmark hospitals and

surgeons in recent years. This was developed in a multicenter

study in Europe in 1997 and improvements in overall

quality of care in the United Kingdom are such that it no

longer reflects current practice [6]. In the United Kingdom,

any cardiac surgeon who is not currently performing significantly

better than predicted by the EuroSCORE would

Key points

• Audit is the comparison of surgical results

against a previously defined and accepted

standard.

• Published results may be better than the normal

surgeon's.

• Complexity specific audit is important.

• Dealing with outlying performance can be

"directive" or "collaborative" depending on the

surgeon.

• Surgeons are responsible for ensuring

satisfactory quality of care.

2

Pediatric Urology: Surgical Complications and Management

Edited by Duncan T. Wilcox, Prasad P. Godbole and Martin A. Koyle

© 2008 Blackwell Publishing Ltd. ISBN: 978-1-405-16268-5

Chapter 2 Evaluating Personal Surgical Audit 9

have a mortality rate that was higher than that of their

peer group. It is unclear whether it still accurately predicts

mortality elsewhere in Europe. This concept of "calibration

drift" for cardiac surgery has been seen in both the United

Kingdom and the United States.

It is possible to compare outcomes between units or

surgeons simply by using "crude" or nonrisk-adjusted

data. Cardiac surgeons have focussed on mortality, as

it is a robust primary end point. In pediatric urology,

mortality is not frequent enough to provide a meaningful

measure and more appropriate end points need to be

developed.

Using nonrisk-adjusted data has simplicity and transparency

on its side but is not embraced with enthusiasm

by the majority of surgeons. It is clear that there are quite

marked differences in patients' characteristics between

different units in cardiac surgery and this variability is

probably greater between surgeons who have different

subspecialist interests [7]. These issues will surely apply to

other areas of surgery. Many surgeons are concerned that

any attempt to produce comparative performance using

nonrisk-adjusted data will stimulate a culture whereby

higher risk patients are denied surgery to help maintain

good results - the so-called risk averse behavior. To make

data comparable between individual surgeons and units,

there have been a number of attempts to adjust for operative

risk in cardiac surgery [8-10]. Other specialties will

need to develop appropriate methodology, and ideal tools

should be accurate numerical predictors of observed risk

(i.e. be calibrated correctly) and be able to discriminate

appropriately across the spectrum of risk (i.e. accurately

differentiate between lower- and higher-risk patients).

In addition to the appropriate use of risk adjustment,

some units have found graphical techniques of

presenting outcomes data useful to monitor performance.

Various techniques have been used to help analyze

results and detect trends or outlying performance at an

early stage, such as cumulative summation or variable

life-adjusted display plots. These curves may be adapted

to include predicted mortality to enable observed and

expected mortality to be compared. These techniques are

well described by Keogh and Kinsman [2]. More recently

interest is developing for measuring outcomes using statistical

process control charts, which are widely used in

manufacturing industry. These charts use units of time,

typically months when institutions are under scrutiny

and the outcome of interest is mortality, and display

actual mortality against expected mortality, using control

limits to define acceptable and unacceptable performance

[11].

The use of funnel plots is becoming popular as a way of

displaying hospital or individual mortality [12]. They are

simply a plot of event rates against volume of surgery, and

include exact binomial control limits, to allow excessive

mortality to be easily detected. They give a "strong visual

display of divergent performance" [2]. They have been

used to analyze routine data to define clinical casemix and

compare hospital outcomes in urology [13].

Classical statistical techniques may be used to compare

individual outcomes with a benchmark. When analyzing

data from an individual hospital or surgeon, it is

probably appropriate to select 95% confidence intervals

such that if significant differences are observed, there is

a 1 in 20 probability that these are due to chance alone.

Things become more difficult when many hospitals or

surgeons are compared to a national benchmark. In the

United Kingdom there are over 200 cardiac surgeons and

any comparison of the group against the pooled mortality

using 95% confidence intervals would raise a high

probability of detecting outlying performance due to

chance alone because of multiple comparisons, and it is

appropriate to adjust for this. The choice of confidence

intervals will always end up as a balance between ensuring

that true outlying performance is detected without

inappropriately creating stigma for surgeons with satisfactory

outcomes. It may be useful to select different

confidence limits for different purposes. Tight limits

may be appropriate for local supportive clinical governance

monitoring; one hospital in North West England

launches an internal investigation into practice if a cardiac

surgeon's results fall outside 80% confidence limits

but wider limits of 99% have been used to report those

surgeons' outcomes to the public [14].

Dealing with outlying performance

Detecting clinical outcomes that fall outside accepted

limits does not necessarily indicate substandard patient

care, but any analysis which indicates concern should

trigger further validation of the data if appropriate and

then, if indicated, an in-depth evaluation of clinical practice

which may include analysis of subspecialty, casemix,

and an exploration of the exact mechanisms of death

or complications. This process may lead to reassurance

that practice is satisfactory. Ideally this should be initiated

by the concerned clinician who should be keen to

learn from the experience to improve their practice. An

excellent example comes from pediatric cardiac surgery

where a surgeon developed concerns about his mortality

10 Part I Principles of Surgical Audit

outcomes following the arterial switch operation (which is

complex, technically challenging, and congenital surgery)

[15]. He studied his outcomes in detail using Cumulative

Summation (CUSUM) methodology and determined

that things were worse than he would have expected due

to chance alone. He then underwent retraining with a colleague

from another hospital with excellent outcomes,

adapted his practice, and subsequently went on to demonstrate

good outcomes in a further series of consecutive

cases.

On occasion, the process of investigating outlying

outcomes may be difficult for the individual hospital or

surgeon involved. The investigation may raise significant

methodological questions about the techniques of analysis

and subsequent examinations. The cause of substandard

results may be difficult to detect but may relate to

failures in the systems of care in the hospital or department,

or failures in the individual [16,7].

Clinical governance is an individual, departmental,

and hospital responsibility. While the onus should be on

the individual with unsatisfactory outcomes to investigate

and change their practice, they may need support,

advice, and direction from their clinical and managerial

colleagues. Over recent years the roles of different organizations

in clinical governance is becoming clearer. Most

hospitals should now have increasingly effective management

structures for promoting quality improvement and

detecting suboptimal performance [17,18].

The investigation of unsatisfactory outcomes can be

facilitated by an appropriate clinical leadership, and

different techniques may be necessary for different circumstances

with the concepts of "situational leadership"

being useful to match the managerial intervention to the

willingness and the readiness of the individual whose

practice is being investigated [19]. Two examples make

this point. A newly appointed cardiac surgeon had three

adverse outcomes following the same type of operation

that seemed to the colleagues to be due to a similar

mechanism. Despite discussions the surgeon involved

had little or no insight into the problem. No confidence

intervals for performance were crossed because of the

small volume of cases involved but, due to the clinical

concerns, the surgeon was subjected to forced but

supportive retraining of his intraoperative techniques,

which led to re-introduction of full independent practice

within a few months and excellent publicly reported

results for that operation several years later. This would

be described in a situational leadership model as a "directive"

approach. A second example is that of a senior surgeon

with a low volume mixed cardiothoracic practice

who had a "bad run" of cardiac results, which again led

to outcomes that failed to generate statistically significant

mortality outcomes. At his own initiation he involved

his clinical managers and launched an in-depth analysis

of his practice and detected that he was conducting

very high-predicted risk surgery despite lower volumes

of surgery than some single specialty colleagues. He was

also suspicious of a potential common mechanism of

adverse outcomes in several cases of mortality and some

cases of morbidity. Along with colleagues he changed

his referred practice to make it more compatible with

low volume mixed cardiothoracic surgery and adapted

his technique of surgery to avoid further problems. This

again resulted in excellent subsequent outcomes. This

would be described in a situational leadership model as a

"collaborative" approach. From a managerial perspective

both examples led to satisfactory ends, but adopting the

appropriate leadership style was important in reaching

the desired conclusions.

In addition to the roles of the individual and the hospital

in ensuring satisfactory outcomes, other agencies

should be acting to support the process. In the United

Kingdom the Chief Medical Officer has recently produced

a report entitled "Good Doctors, Safer Patients"

about regulation of health care, where it is proposed that

the General Medical Council will have overall responsibility

for professional regulation, but will pass significant

responsibilities down to employers [17]. It is suggested

that professional societies should set clear unambiguous

standards for care, and recertification of doctors should

be dependent on achieving those standards. Patient consultation

as part of this report has suggested that patients

are keen to see that satisfactory outcomes of treatment

by their doctors form part of this process. This direction

of travel in the United Kingdom is similar to that proposed

by the American Board of Medical Specialties and

is a long way from the culture in which most doctors

were trained. It will be a challenge for professional societies

and the profession to deliver on this agenda.

Summary

Most audit projects will deliver results that demonstrate

clinical practice is satisfactory. There is some evidence

that scrutiny of results alone can contribute to improvements

in quality. On occasions audit will flag up concern

about clinical processes or outcome, but it is important

that the data and the methods are "fit for purpose." The

responsibility for ensuring that satisfactory quality of

Chapter 2 Evaluating Personal Surgical Audit 11

care is given and demonstrated is the responsibility of

all involved in health care delivery including individual

practitioners, employers, commissioners, professional societies,

and regulators.

References

1 Grover FL, Shroyer LW, Hammermeister K, Edwards FH,

Ferguson TB, Dziuban SW et al. A decade of experience

with quality improvement in cardiac surgery using the veterans

affairs and society of thoracic surgeons national databases.

Ann Surg 2001;234:464-74.

2 Keogh BE, Kinsman R. Fifth national adult cardiac surgical

database report 2003.

3 Available at http://www.nnecdsg.org/. Accessed 25.01.2008

4 Available at www.scts.org. Accessed 25.01.2008

5 Roques F, Nashef SA, Michel P et al. Risk factors and

outcome in European cardiac surgery; analysis of the

EuroSCORE multinational database of 19,030 patients. Eur

J Cardiothorac Surg 1999;15:816-23.

6 Bhatti F, Grayson AD, Grotte GJ, Fabri BM, Au J, Jones MT

et al. The logistic EuroSCORE in cardiac surgery: How well

does it predict operative risk? Heart 2006;92:1817-20.

7 Bridgewater B, Grayson AD, Jackson M et al. Surgeon

specific mortality in adult cardiac surgery: Comparison

between crude and risk stratified data. BMJ 2003;327:13-7.

8 Parsonnet V, Dean D, Bernstein AD. A method of

uniform stratification of risk for evaluating the results of

surgery in acquired heart disease. Circulation 1989;79:I3-12.

9 Roques F, Michel P, Goldstone AR, Nashef SAM. The logistic

EuroSCORE. Eur Heart J 2003;24:1-2.

10 Roques F, Nashef SA, Michel P et al. Risk factors and

outcome in European cardiac surgery; analysis of the

EuroSCORE multinational database of 19,030 patients. Eur

J Cardiothorac Surg 1999;15:816-23.

11 Benneyan RC, Lloyd RC, Plsek PE. Statistical process control

as a tool for research and healthcare improvement. Qual Saf

Health Care 2003;12:458-64.

12 Speigelhalter D. Funnel plots for comparing institutional

performance. Stat Med 2005;24:1185-202.

13 Mason A, Glodacre MJ, Bettley G, Vale J, Joyce A. Using routine

data to define clinical case-mix and compare hospital

outcomes in urology. BJU Int 2006;97:1145-7.

14 Bridgewater B on behalf of the adult cardiac surgeons on

NW England. Mortality data in adult cardiac surgery for

named surgeons: Retrospective examination of prospectively

collected data on coronary artery surgery and aortic

valve replacement. BMJ 2005;330:506-10.

15 de Leval MR, Francois K, Bull C et al. Analysis of a cluster of

surgical failures. Application to a series of neonatal arterial

switch operations. J Thorac Cardiovasc Surg 1994;107:914-23.

16 Learning from Bristol: The report of the public inquiry into

children's heart surgery at the Bristol Royal Infirmary 1984-

1995. Available at http://www.bristol-inquiry.org.uk/. http://

www.dh.gov.uk/en/Publicationsandstatistics/Publications/

PublicationsPolicyAndGuidance/DH_4002859 accessed

25.01.2008

17 Good Doctors, safer patients. Proposals to strengthen the

system to assure and improve the performance of doctors

and to protect the safety of patients. A report by the Chief

Medical Officer. http://www.dh.gov.uk/en/Publicationsands

tatistics/Publications/PublicationsPolicyAndGuidance/DH_

4002859 accessed 25.01.2008

18 An organisation with a memory. Report of an expert group

on learning from adverse events in the NHS chaired by the

Chief Medical Officer. http://www.dh.gov.uk/en/Publication

sandstatistics/Publications/PublicationsPolicyAndGuidance

/DH_4002859 accessed 25.01.2008

19 Hersey P, Blanchard KH. Leadership and the One Minute

Manager. William Morrow, 1999. HarperCollins Business;

New edition (1 Mar 2000).

12

The Implications of a Poor

Surgical Outcome

Robert Wheeler

Introduction

One of the few stimuli prompting a unanimous response

from any group of surgeons is the failure to produce the

desired result, either from an operation or from a program

of management. The response will be a mixture

of empathy, regret, disappointment, frustration ... and

a lingering fear that litigation, or worse, may ensue. In

the early 21st-century practice, a "poor outcome" can

encompass anything from the irritation of a minor delay

due to a misplaced ultrasound report, to the loss of the

wrong kidney, or possibly a life. This chapter deals with

how to approach a patient with a poor outcome while

describing the legal pathways that can be taken. This

chapter describes the situation from the viewpoint of the

United Kingdom's legal system. Every country has slightly

different laws but this chapter remains relevant for those

surgeons practicing outside of the United Kingdom, as

parallels can be drawn with most legal systems.

Disclosure of poor outcome

An apology

Doctors often debate the advisability of an apology in

these circumstances, fearing that this could be construed

as an admission of guilt, analogous to the advice given

to motorists by their insurance companies if involved in

a collision. The analogy is flawed. A poor outcome from

treatment may be the result of mismanagement; but the

final determination of fault, if present at all, will result

from a complex investigation and logical assessment.

While it could be argued that a fulsome apology may be

seen as an indication of "guilt," this will have a minimal

effect on the process that will establish whether a doctor's

behavior has fallen below the reasonable standard

(and if so, whether this lapse has caused the harm that

is alleged). This effect has to be balanced against the

undoubted good that an apology will do, i.e. benefiting

the patient and reflecting well on the doctor's propriety

and openness. An apology is therefore very strongly recommended;

at the very least, failing this, an expression

of regret is mandatory.

An explanation

An explanation as to how the suboptimal result has

occurred is also necessary. Our society has chosen to regard

a patient's autonomy, their right of self-determination,

as the paramount consideration when dealing with their

health. This manifests as a primacy for confidentiality

and for the need to provide consent, both of which are

sometimes put ahead of what may be in the (medical) best

interests of the patient.

It therefore follows that any information that a doctor

possesses concerning a patient must be shared with

that patient, or the parents. There is a theoretical tension

when considering children, since a child who is judged

to be competent to provide consent is also entitled to

decide whether their information should be shared with

their parents. In practice, this entitlement should be

honored by simply checking with the competent child

that they have no objection to their parents being told;

in the vast majority of cases, there will be none. So a full

3

Pediatric Urology: Surgical Complications and Management

Edited by Duncan T. Wilcox, Prasad P. Godbole and Martin A. Koyle

© 2008 Blackwell Publishing Ltd. ISBN: 978-1-405-16268-5

Chapter 3 The Implications of a Poor Surgical Outcome 13

discussion should follow, to enlighten the family as to

how the poor outcome has occurred.

An obligation to disclose errors that are

not immediately obvious to the patient?

One particular difficulty is where an error has been made

that is not immediately obvious to the patient, but has

caused some tangible harm. There may be a temptation

not to disclose. However, truth telling is a cornerstone

of a trusting relationship, and trust between individuals

is central to civilized life. Since "morality" pertains to

character and conduct and has regard to the distinction

between right and wrong, truth telling seems to be, inescapably,

a moral activity. In families' moral education of

their children, the universal duty adults impose on children

to "own up" misdemeanors reflects this necessity to

ensure that ordinary citizens are honest with each other.

It also implies that "honesty" concerns the disclosure of

hidden information, not simply the avoidance of the lie.

The relationship between doctors and their patients is

not ordinary. It is described as a fiduciary relationship,

emphasizing the necessity for mutual trust, confidence,

and certainty (L. fiderer: to trust; fides: faith). In conclusion,

considering the fiduciary relationship between

doctors and their patients, and the lack of distinction

between a lie and failing to disclose hidden information,

there is a moral obligation to disclose.

Within the doctrine of behavioral ethics, the central

"good" elements of human behavior rest upon honesty,

probity, and truthfulness. From this perspective, disclosure

of error would be considered as an ethical obligation.

How does the general public approach disclosure? In

reality, generally, by ignoring the ethical and moral obligations

outlined above. The man who owns up to scratching

his neighbor's boat while it was unattended would be perceived

to have done the "right thing," but such behavior

might generate both mild surprise and congratulations

on being "decent." Failure to report the damage would

lead to a disconsolate but unsurprised owner, resigned to

the fact that "no one ever owns up these days."

On a larger scale, viewing "acknowledging error" on

Google reveals a robust avoidance of the obligation,

"White House strategists conclude that acknowledging

error is not an effective political tactic" [1]. Such comments

recognize the moral obligation, but honor it in its

avoidance. The general tenet of civil law is that the citizen

should look after himself. There is no evidence of a

civil obligation to report an error.

But the General Medical Council (GMC) [2] advises

that doctors should report mistaken diagnoses. This

advice is reiterated by the British Medical Association

(BMA). Such advice does not specifically cover the area

of operative error, although does include circumstances

where patients "suffer harm."

Although this advice is not binding, it does represent

the view of a recognizable body of medical opinion.

Although civil judges do not invariably follow the GMC/

BMA guidance, they may be influenced by it, and if they

choose to, may reflect the guidance in any future judgements.

What damage could flow from failure to know

that something has gone wrong? The patient's eventual

discovery may cause distress, but it could be difficult

to claim that this distress equates to personal damage

of a degree that a court would view as worthy of financial

recompense. Chester v. Afshar [2004] made lawyers

(briefly) believe that there was an appetite among senior

judges for expanding the law [3], creating a new tort

of "failure to report a medical error," a novel category of

clinical negligence. Within a matter of weeks, a further

House of Lords case (Gregg v. Scott [2005]) indicated

that radical expansion was most unlikely.

Many NHS hospitals regard failure to report a serious

untoward incident to the hospital as a disciplinary offence.

However, there is no defined obligation to disclose the

information to the patient, and one can see a potential

conflict of interest on behalf of the hospital when deciding

to disclose or not. However, should your hospital

take a similar line, reporting a clinical error that could

be construed as serious would seem prudent. But from

a moral and ethical point of view, patients should also

be told, before the hospital, because of the GMC's guidance

and the fiduciary relationship a doctor has with the

patient. Failing to disclose to the patient does not appear

to create a liability in negligence, and even if the failure

were admitted as falling below the reasonable standard of

care, the claimant would have an uphill struggle in proving

causation (vide infra). The National Patient Safety

Authority's (NPSA) safer practice notice [4] advises

health care staff to "apologize to patients, their families or

carers if a mistake or error is made that leads to moderate

or severe harm or death, explain clearly what went wrong

and what will be done to stop the problem happening

again." This consolidates the advice from the GMC/BMA,

so the chances of being liable to civil actions will increase;

but will only succeed if judges choose to follow the line

of expanding the scope of clinical negligence.

From the professional point of view, given the GMC

guidance, full disclosure is appropriate. In the rare case

where disclosure would cause clinical harm, perhaps psychiatric

injury, the doctrine of therapeutic privilege will

14 Part I Principles of Surgical Audit

protect the doctor who correctly applies it and withholds

disclosure. As a clinical decision, disclosure of medical

error puts the doctor in an unassailable position. The

hospital may wish disclosure had not occurred, but will

hardly make their displeasure visible. Paradoxically, there

is evidence that disclosure of error reinforces, rather than

diminishes, the relationship between doctor and patient.

Even if the admission leads to litigation, the court is

likely to view the voluntary disclosure much more favorably

than apparent concealment.

From the perspective of the patient

Local resolution

If the patient complains of the outcome, the complaint

will initially be dealt with locally, in the hope that resolution

can be achieved. At the time of writing, this means

that the claim is investigated by the hospital, which may

involve experts from within or outwith the organization

to take an initial view on whether the hospital should

accept liability for the poor outcome. If this initial investigation

and suggested remedy satisfies the patient, the

matter is brought to a close. If not, then the complainant

may request a convenor to appoint a panel to hear

the case. The panel consists of a lay chairman and two

members independent of the hospital. Clinical assessors

are appointed to advise the panel when the complaint

involves the exercise of clinical judgement. There is no

provision for an appeal of the panel's decision.

The Ombudsman

If the complainant is refused panel review, he may refer

that decision to the Health Service Commissioner [5]

(Ombudsman), who may recommend that the decision to

refuse a panel review be reconsidered. The Ombudsman

is able to investigate complaints about clinical judgement,

including those arising from independent providers

of health services [6] and retired practitioners

[7]. The Commissioner provides comments and recommendations,

but has no power to award compensation,

other than ex gratia payments for out of pocket expenses.

Referral to the panel or the Ombudsman is not allowed

if either civil or criminal proceedings have started.

NHS redress

Following a report [8] by the Chief Medical Officer

(CMO) in 2003, there are now arrangements in England

and Wales to create a scheme for redress [9] without

civil proceedings. This scheme will cover injuries caused

to patients by an act or omission concerned with diagnosis

of illness, care, or treatment. Despite what some

Parliamentarians may have believed during debates, this

is not a no-fault compensation scheme. It is anticipated

that it will cover treatment by the NHS, even if provided

in private hospitals. However, the scheme cannot be

engaged if civil proceedings have begun, and will terminate

immediately if they commence.

The scheme must comprise: an explanation, an apology,

a report on the action proposed to avoid future similar

cases, and an offer of compensation. The latter may be

monetary, or could take the form of a contract to provide

restorative care and treatment. If monetary compensation

is awarded for pain, suffering, and loss of amenity, there

must be an upper limit on the amount to be offered. The

implementation of such a scheme will be dependent on

political will and the funding that is diverted to pay for it.

If it is implemented in the described form, there may be

a perverse incentive for the hospitals to settle low value

claims irrespective of liability. If the complainant agrees

to the offer, civil action will no longer be available to

them. But even a relatively low guaranteed offer will seem

attractive to both the hospital and the complainant, since

it provides financial closure to both parties, a far cry from

the uncertainty and expense of civil actions. What it fails

to provide is the guarantee that the doctor who is not at

fault will have their blamelessness and hence good reputation

publicly acknowledged.

Litigation

If the local or extended resolution process fail, or the

patient wish for redress in the civil courts, litigation will

commence. The action will usually be directed against

the NHS hospital; only naming the defending surgeon

if the complaint arose from private practice. The purpose

will be ostensibly to provide the claimant with the

full facts relating to the case, and financial compensation.

The claimant is likely to be suing on the grounds

of the tort (civil wrong) of negligence. To establish this,

several separate elements will need to be established.

Firstly, the surgeon was responsible for the claimant's

care at the time of the incident. In hospital practice this

is usually straightforward. A doctor has a single and

comprehensive duty to exercise reasonable care and skill

in diagnosing, advising, and treating the patient [10].

The test is whether the surgeon's conduct was reasonable,

and this will be determined in comparison to

the objective standard, which is the standard of his or

her peers; "it is sufficient if he exercises the ordinary

skill of an ordinary competent man exercising that

Chapter 3 The Implications of a Poor Surgical Outcome 15

particular art" [11]. Therefore the claimant has to show

that the surgeon's practice fell below the standard that

would be set by his or her professional peer group [12].

It is this "standard of care" that is established by the

expert witnesses who will be consulted concerning the

case. However, the expert witnesses will also have to satisfy

the judge that the standard they have identified can

stand up to scrutiny, and be found to be coherent and

logical [13]. Finally, the claimant has to show that the

injury sustained was caused by the lapse in the standard

of care, and that the damage must be such that the law

regards it proper to hold the defendant responsible for it.

The first of these two elements of causation is established

on the basis of expert evidence, the latter by the court.

In private practice, the patient has the additional

option of bringing an action in the law of contract, on

the basis that they have purchased a service that has been

imperfectly delivered. If a consent form has identified a

particular surgeon, and the operation is performed by

another; or if a surgeon fails to perform a promised procedure,

then breach of contract will occur. An explicit

and unequivocal guarantee, "I assure you that your

vasectomy will be successful and you will never father

children again" creates a contractual warranty on the

basis of which action may be taken.

From the perspective of the surgeon

Local

The consequences for the surgeon are largely dependent

on the magnitude of the damage caused. There is

no doubt that close attention to and compliance with

local procedures of incident reporting are vital to limit

the negative consequences for the surgeon. The medical

defense organizations emphasize the importance

of alerting them to any potential claims as soon as a

complaint has been made. If frequently repeated irritating

inconveniences are being caused to patients, it is

likely that the surgeon will be asked to play his or her

part in eliminating any procedural errors responsible.

Where serious injury has been caused, many hospitals

will investigate the matter either through a system of

Root Cause Analysis [14], or using some form of review

group, usually composed of senior clinicians, risk managers,

and executives. Should a serious breach of professional

conduct be suspected, the Medical Director of the

hospital will become involved and may refer the matter

for local adjudication (the "three wise men" approach)

or may refer on to the GMC. In the rare circumstance

when a patient's death may have been caused by gross

incompetence, the possibility of a criminal charge may

be considered. The police would interview all concerned,

and the Crown Prosecution Service (in England) would

then consider whether a conviction was likely on the

basis of the written evidence, and whether it was in the

interests of justice that the doctor should be charged

with gross negligence manslaughter. It is noteworthy that

the indemnity insurance that covers hospitals in England

[15] is not available for criminal matters, reinforcing the

importance of maintaining close contact with the medical

defense organization.

General Medical Council (GMC)

Having been established as a result of lobbying by the

BMA in 1858 [16], the GMC [17] sets standards for

doctors and examines their performance and behavior

against those standards. This important role acts as a

safety net, allowing cases to be considered that are not

actionable at law, but fall below the standard that should

be expected of an ethical practitioner.

Having changed radically in recent times [18], further

changes are suggested by the CMO [19], including

the devolution of some powers to a local level. From the

surgeon's point of view, it is the Fitness to Practise Panel

that will determine whether his or her fitness to practice

is impaired. If it is, a reprimand may be issued; conditions

may be imposed on the doctor's registration, or

suspension, or erasure ordered. An appeal mechanism to

the courts is available to doctors throughout the United

Kingdom. But an appeal is also available to complainants,

if dissatisfied with CMC decision-making.

Council for Healthcare Regulatory

Excellence

This organization exists to review the decisions of regulators,

including those of the GMC. It has a duty to

challenge the results of decisions that it considers to be

excessively severe or lenient, although most of its challenges

are on the latter grounds. The Council will review

complaints about the GMC decisions and also scrutinizes

decisions on its own behalf. The Council has the power

to refer decisions to the High Court for reconsideration,

if it is considered that their leniency was incompatible

with adequate protection of the public. The effect of this

process is that a complaint to the GMC may result in

either an acquittal or reprimand falling short of suspension

from the GMC, but the case being reopened by the

Council for Healthcare Regulatory Excellence (CHRE)

and a retrial being ordered in the High Court. This

16 Part I Principles of Surgical Audit

makes the process of professional regulation uncertain,

and it is hard to imagine how a doctor who is acquitted

by the GMC, only to have the judgement reversed by the

High Court, would feel.

The media response

The feeding frenzy created when a poor surgical outcome

is reported in the media needs no further description, but

has to be acknowledged as a key element that needs to

be considered and "managed" in the broadest sense. For

reasons possibly derived from self-interest, hospitals are

becoming more accustomed to dealing with the media,

and it would make sense to shelter under any cover that

may so be provided. It seems unlikely that many surgeons

will "win" in a direct encounter with journalists, and

there is ample evidence that neither adequate recompense

nor retractions will follow unjust or misleading reporting.

Bold public assertions are therefore inadvisable.

From the perspective of the hospital

Adverse event reporting and clinical

governance

The response to failures at either end of this spectrum

should be proportionate, but follow a surprisingly similar

pattern. In each case, an apology and explanation is

appropriate. In many institutions, all "adverse events" are

collected into a central database, better to understand the

systemic weaknesses that may have caused or contributed

to the failure. These institutions in their turn share the

database with those governing health care. In the context

of the NHS, the data are collated with the NPSA, who in

turn reports to the Healthcare Commission, providing

the overarching control of "quality" within the national

service.

NHS Act 1977 Practice Direction 2006

This legislation gives a Strategic Health Authority (SHA)

the power to issue an "alert notice," naming an individual

whom it considers poses "a significant risk of harm

to patients, staff, or public, and who may seek work in

the NHS" [20]. A draconian measure, the issue of such

an alert may be requested by the chief executive or executive

board member of an NHS body. The notice is sent

to the National Clinical Assessment Service, the chief

executive of each SHA in England, and the CMO for the

other areas in Great Britain. It may then be sent to any

NHS body that may be approached by the subject of the

notice in search of work in the NHS. Such a notice must

be reviewed at intervals of no more than six months.

Alert letters have been available since 2002 [21], but the

new guidance is a timely reminder of the efforts being

made to protect the public from harm.

What is alarming is that the qualifying criteria for the

issue of an alert could be interpreted by an executive in

an ill-considered way, at a time when they were feeling

vulnerable, perhaps having attracted unwelcome media

interest. Although there is provision for revocation of

a notice, the damage to a clinician's reputation caused

by a false allegation will be impossible fully to retract.

Furthermore, the SHA is required to maintain a record

of revoked notices for five years following revocation.

It seems at least possible that this may disadvantage an

"innocent" revokee who applies for work in the SHA

area during the time period.

There is a requirement for the SHA to satisfy itself of

the evidence supplied by the hospital, supporting the

contention that there is a significant risk of harm. But

it should be noted that it is the risk of harm that has to

be significant, not the degree of harm itself. Should the

climate develop in which unscrupulous health service

managers behave aggressively toward clinical staff, it

could be seen how a poor surgical result, from the practice

of a "troublesome" clinician, could lead to a disproportionate

and unjust outcome. It would be incorrect to

leave the impression that such devastating consequences

are likely to flow from a poor surgical outcome. Such an

outcome would be disproportionate, and in the present

climate, exceedingly unlikely. However, the NHS is going

through an unprecedented transformation of such magnitude

that the "old rules" can no longer be relied upon.

Government thinking appears to be challenging all "core

values," relating to where patients are treated, and what

level of training needs to be achieved as a prerequisite

for treating them. In this climate, it will be prudent to

acknowledge the potential, as well as the probable, consequences

of a poor surgical outcome.

Conclusion

A poor surgical outcome is a miserable business, for

both patient and surgeon. Mercifully, few poor outcomes

lead to litigation, and fewer still to the GMC or criminal

courts. A prompt apology and full explanation will do a

great deal more good than harm in the long term, and

will make it more likely that the relationship between the

surgeon and the family will recover and prosper. In many

parts of the world, including England, there is a search

Chapter 3 The Implications of a Poor Surgical Outcome 17

for alternative modes of recompensing patients who

claim to have suffered harm. This could lead to schemes

that pay out small sums of money on the basis of scant

evidence. Such schemes will flourish if they result in

reducing the national financial burden of clinical negligence

litigation, which is the reason for their existence.

Hospitals may settle such claims irrespective of the effect

on the surgeon's reputation, and we all need to consider

how we will handle that. As governments become more

aware of the voters' focus on the provision of health care,

they are identifying measurable "quality" as a surrogate

for success. In the United Kingdom, this scramble for the

high ground of quality assurance has led government to

provide itself with additional statutory tools of scrutiny

and control. In this environment, careful compliance

with local procedure in the event of an unwanted outcome

becomes increasingly important.

References

1 Available at www.google.com.

2 Good Medical Practice London 2001, para 22.

3 House of Lords create new approach to causation Medical

Law Monitor 2004, Vol. 11, No. 11, p. 1.

4 National Patient Safety Agency. Being Open. London, 2005.

5 Available at www.ombudsman.org.uk.

6 Health Services Commissioners (Amendments) Act 1996.

7 Health Services Commissioners (Amendments) Act 2000.

8 Department of Health. Making Amends: A Consultation

Paper Setting Out Proposals for Reforming the Approach to

Clinical Negligence in the NHS, 2003.

9 NHS Redress Act 2006.

10 Grubb A. Principles of Medical Law. Oxford University Press:

Oxford, p. 323, 2004.

11 Bolam v. Friern HMC [1957] 2 All ER 118, 121.

12 Bolam v. Friern HMC [1957] 2 All ER 118.

13 Bolitho v. City & Hackney HA [1997] 4 All ER 771.

14 Available at www.npsa.nhs.uk/health/resources/root_

cause_analysis.

15 Clinical Negligence Scheme for Trusts, NHS Litigation

Authority.

16 Medical Act 1858.

17 Available at www.gmc-uk.org.

18 Mason JK, Laurie GT. Law and Medical Ethics. Oxford

University Press: Oxford, 1.33, 2006.

19 Donaldson L. Good doctors, Safer patients: Proposals to

strengthen the system to assure and improve the performance

of doctors and to protect the safety of patients. DH: London,

2006.

20 Healthcare Professionals Alert Notices Directions 2006

S 1(3).

21 Health Service Circular 2002/011.

II General Principles

Pediatric Urology: Surgical Complications and Management

Edited by Duncan T. Wilcox, Prasad P. Godbole and Martin A. Koyle

© 2008 Blackwell Publishing Ltd. ISBN: 978-1-405-16268-5

21

The Metabolic and Endocrine

Response to Surgery I

Laura Coates and Joe I. Curry

Introduction

The body's metabolic and endocrine responses to tissue

damage follow similar pathways regardless of whether

that tissue damage is accidental, for example as a result

of trauma, or deliberate, as a result of surgery. There is,

however, an inflammatory component which appears to

vary with the type and size of the insult, and it seems that

both the inflammatory factors and the changes in the

metabolic state are simultaneously necessary to provide

optimal healing and tissue repair. Trauma produces rapid

changes in both hormone release and in the way that

substrates are mobilized for energy, although the exact

mechanisms are still being understood. Among the main

changes that have been measured are increases in the concentrations

of adrenocorticotrophic hormone (ACTH),

cortisol, catecholamines, glucagon, insulin, growth hormone

(GH), and in various substrates and their metabolites,

for example glucose, lactate, and glycerol [1].

The metabolic response

The end objectives of the metabolic response are to provide

optimal cellular respiration and nutrition via:

• Increased oxygen availability

• Mobilization of protein and other body fuels

• Maintenance of fluid and electrolyte balance

• Disposal of the substrates produced by tissue

damage [2].

The end product is the result of a complex interplay

between physiological and biochemical functions, which

appear to show great variation between patients and

bring about rapid changes that do not seem to be totally

predictable [1].

F.D. Moore in 1959 described four phases of the

metabolic response which are still recognized today

[2,3]:

1 The initial phase is related directly to the trauma and

there are immediate physiological, hormonal, and biochemical

changes within the body.

2 This describes the "turning point" when recovery

begins and wound healing begins to mature. The initial

changes noted in the metabolic and endocrine functions

return to normal.

Key points

• After surgery, there is an initial catabolic stage

followed by an anabolic stage.

• The catabolic response consists of secretion of

cortisol, catecholamines, and glucagon.

• The anabolic response includes secretion of

insulin and growth hormone.

• Fluid and electrolyte balance is mainly under the

control of aldosterone and antidiuretic hormone.

• There is an immune component to the metabolic

response that is not fully understood.

• Nonshivering thermogenesis plays a major role

in neonatal thermoregulation.

• Studies comparing laparoscopic and open

surgery have shown variable results with

respect to the endocrine and metabolic

response.

4

Pediatric Urology: Surgical Complications and Management

Edited by Duncan T. Wilcox, Prasad P. Godbole and Martin A. Koyle

© 2008 Blackwell Publishing Ltd. ISBN: 978-1-405-16268-5

22 Part II General Principles

3 This phase usually takes place some time after the

original insult and is characterized by the regaining of

muscle strength. There is protein anabolism and a positive

nitrogen balance.

4 The restoration of fat mass.

This is a more detailed description of what was previously

described as the ebb and flow stages of trauma;

more simply, it describes a catabolic ("hypometabolic")

stage followed by an anabolic ("hypermetabolic") stage.

The catabolic response

Catabolic responses within the body are concerned with

the breakdown of organic polymers and molecules into

simpler molecules for the purpose of releasing energy.

The catabolic hormones principally released as part of

the response to trauma are cortisol, the catecholamines,

and glucagon; their net result is to raise the basal metabolic

rate, increase glucose concentrations, increase circulating

insulin, and increase nitrogen excretion. The

effect of these catabolic hormones is not only to increase

the glucose concentration, but also to utilize less glucose

for a given level of insulin.

Cortisol

Cortisol is a steroid hormone that accounts for approximately

95% of the glucocorticoid activity within the

body. It is produced via the hypothalamo-pituitary axis

(Figure 4.1).

Corticotrophin-releasing factor (CRF) is released from

the hypothalamus under the influence of the body's circadian

rhythm, stress, and the plasma levels of corticosteroids

under a negative feedback system. CRF travels via

the portal system to the anterior pituitary where it stimulates

corticotrophs to produce ACTH, which in turn

stimulates the adrenal cortex to produce cortisol.

The functions of cortisol are:

• Protein catabolism, primarily in muscle with the result

of increasing plasma amino acids

• Promotion of gluconeogenesis

• Stimulating lipolysis

• Increasing sensitivity to vasoconstrictors, thereby raising

blood pressure

• Anti-inflammatory and anti-immune effects. Glucocorticoids

in general:

- Have antihistamine effects by reducing mast cell

concentration

- Stabilize lysosomal membranes and thus slow the

release of destructive enzymes

- Decrease capillary permeability

- Depress phagocytosis [4].

Immediately after trauma, ACTH levels increase rapidly

and have been found to be far higher than those required

for maximal stimulation of the adrenal cortex [2],

although these excessive levels appear to be only transient

[5]. Plasma cortisol levels, however, remain high

for 2-3 days and cortisol breakdown products continue

to be excreted in the urine for many days post-trauma.

Catecholamines

The major catecholamines, adrenaline and noradrenaline,

are part of a group of neurotransmitters known as biogenic

amines, which are modified decarboxylated amino

acids. The axis through which they are produced and regulated

is known as the sympatho-adrenal axis (Figure 4.2).

They are the two principal hormones synthesized by the

adrenal medulla, and are responsible for the sympathomimetic

component of the "fight-or-flight" response.

The hypothalamus sends impulses to the sympathetic

preganglionic neurons, which in turn stimulate the production

of adrenaline and noradrenaline from the chromaffin

cells of the adrenal medulla. The catecholamines

then target their α- and β-adrenergic receptors on effectors

Stress

Stimulates

Hypothalamus

Produces

CRF

Travels via portal system to

Anterior pituitary

Produces

ACTH Negative

feedback

Travels to

Adrenal cortex

Produces

Cortisol

Figure 4.1 Regulation of cortisol secretion.

Chapter 4 The Metabolic and Endocrine Response to Surgery I 23

innervated by sympathetic postganglionic axons. On the

whole, α-receptors are excitatory, while β-receptors are

variable in terms of their responses. Adrenaline stimulates

both α- and β-adrenergic receptors and at low

administered doses results in tachycardia with a simultaneous

fall in systemic vascular resistance, mainly

from its β effects. At higher doses, α effects predominate

and vasoconstriction occurs. This has the effect

of increasing perfusion pressure and thus maintaining

renal blood flow and urine output, but only to a point -

at much higher doses, cardiac output falls, and further

vasoconstriction causes a decrease in renal perfusion.

Adrenaline is a highly effective promoter of gluconeogenesis

by its action on hepatic and muscle phosphorylases

[6]. The catecholamines inhibit insulin release and

reduce peripheral glucose uptake, thus further increasing

the plasma glucose and leading to the characteristic postsurgery

hyperglycemia. Both adrenaline and noradrenaline

increase free fatty acid mobilization and elevate

oxygen consumption [2]. Plasma levels of the catecholamines

are found to be raised immediately post stress

and the levels are directly proportional to the extent of

trauma. Again, as with cortisol, plasma levels are raised

for a relatively short length of time but urinary excretion

has been noted for a much longer period [7].

Glucagon

Glucagon is a peptide hormone produced by the α cells

of the pancreatic islets. β-adrenergic activity stimulates

these α cells to produce glucagon immediately posttrauma.

The effects of glucagon are in direct contrast to

the effects of insulin (Figure 4.3).

It raises plasma glucose levels by speeding up hepatic

glycogenolysis and gluconeogenesis.

Glucagon release is also stimulated by:

• Increased sympathetic autonomic nervous system

(ANS) activity, e.g. during exercise or trauma

• An increase in plasma amino acid concentration if the

plasma glucose is low, e.g. following a protein-only meal

and its release is inhibited by both insulin and somatostatin

[4].

The anabolic response

The reactions within the body that combine simple

molecules to form complex polymers are known as the

anabolic reactions. Anabolism uses the energy created

from catabolism to build the structural and functional

Hypothalamus

Stimulates

Sympathetic pre-ganglionic neurons

Stimulate

Chromaffin cells of adrenal medulla

Produce

Adrenaline and Noradrenaline

Effect

- and -receptors

Figure 4.2 Production of catecholamines.

↓ Blood glucose ↑ Blood glucose

Inhibits

↑ Glucagon ↑ Insulin

↑ Glycogenolysis ↑ Glyconeogenesis ↑ Glycogenesis ↑ Lipogenesis

↑ Blood glucose ↓ Blood glucose

↑ Protein

synthesis

Figure 4.3 Regulation of glucagon and insulin secretion.

24 Part II General Principles

components of the body, for example the synthesis of

proteins from amino acids or glycogen from glucose

monomers. Insulin and GH are the two main hormones

responsible for these synthesis reactions within the

human body.

Insulin

Insulin is another peptide hormone produced by the

pancreas - this time, from the β cells, which make up

70% of the total number of pancreatic islet cells. Insulin

can be seen in Figure 4.3 to have contrasting actions to

glucagon by ultimately decreasing blood glucose. Its

other actions are to:

• Encourage diffusion of glucose into cells

• Accelerate glycogenesis

• Increase amino acid uptake and in turn increase protein

synthesis

• Encourage lipogenesis

• Slow glycogenolysis and gluconeogenesis [4].

Insulin release is mainly stimulated by a rising blood

glucose level, although many other substances also influence

and regulate the plasma levels of insulin:

• Acetylcholine, via the vagal innervation of the

pancreas

• Arginine and leucine - two amino acids

• Glucagon

• Gastric inhibitory peptide (GIP), a peptide released by

the enteroendocrine cells of the small bowel in response

to a postprandial glucose load

• GH and ACTH (indirectly) due to their effect of raising

plasma glucose levels [4].

It can be seen that although glucagon stimulates insulin

release, there is no reciprocal increase; insulin actually

suppresses the release of glucagon. As blood glucose levels

drop, and levels of insulin simultaneously decrease,

more glucagon is released from the pancreas, which then

increases blood glucose and stimulates once more the

release of insulin, which then causes the glucose level to

fall and the cycle begins once more.

Initially after trauma insulin levels are decreased

[8], but these rise again after the first 24 h as the initial

response to trauma stabilizes [9].

Growth hormone

GH is released from the anterior pituitary under the

influence of growth hormone releasing hormone

(GHRH) which is secreted into the portal system from

the hypothalamus. Its release is inhibited by somatostatin,

also known as growth hormone releasing inhibitory

peptide (GHRIP). GH stimulates the production of

insulin-like growth factor-1 (IGF-1) within the liver - and

it is this which stimulates body and tissue growth in

humans. The effects of this process are as follows:

• To increase protein and collagen synthesis

• To increase the basal metabolic rate

• To encourage the preservation of anabolic substrates,

namely calcium, phosphorus, and nitrogen

• To increase fat oxidation

• To counter the effects of insulin.

The release of GH in the healthy individual is mainly

nocturnal and pulsatile although injury and stress serve

to stimulate its release, while hyperglycemia counteracts

this and leads to suppression of the hormone.

GH levels have been shown to rise following trauma and

gradually revert to normal within a few days. IGF-1 levels,

similarly, decrease in 4-5 days following trauma [10].

Fluid balance

Sodium

Just as the glucocorticoids produced in the adrenal cortex

influence glucose homeostasis in the body, so the mineralocorticoids

influence mineral homeostasis; or more

specifically, fluid and electrolyte balance. Approximately

95% of the mineralocorticoid activity is due to aldosterone,

a steroid hormone manufactured in the zona

glomerulosa of the adrenal cortex. This hormone targets

the renal tubules to stimulate the reabsorption of

sodium ions (Na). This in turn increases reabsorption

of chloride and bicarbonate ions and leads to the retention

of water. Aldosterone also promotes the excretion

of potassium ions (K) and hydrogen ions (H) in the

urine, thus helping to correct acidosis and restore pH.

Aldosterone control is under the influence of the

renin-angiotensin system (Figure 4.4).

Hypotension, dehydration, or Na loss stimulates the

release of renin from the juxtaglomerular cells of the

kidney. Angiotensinogen, meanwhile, is being produced

in the liver and is converted by renin into angiotensin I,

which then is further converted in the lung capillary beds

into angiotensin II under the influence of angiotensin

converting enzyme (ACE). This has two main effects:

1 To stimulate the adrenal cortex to secrete aldosterone.

This increases tubular reabsorption of Na and thus

increases water reabsorption, leading to a restoration of

blood volume.

2 To act on the smooth muscle in arteriolar walls, leading

to the vasoconstriction of arterioles, which in turn

leads to an increase in blood pressure.

Chapter 4 The Metabolic and Endocrine Response to Surgery I 25

Aldosterone levels are known to increase rapidly postsurgery

and an impairment in Na excretion has been

shown to last for up to a week [11].

Atrial natriuretic peptide (ANP) has also been implicated

in the regulation of sodium. It is a hormone that is

secreted in both cardiac atria in response to atrial stretching

(i.e. on "overfilling" or overcompensation of a low

blood volume) and it promotes excretion of both water

and sodium. It also suppresses the secretion of antidiuretic

hormone (ADH; see below), renin, and aldosterone.

Water balance

ADH is a neuropeptide which is synthesized in the

hypothalamus and stored in the posterior pituitary. Its

main function is to retain body water and prevent water

losses both in the urine and as sweat. Its production is

regulated by osmoreceptors in the hypothalamus, which

detect dehydration or hypotension. An increase in the

plasma level of ADH has three main actions, all of which

serve to increase body water:

• Renal tubules retain more water

• Sweat glands decrease output and sweating is

diminished

• Arterioles constrict.

Levels of ADH increase following surgery, and administration

of morphine, acetylcholine, and nicotine have all

been shown to increase ADH production, leading to fluid

retention and increased tissue perfusion. This brings

with it the potential risk of fluid overload and edema,

necessitating careful postoperative fluid monitoring.

↓ BP

Dehydration

Na loss

Stimulate

Juxtaglomerular

cells

Produce

Liver ↑ Renin

Produces Converts

Angiotensinogen Angiotensin I

Converted to

Angiotensin II

Stimulates Stimulates

Adrenal cortex Vasoconstriction

Produces

↑ Aldosterone Negative

feedback

↑ Na+ reabsorption

↑ Blood volume

↑ BP

Figure 4.4 Regulation of aldosterone secretion.

26 Part II General Principles

Protein metabolism

After trauma of any kind in adults, there is an increase in

the basal metabolic rate, breakdown of body protein, and

increased nitrogen excretion. The result is a net loss of

protein. In the latter stage of the metabolic response to

trauma, however, protein anabolism takes over and new

protein is synthesized for repair and tissue growth. There

is, however, some debate over whether children show the

same response as adults; some studies have shown that

children and infants show a lack of catabolism following

surgery and no increase in protein turnover [12]. Others

have shown an increase in protein turnover specifically

in neonates on life support [13]. Surgery and its complications,

along with sepsis, are the most common causes

of protein loss in hospital, otherwise known as proteinenergy

malnutrition. This term is used to describe the

weight loss that occurs as a consequence of either:

• Poor enteral intake secondary to anorexia secondary to

the underlying condition

• Increased catabolism, e.g. due to sepsis

• Tumor necrosis factor (TNF) in patients with cancer

• Malabsorption [14].

Nitrogen balance is used as a measure of net protein

metabolism. Simplified, this is nitrogen input minus total

urinary nitrogen excretion, although the actual results

can be rather more difficult to calculate. An increase in

urinary urea and ammonia excretion occurs following

surgery and negative nitrogen balances are usually seen

as a result [2]. This increase in urinary excretion is the

effect of protein breakdown in:

• Muscle

• Breakdown of dead and damaged cells

• Hematoma and blood cells.

Of these, muscle breakdown accounts for the majority of

the protein loss.

In the starvation state, the small amount of glycogen

that is stored in the liver is utilized usually in the first

24 h. Gluconeogenesis is therefore necessary to maintain

a supply of glucose for energy. Pyruvate, lactate, glycerol,

and amino acids (mainly alanine and glutamine) are

the main sources of gluconeogenesis. The majority of

protein breakdown occurs in muscle which leads to the

inevitable loss of muscle bulk in chronic starvation [14].

Stored triglycerides are also hydrolyzed to glycerol (used

for gluconeogenesis as above) and to fatty acids that may

be oxidized further to form ketone bodies.

In starvation, adaptation occurs and the basal metabolic

rate decreases in an attempt to conserve fuel stores.

The brain's choice of substrate changes from glucose to

ketone bodies and hepatic gluconeogenesis decreases.

Following surgery or trauma, however, that adaptation

does not take place; and, coupled with the increase in

catecholamines and glucocorticoids, gluconeogenesis

and protein breakdown continue in an attempt to provide

enough energy to overcome the stress. An enzymatic

pathway called the ubiquitin-proteasome pathway

(which is a selective degrader of intracellular proteins) is

stimulated by these glucocorticoids and cytokines and is

responsible for accelerating protein breakdown in muscle

in many disease processes [14].

The immune response

There is an immune component to the postsurgery

response that is still being understood. It has long been

known that cytokines are mediators of the acute phase

stress response and it is now known that interleukins

and TNF play an additional role in the regulation of the

metabolic response.

Interleukins, especially IL-1 and IL-6, stimulate hepatic

lipogenesis and IL-1 has an additional role in hepatic

gluconeogenesis and in promoting muscle proteolysis

[15]. IL-6 is a marker of the stress response in neonates

and increases in proportion to the magnitude of the

operative insult [16].

TNF increases glucose transport and also promotes

hepatic lipogenesis and muscle proteolysis along with IL-1.

It also inhibits lipoprotein lipase and has been shown to

be the main cause of cachexia in patients with cancer. It

has also been postulated that the interleukins and TNF

may show a synergistic effect in regulating the metabolic

response to trauma [17].

Thermoregulation

Neonates and infants have much more difficulty in regulating

and maintaining their own body temperature

than older children and adults. This is partly due to an

increased surface area/body mass ratio, and partly to do

with the make up of their fuel stores. Adults adapt to cold

by shivering and vasoconstriction, and conversely to heat

by sweating and vasodilation. Newborn babies are unable

to mount a shivering response to cold, although they do

demonstrate an intact vasoconstriction response [18].

Infants have a particular way of generating heat in the

absence of shivering, by way of a specific type of adipose

tissue called brown fat which dissipates energy in the form

Chapter 4 The Metabolic and Endocrine Response to Surgery I 27

of heat and generates so-called nonshivering thermogenesis.

This doubles the normal metabolic rate of the infant,

and is accompanied by a threefold rise in the plasma concentration

of noradrenaline [19]. During environmental

cooling, blood supply to the brown fat increases and heat

is generated by the mitochondria within the fat cells [15].

Several studies have shown that under anesthesia (particularly

fentanyl), infants are unable to mount a nonshivering

thermogenic response which can lead to a sudden

increase in their metabolic rate when anesthetic administration

is terminated [19]. This reinforces the importance

of maintaining a warm environmental temperature both

in theater and postoperatively, and of making sure that

the stresses on the child are as low as possible in terms of

maintaining body temperature.

Laparoscopy versus open surgery

Laparoscopic surgery is increasingly used for a wide variety

of operations, associated as it is with reportedly shorter

hospital stays, less analgesic requirements postoperatively,

and a more rapid postoperative recovery [20-22].

Several studies have investigated whether the body's

normal metabolic response to surgery alters in cases

where laparoscopy is used as opposed to open surgery.

Adult studies have demonstrated that laparoscopy is associated

with a small decrease in the inflammatory response

with little or no difference in the metabolic response [22,

23]. Conversely, McHoney et al. in 2006 found that laparoscopy

in children is associated with an intraoperative

hypermetabolic response, where open surgery has no corresponding

rise in metabolic rate [24]. The oxygen consumption

(VO2) in children during surgery was used as

a marker of metabolic rate and it was found to rise steadily

throughout the duration of the laparoscopic surgery.

There was also a corresponding rise in core temperature,

despite using unwarmed CO2 for insufflation.

On reviewing the literature for laparoscopic surgery in

adults, Vittimberga et al. stated that the body's response

to laparoscopy is one of "lesser immune activation"

rather than immunosuppression [25]. They also discussed

how laparoscopic surgery has been shown to:

• Decrease C-reactive protein (CRP) in cases of

cholecystectomy

• Decrease IL-6 concentrations after laparoscopic

procedures.

This has also been demonstrated in a study looking

specifically at cases of laparoscopy in newborn infants.

The operations studied included nephrectomies and

salpingo-oophorectomies, and corroborated the evidence

for a significant decrease in the acute phase response in

laparoscopic surgery in infants

• Increase histamine response

• Decrease T-cell function

• Decrease postoperative immunosuppression.

There appears to be, however, ongoing debate regarding

the changes listed above. Bozkurt et al. [26] studied

IL-6 concentrations during emergency laparoscopy and

laparotomy in children and found no difference between

the two groups. They also undertook measurements of

blood prolactin, cortisol, glucose, insulin, lactate, and

adrenaline and found that the rise in these substances

was equal in both groups [26].

A further study looking at open versus laparoscopic

Nissen's fundoplication in children also did not show any

difference in concentrations of TNF or IL-1 between the

two groups. There was, however, a slight increase in postoperative

immune suppression in the open group [27].

This does suggest that the metabolic response is highly

variable. The fact that so many studies have been undertaken

and yet show differing results does seem to imply

that the metabolic response to surgery, while following a

similar pathway each time, is not necessarily predictable

or quantifiable in different children undergoing different

operations.

References

1 Barton RN, Cocks RA, Doyle MO, Chambers H. Time course

of the early pituitary-adrenal and metabolic responses to

accidental injury. J Trauma 1995;39:888-94.

2 Burnand KG, Young AE. The new Aird's companion in surgical

studies. Churchill Livingstone: London, 1992.

3 Moore FD. Metabolic Care of the Surgical Patient. WB

Saunders: Philadelphia, 1959.

4 Tortora GJ, Grabowski SR. Principles of Anatomy and

Physiology. Harper Collins, Newyork, 1996.

5 Cooper CE, Nelson DH. ACTH levels in plasma in preoperative

and surgically stressed patients. J Clin Invest 1962;

41:1599-1605.

6 Barton RN. Neuroendocrine mobilization of body fuels

after injury. Brit Med Bull 1985;41:218-25.

7 Walker WF, Johnston IDA. The Metabolic Basis for Surgical

Care. Heinemann: London, 1971.

8 Traynor C, Hall GM. Endocrine and metabolic changes

during surgery: anaesthetic implications. Brit J Anaesth

1981;53:153-60.

9 Stoner HB, Frayn KN, Barton RN, Threlfall CJ, Little RA.

The relationships between plasma substrates and hormones

and the severity of injury in 277 recently injured patients.

Clin Sci 1979;56:563-73.

28 Part II General Principles

10 Frayn KN, Price DA, Maycock PF, Carroll SM. Plasma

somatomedin activity after injury in man and its relationship

to other hormonal and metabolic changes. Clin

Endocrinol 1984;20:179-87.

11 Cochrane JPS. The aldosterone response to surgery and the

relationship of this response to postopertive sodium retention.

Brit J Surg 1978;65:744-7.

12 Powis M, Smith K, Rennie M, Halliday D, Pierro A. Effect of

major abdominal operations on energy and protein metabolism

in infants and children. J Paediatr Surg 1998;33:49-53.

13 Keshen TH, Miller RG, Jahoor F, Jaksic T. Stable isotope

quantification of protein metabolism and energy expenditure

in neonates on pre- and post-extracorporeal life support.

J Paediatr Surg 1997;32:958-63.

14 Kumar P, Clark M (Editors). Clinical Medicine. WB

Saunders, London, 1998.

15 Pierro A. Metabolic response to neonatal surgery. Curr Opin

Paediatr 1999;11:230-6.

16 Jones MO, Pierro A, Hashim IA, Shenkin A, Lloyd DA.

Postoperative changes in resting energy expenditure and

interleukin-6 in infants. Brit J Surg 1994;81:536-8.

17 Hill AG, Hill GL. Metabolic response to severe injury. Brit J

Surg 1998;85:884-90.

18 Plattner O, Semsroth M, Sessler D, Papousek A, Klasen C,

Wagner O. Lack of nonshivering thermogenesis in infants

anesthetized with fentanyl and propofol. Anesthesiology

1997;86:772-7.

19 Dawkins MJR, Scopes JW. Non-shivering thermogenesis and

brown adipose tissue in the human new-born infant. Nature

1965;206:201-2.

20 Berggren U, Gordh T, Grama D, Haglund U, Rastad J,

Arvidsson D. Laparoscopic versus open cholecystectomy:

Hospitalisation, sick leave, analgesia and trauma responses.

Brit J Surg 1994; 81:1362-5.

21 Joris J, Cigarini I, Legrand M, Jacquet N, De Groote D,

Franchimont P et al. Metabolic and respiratory changes

after cholecystectomy performed via laparotomy or laparoscopy.

Brit J Anaesth 1992;69:341-5.

22 Kehlet H. Surgical stress response: Does endoscopic surgery

confer an advantage? World J Surg 1999;23:801-7.

23 Gupta A, Watson DI. Effect of laparoscopy on immune

function. Brit J Surg 2001;88:1296-1306.

24 McHoney MC, Corizia L, Eaton S, Wade A, Spitz L,

Drake DP et al. Laparoscopic surgery in children is associated

with an intraoperative hypermetabolic response. Surg

Endosc 2006;20:452-7.

25 Vittimberga FJ, Foley DP, Meyers WC, Callery MP.

Laparoscopic surgery and the systemic immune response.

Ann Surg 1998;227:326-4.

26 Bozkurt P, Kaya G, Altintas F, Yeker Y, Hacibekiroglu M,

Emir H et al. Systemic stress response during operations for

acute abdominal pain performed via laparoscopy or laparotomy

in children. Anaesthesia 2000;55:5-9.

27 McHoney M, Eaton S, Wade A, Klein N, Stefanutti G,

Booth C et al. Inflammatory response in children after

laparoscopic vs open Nissen fundoplication: Randomised

controlled trial. J Paediatr Surg 2005;40:908-13.

29

The Metabolic and Endocrine

Response to Surgery II:

Management

Benjamin P. Wisner, Douglas Ford and Martin A. Koyle

Introduction

The previous chapter has described the various metabolic

and endocrine responses to surgical trauma. This

chapter will focus on diagnosis and management of

metabolic and endocrine derangements in the pediatric

urologic patient.

Developmental changes in renal function

Compared with the mature kidney, the neonatal kidney

has impaired concentrating ability as well as lessened

abilities for tubular reabsorption of sodium and secretion

of potassium and hydrogen. The neonatal kidney also

receives a lesser proportion of the cardiac output, which

contributes to lower glomerular filtration rate (GFR) [1].

GFR increases markedly over the first 3 months of life,

with the transition to adult levels by 2 years of age [2].

Due to impaired countercurrent exchange, maximal neonatal

urine concentration is 500-700 mOsm/kg.

Routine fluid and electrolyte therapy

The daily water requirements of infants and children

are based on estimated caloric expenditures [3]. In a

24-h period, approximately 100 ml water is needed per

100 kcal/kg of energy expended. Sodium and chloride

replacement is required at 2-3 mEq/100 ml water per

day, and potassium replacement is required at 1-2 mEq/

100 ml water per day. Urine is the main source of electrolyte

loss; however, in the postsurgical patient, losses from

GI sources (e.g. NG suction) may be substantial [4].

Traditional fluid replacement in infants and children with

hypotonic fluid has been based on the above-calculated

requirements [5].

Disorders of sodium and water balance

Total body water (TBW) consists of both intracellular

and extracellular fluid (ECF). ECF makes up approximately

45% of TBW in neonates, this percentage

decreases rapidly in the first year of life and then gradually

throughout childhood [7]. Renal sodium reabsorption

occurs primarily in the proximal tubule via the

Na-H antiporter on a gradient generated by Na-

K-ATPase. In the collecting duct, sodium resorbtion is

Key points

• The neonatal kidney has lower glomerular

filtration rate and impaired concentrating ability

and electrolyte handling.

• Hypotonic fluid administration as well as

increased circulating antidiuretic hormone is

common cause of postoperative hyponatremia.

• Most postobstructive diuresis is physiologic.

• Bowel segments in continuity with the urinary

tract predispose to a number of short- and

long-term metabolic consequences.

5

Pediatric Urology: Surgical Complications and Management

Edited by Duncan T. Wilcox, Prasad P. Godbole and Martin A. Koyle

© 2008 Blackwell Publishing Ltd. ISBN: 978-1-405-16268-5

30 Part II General Principles

regulated mainly by aldosterone and sodium intake, and

water handling by ADH [8].

Hyponatremia

Postoperative hyponatremia is a relatively common

phenomenon, occurring in approximately 4-20% of

patients [5,6,9]. Overall, hyponatremia is the most common

electrolyte abnormality observed in hospitalized

children [10]. There are many etiologies of postoperative

hyponatremia; however, the most common is administration

of hypotonic fluid [9]. The hyponatremic effect

of free water excess is compounded by an excess of ADH

in the first 72 h postoperatively [11-13]. Drugs given in

the postoperative period may also contribute to sodium

disturbance. Common medications given in children

include opioids, NSAIDs, and acetaminophen. Opioids

enhance ADH action via mu-receptors [14]. NSAIDs and

acetaminophen are known to potentiate water retention

by inhibiting prostaglandin synthesis [8]. Vomiting or

nasogastric suction can also contribute to hyponatremia.

In a retrospective study of hyponatremia in hospitalized

children age 1 month to 18 years, Wattad et al.

found that 97.5% of cases fell into the categories of mild

or moderate hyponatremia (Serum Na 121-129), and

only 2.5% of patients had severe hyponatremia (Serum

Na 120). Nine percent of patients with mild and 48%

of patients with moderate hyponatremia were symptomatic,

most commonly with lethargy and irritability.

Two percent of children with mild hyponatremia, 3%

with moderate hyponatremia, and 100% with severe

hyponatremia had demonstrable neurologic deficits,

and, although these children had severe comorbid illness,

most had persistence of deficits on discharge from

the hospital [15]. In a retrospective series and review of

the literature, Medani determined that the average serum

sodium in children presenting with seizures was 118

4.3 mEq/l [16]. Hyponatremia has been variably linked

to poor outcomes in children, with mortality rates ranging

from 8.4% [17] to 12% [15]. It appears that symptomatic

hyponatremia is more likely to result in symptoms

attributable directly to hyponatremia (e.g. seizures),

whereas sequelae such as demyelination are related

to rapid correction (2 mEq/l/h or 20 mEq/l/24 h)

and are more likely to occur in patients with chronic

hyponatremia [18-20].

The most feared complication of correction of

hyponatremia is central pontine myelinolysis (CPM)

[21], also termed osmotic demyelination syndrome [22].

In children, CPM has been described in hyponatremic

patients; however, these children typically have additional

comorbid factors such as liver disease. In a single

institution series, only 9/17 cases of CPM occurred in

hyponatremic patients, whereas 5/17 had normal sodium

and 3/17 had hypernatremia [10]. Most hyponatremia in

infants and children is acute in nature, and the benefits

of rapid correction to alleviate seizures typically outweighs

the risks of demyelination [16, 23, 24].

The first step in the evaluation of hyponatremia is a

clinical assessment of volume status. This can be done

by evaluation of mucous membranes, urine output,

body weight, orthostatic blood pressure, and skin turgor.

Next, the urinary sodium concentration allows for

determination of whether or not the renal response to

hyponatremia is appropriate. This is especially useful in

the setting of hypovolemic hyponatremia, in which the

normal kidney avidly retains sodium, therefore leading to

a low urinary sodium concentration (U[Na] 20 mEq/l).

Based on clinical assessment and urinary sodium concentration,

the patient can be classified according to the

diagram in Figure 5.1. When treating hyponatremia,

the importance of serial sodium monitoring cannot be

overemphasized.

Hypovolemic hyponatremia

In the pediatric urologic patient, most postoperative

hypovolemic hyponatremia is secondary to extrarenal

losses. Replacement of sodium and water is required, and

isotonic saline is generally appropriate for initial replacement.

Once the ECF has been repleted, the stimulus for

AVP decreases and rapid correction of hyponatremia

occurs via excretion of dilute urine. For this reason, use

of hypotonic fluid may be appropriate after repletion

of the extracellular fluid volume [24-26]. The sodium

deficit calculation may be a useful adjunct to therapy;

however, this formula will underestimate the anticipated

correction in patients with extrarenal sodium losses such

as NG suction or high fever. It should therefore be used

only as a guide and is not a substitute for monitoring

plasma sodium during deficit replacement.

Sodium deficit TBW (kg) (desired [Na] mEq/l -

actual [Na] mEq/l) where TBW is estimated as lean body

weight (kg) times 0.5 kg1 for women, 0.6 kg1 for men,

and 0.6 kg1 for children [24]. The result of this formula

is the number of mEq of sodium needed to replace the

deficit. This can be given over an appropriate time course

to prevent overly rapid correction.

Chapter 5 The Metabolic and Endocrine Response to Surgery II: Management 31

Euvolemic hyponatremia

Euvolemic hyponatremia in the pediatric patient can

be due to stress, vasopressin administration (for von

Willebrand's disease), drugs, glucocorticoid deficiency,

hypothyroidism, or SIADH. Should a child have acute

(48 h duration) severely symptomatic euvolemic

hyponatremia, correction with hypertonic saline (3%) at

1-2 ml/kg/h plus lasix administration can be undertaken

[23]. The goal of therapy in adolescents and adults is to

raise serum sodium at 1-2 mEq/l/h until seizures subside

[24]. Additional correction should take place at a rate

not to exceed 0.33-0.5 mEq/l/h or 8-12 mEq/l in a 24-h

period [22,24,27]. In infants and children with seizures,

however, more rapid correction may be appropriate if

the clinician is confident of an acute symptomatic disturbance

in sodium [23].

Asymptomatic euvolemic hyponatremia is treated with

fluid restriction to produce a negative free water balance.

Restriction to 50% of normal daily goals may be required

in some instances [24]. Once again, gradual correction is

the goal unless the patient is acutely symptomatic.

Hypervolemic hyponatremia

Hypervolemic hyponatremia is common in infants in

children due to water intoxication. It may also be caused

by chronic renal or cardiac disease, but these are much

less common. Treatment consists of fluid restriction in

the asymptomatic patient. In the child with CNS symptoms

such as lethargy or seizures, acute correction with

3% saline 5 ml/kg over 10-30 min is an effective strategy,

and would tend to raise serum sodium by approximately

5 mEq/l [10,23] (Figure 5.2).

Hypernatremia

Hypernatremia in the pediatric patient arises from excess

sodium intake, free water deficit (diabetes insipidus,

fever, radiant warmers, phototherapy), or combined

sodium and water deficit (postobstructive diuresis, emesis,

NG suction, diarrhea) [28]. Hypernatremia can lead

to cerebral hemorrhage, and the sodium level should be

corrected gradually at a rate not 12 mEq/l/24 h. Water

deficit can be calculated by the following formula:

Water deficit 0.6 (body weight (kg))

(1 (145/current [Na]))

Replacement can be undertaken with normal saline,

½, or ¼ normal saline, depending upon the degree of

water deficit. Correction should be monitored with serial

plasma sodium measurements.

Disorders of potassium balance

The vast majority of the body's potassium stores are

intracellular. The kidney is responsible for secretion of

Figure 5.1 Classification of hyponatremia and causes.

Hyponatremia

(Serum Na 130 mEq/l)

Clinical assessment of volume

status

Hypovolemic Euvolemic Hypervolemic

Water intoxication

Renal disease

Cardiac disease

SIADH

Vasopressin use

Postoperative state

Drugs

Glucocorticoid deficiency

Hypothyroidism

Extrarenal losses

Vomiting/NG suction

Diarrhea

Fever

Fluid sequestration (ileus)

Renal losses

Diuretic use

Salt wasting nephropathy

32 Part II General Principles

90% of the daily potassium intake [29]. Potassium is

absorbed in the proximal tubule and secreted in the distal

nephron under the influence of mineralocorticoid.

The immature kidney appears to be less efficient in the

secretion of potassium load, making the infant more

susceptible to hyperkalemia in the setting of handling a

potassium load [30].

Hypokalemia

Hypokalemia in the postoperative patient is commonly

iatrogenic and is frequently related to nasogastric suction

or loop diuretic use. Additional causes are vomiting,

diarrhea, insulin administration, and inadequate

potassium replacement or intake. Hyperaldosteronism

and renal tubular acidosis are also potential causes

of hypokalemia. In hyperaldosteronism, hypokalemia

is also accompanied by hypertension and alkalosis,

and in renal tubular acidosis, a hyperchloremic

metabolic acidosis is also present. Symptoms of

hypokalemia typically consist of muscle cramps and

weakness, but can also include gastrointestinal symptoms

with ileus and paresthesias. Treatment of hypokalemia

can be accomplished with either intravenous

or oral potassium chloride. Should an underlying

metabolic abnormality such as renal tubular acidosis

be suspected, appropriate evaluation should follow.

Hyperkalemia

Hyperkalemia is a potentially life-threatening condition.

Mild hyperkalemia may be asymptomatic; however, symptoms

of severe hyperkalemia include characteristic ECG

changes, muscle cramps, arrhythmia, and cardiac arrest.

Hyperkalemia in infants and children is frequently due to

hemolysis, and secondary to the high prevalence of capillary

phlebotomy. Another source of fictitious hyperkalemia

is usage of IV sites running potassium-containing

fluids. Causes of true hyperkalemia include renal failure,

bilateral high-grade obstruction, and release of intracellular

potassium from destroyed cells, such as crush injury,

tumor lysis, or extensive hemolysis. Extracellular shift of

potassium due to extreme acidosis, insulin deficiency, or

impaired renal secretion such as adrenal insufficiency can

also lead to hyperkalemia.

If hemolysis is suspected as the cause of hyperkalemia,

repeating the potassium level from a separate venipuncture

site is helpful. Potassium should be removed from

IV fluids. ECG is helpful in evaluating for cardiac toxicity.

Calcium administration may help to stabilize the

myocardium from the arrhythmogenic effects of potassium.

Excess potassium can be temporarily shifted to an

intracellular location by administration of insulin with

glucose or by β2-agonist inhalers. These agents increase

the Na-K-ATPase activity. Loop diuretics enhance

potassium secretion and may be useful in therapy. Oral

binding solutions such as sodium polystyrene sulfonate

Figure 5.2 Treatment of hyponatremia.

NS, normal saline; ECF, extracellular fluid.

Hyponatremia

Hypovolemic Euvolemic Hypervolemic

If seizures:

3% NS 5 ml/kg IV over 10-15 min

Consider lasix

Fluid restriction

Monitor serum medium

Target correction 0.33-0.5 mEq/l/h

Consider loop diueric

Calculate Na deficit

Fluid restriction

Monitor serum medium

Target correction 0.33-0.5 mEq/l/h

If seizures:

3% NS 5 ml/kg IV over 10-30 min

Consider lasix

Replace deficit with NS

Monitor serum medium

Target correction 0.33-0.5 mEq/l/h

Change to ¼ or ½ NS once

ECF repleted

Chapter 5 The Metabolic and Endocrine Response to Surgery II: Management 33

(Kayexalate) require a functional GI tract but can be a

useful adjunct in therapy. Sodium polystyrene sulfonate

should not be given orally in neonates due to the risk of

gastrointestinal hemorrhage or colonic necrosis.

Postobstructive diuresis

Postobstructive diuresis can be encountered by the pediatric

urologist in a variety of settings. These range from

posterior urethral valves to stones to postoperative mishaps

such as catheter occlusion, urinary retention after

reimplantation, obstruction of a catheterizable stoma,

or, rarely, obstructing malignancy such as rhabdomyosarcoma.

Postobstructive diuresis can even be encountered

in the setting of unilateral obstruction, such as an

obstructing ureteral calculus [31,32]. Postobstructive

diuresis is caused by many factors. Acutely, there is an

early increase in GFR followed by subsequent decrease

due to afferent arteriolar constriction [33]. Urinary

obstruction also leads to an impairment of sodium and

free water reabsorption [34-36]. Altered tubuloglomerular

feedback, impaired ADH response, and ANP accumulation

have also been implicated [37-39].

The diuresis observed after relief of obstruction is

mainly due to the excretion of retained water and solutes

[40]. For this reason, aggressive fluid resuscitation

is not typically necessary. Due to the impaired concentrating

ability and obligatory natriuresis seen as a result

of obstruction, some replacement of salt wand water is

warranted. In unilateral obstruction, the presence of a

normal contralateral kidney typically mitigates natriuresis

[41]. Most postobstructive diuresis is benign and

corrects within 24-48 h. Approximately 10-20% of these

patients, however, will have continued natiuresis that

can lead to profound dehydration [42-44]. Patients with

signs of volume overload and severe renal impairment

may be at higher risk of developing prolonged natiuresis

[45-46]. Gradual decompression does not appear to alter

the course of postobstructive diuresis [47].

Interposed bowel segments

The interposition of bowel segments into the urinary

tract, such as with bladder augmentation, can create

a unique milleu of metabolic derangements. These

problems include diarrhea, vitamin malabsorption,

and electrolyte abnormalities. Ileal or colonic interposition

can result in hyperchloremic metabolic acidosis

due to ammonium and chloride absorption [48].

Gastrocystoplasty, which is used much less commonly

than other methods of augmentation, can result in

development of hypochloremic, hypokalemic metabolic

alkalosis (Table 5.1).

Hyperchloremic metabolic acidosis is the most common

abnormality encountered with interposed bowel

segments in children. Nurse and Mundy [49] found that

the incidence of hyperchloremic metabolic acidosis in

ileocecal substitution, ileal augmentation, and ileal conduit

was 50%, 26%, and 12.5%, respectively. Whitmore

and Gittes [50] found a similar overall incidence in

intestinocystoplasty, with a hyperchloremic metabolic

acidosis occurring in 19% of patients. The risk of developing

electrolyte abnormality has been reported to be

higher in children with preoperative renal insufficiency,

but may also occur in the setting of normal renal function

[48-50]. Acidosis can result in bone demineralization

as calcium is mobilized to buffer the systemic acid

load. Some authors have found a decrease in linear

growth after intestinocystoplasty [51-53]; however,

this finding is not universal. Mingin et al. [54] compared

augmented spina bifida and exstrophy patients

to matched nonaugmented controls. They found that

children with intestinocystoplasty had a subclinical

hyperchloremic metabolic acidosis, but there were no

Table 5.1 Bowel segments and associated metabolic derangements.

Bowel segment Metabolic derangement Mechanism

Stomach Hypokalemic, hypochloremic metabolic alkalosis H and Cl loss

Jejunum Hyperkalemic, hyponatremic metabolic acidosis Na and Cl loss

K reabsorption

Ileum Hyperchloremic metabolic acidosis Ammonium reabsorption

Colon Hyperchloremic metabolic acidosis Ammonium reabsorption

34 Part II General Principles

differences in forearm bone densiometry, height percentile,

calcium metabolism, or calcium loss on 24 h urine

collection.

Children with interposed bowel segments should be

periodically monitored for metabolic and electrolyte

abnormalities. Correction of metabolic acidosis can be

undertaken with bicarbonate therapy. Severe hyperchloremia

can be treated with chlorpromazine or nicotinic

acid [4]. Treatment of metabolic acidosis typically

requires 0.5-1.0 mEq/kg/d. The amount of bicarbonate

needed can be calculated using the following formula:

Biocarbonate deficit Weight (kg) base deficit

0.3 mEq/kg/d

Conclusion

Immature renal function in infancy and early childhood,

in conjunction with postoperative medications

and physiologic changes in ADH and other circulating

hormones, makes pediatric patients especially susceptible

to water and electrolyte abnormalities. Hyponatremia

is by far the most common postoperative electrolyte

abnormality, and, when present, appropriate diagnosis

is necessary for effective treatment. Care must be taken

to provide appropriate postoperative fluids, as well as to

monitor patients at risk for electrolyte anomalies.

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2 Grosfeld JL (Ed). Pediatric Surgery, 6th edn. Mosby,

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3 Hellerstein S. Fluid and electrolytes: Clinical aspects. Pediatr

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4 Filston HC, Edwards 3rd, CH, Chitwood R, Jr. et al.

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5 Duke T, Molyneux EM. Intravenous fluids for seriously ill

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6 Burrows FA, Shutack JG, Crone RK. Inappropriate secretion

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8 Barratt MT, Avner ED, Harmon WE (Eds). Pediatric

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9 Chung HM, Kluge R, Schrier RW, Anderson RJ.

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10 Gruskin AB, Sarnaik A. Hyponatremia: Pathophysiology and

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11 Moran WH, Jr, Miltenberger FW, Shuayb WA et al. The relationship

of antidiuretic hormone secretion to surgical stress.

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12 Irvin TT, Modgill VK, Hayter CJ et al. Plasma-volume

deficits and salt and water excretion after surgery. Lancet

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13 LeQuesne LP, Lewis AAG. Postoperative water and sodium

retention. Lancet 1953;153-8.

14 Brenner BM, Rector FC, Jr (Eds). The Kidney, 5th edn.

Philadelphia: WB Saunders, 2008.

15 Wattad A, Chiang ML, Hill LL. Hyponatremia in hospitalized

children. Clin Pediatr (Phila) 1992;31:153-157.

16 Medani CR. Seizures and hypothermia due to dietary water

intoxication in infants. South Med J 1987;80:421-5.

17 Arieff AI, Ayus JC, Fraser CL. Hyponatremia and death

or permanent brain damage in healthy children. BMJ

1992;304:1218-22.

18 Verbalis JG, Martinez AJ. Neurological and neuropathological

sequelae of correction of chronic hyponatremia. Kidney

Int 1991;39:1274-82.

19 Berl T. Treating hyponatremia. Are we Damned if we do and

Damned if we don't? Kidney Int 1990;37:1008-18.

20 Laureno R, Karp BI. Myelinolysis after correction of

hyponatremia. Ann Intern Med 1997;126:57-62.

21 Adams RD, Victor M, Mancall EL. Central pontine myelinolysis:

A hitherto undescribed disease occurring in alcoholic

and malnourished patients. Arch Neurol Psychiatry

1959;81:154-72.

22 Sterns RH, Riggs JE, Schochet SS. Osmotic demyelination

syndrome following correction of hyponatremia. NEJM

1986;314:1535-42.

23 Sarniak AP, Meert KM, Hackbarth R, Fleischmann L.

Management of hyponatremic seizures in children with

hypertonic saline: A safe and effective strategy. Crit Care

Med 1991;19:758-62.

24 Adrogue HJ, Madias NE. Hyponatremia. NEJM

2000;341:1581-89.

25 Oh MS, Kim HJ, Carroll HJ. Recommendations for

treatment of symptomatic hyponatremia. Nephron

1995;70:143-50.

26 Kamel KS, Bear RA. Treatment of hyponatremia: A quantitative

analysis. Am J Kidney Dis 1993:21:439-43.

27 Gross P, Treatment of severe hyponatremia. Kidney Int

2001;60:2417-27.

28 Kleigman RM (Ed). Nelson Textbook of Pediatrics, 18th edn.

Saunders, Philadelphia, 2007.

29 Giebish G, Malnic G, Berliner RW. Control of renal potassium

excretion. In The Kidney, 5th edn. Edited by BM Brenner,

FC Rector, Jr. Philadelphia: WB Saunders, 1996, pp. 371-407.

30 Lorenz JM, Kleinman LI, Disney TA. Renal response of

newborn dog to potassium loading. Am J Physiol 1986;251:

F513-19.

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31 Schlossberg SM, Vaughan ED. The mechanism of unilateral

post-obstructive diuresis. J Urol 1984;131:534-6.

32 Better OS, Arieff AI, Massry SG et al. Studies on renal function

after relief of complete unilateral ureteral obstruction of

three months' duration in man. Am J Med 1973;54:234-40.

33 Moody TE, Vaughan ED, Jr., Gillenwater JY. Comparison of

the renal hemodynamic response to unilateral and bilateral

ureteral occlusion. Invest Urol 1977;14:455-9.

34 Buerkert J, Martin D, Head M et al. Deep nephron function

after release of acute unilateral ureteral obstruction in the

young rat. J Clin Invest 1978;62:1228-39.

35 Sonnenberg H, Wilson DR. The role of medullary collecting

ducts in post-obstructive diuresis. J Clin Invest

1976;57:1564-74.

36 Li C, Wang W, Kwon TH et al. Altered expression of major

Na transporters in rats with bilateral ureteral obstruction

and release of obstruction. Am J Physiol Renal Physiol

2003;285:F889-901.

37 Wahlberg J, Stenberg A, Wilson DR et al. Tubuloglomerular

feedback and interstitial pressure in obstructive nephropathy.

Kidney Int 1984;26:294-302.

38 Frokiaer J, Marples D, Knepper MA et al. Bilateral ureteral

obstruction downregulates expression of vasopressinsensitive

AQP-2 water channel in rat kidney. Am J Physiol

1996:270:F657-68.

39 Ryndin I, Gulmi FA, Chou SY, Mooppan UMM, Kim H.

Renal responses to atrial natiuretic peptide are preserved

in bilateral ureteral obstruction and augmented by neutral

endopeptidase inhibition. J Urol 2005;173:651-6.

40 Howards SS, Post-obstructive diuresis: A misunderstood

phenomenon. J Urol 1973;110:537-40.

41 Wilson DR. Micropuncture study of chronic obstructive

nephropathy before and after release of obstruction. Kidney

Int 1972;2:119-30.

42 Baum N, Anhalt M, Carlton CE, Jr., Scott R, Jr. Postobstructive

diuresis. J Urol 1975;114:53-6.

43 Bishop MC. Diuresis and renal functional recovery in

chronic retention. Br J Urol 1985;57:1-5.

44 O'Reilly PH, Brooman PJC, Farah NB et al. High pressure

chronic retention. Incidence, aetiology and sinister implications.

Br J Urol 1986;58:644-6.

45 Jones DA, George NJR, O'Reilly PH. Postobstructive renal

function. Semin Urol 1987;5:176-90.

46 Vaughan ED, Jr., Gillenwater JY. Diagnosis, characterization

and management of post-obstructive diruesis. J Urol

1973;109:286-92.

47 Nyman MA, Schwenk NM, Silverstein MD. Management of

urinary retention: Rapid versus gradual decompression and

risk of complications. Mayo Clin Proc 1997;72:951-6.

48 Hall MC, Koch MO, Mc Dougal WS. Metabolic consequences

of urinary diversion through intestinal segments.

Urol Clin North Am 1991;18:725-35.

49 Nurse DE, Mundy AR. Metabolic complications of cystoplasty.

Br J Urol 1989;63:165-70.

50 Whitmore WF, Gittes RF. Reconstruction of the urinary

tract by cecal and ileocecal cystoplasty: Review of a 15 year

experience. J Urol 1983;129:494-8.

51 Mundy AR, Nurse DE. Calcium balance, growth and skeletal

mineralization in patients with cystoplasties. Br J Urol

1992;69:257-9.

52 Wagstaff KE, Woodhouse CR, Duffy PG et al. Delayed linear

growth in children with enterocystoplasties. Br J Urol

1992;69:314.

53 Gross DA, Lopatin UA, Gearhart JP et al. Decreased linear

growth associated with intestinal bladder augmentation in

children with bladder exstrophy. J Urol 2000;164:917-20.

54 Mingin GC, Nguyen HT, Mathais RS et al. Growth and metabolic

consequences of bladder augmentation in children

with myelomeningocele and bladder exstrophy. Pediatrics

2002;110:1193-8.

36

Perioperative Anesthetic

and Analgesic Risks and

Complications

Philippa Evans and Mark Thomas

Introduction

Modern anesthesia is extremely safe. In fact the risk of serious

injury or death is so small that it makes it difficult to

measure accurately. National Confidential Enquiry into

Peri-operative Deaths (NCEPOD) data in the UK [1], the

Australian Incident Monitoring System (AIMS) project in

Australia [2], and the closed claims process in the United

States [3] are the best sources of recent data. However, while

these sources provide details of death or serious injury they

do rely on accurate reporting. Furthermore, they give no

indication of the numbers of total anesthetics administered

and without this denominator it is not possible to quote

accurate rates of risk.

What is clear from the literature is that the mortality

associated with anesthesia has decreased dramatically

from 6 per 10,000 in the 1950s to 0.36 per 10,000 by the

start of this millennium [4-6].

There are several reasons for this. Firstly, we have become

much more aware of the need for accurate audit over this

time and have used audit as a powerful tool to develop safer

practice. Secondly, we have become more subspecialized.

There is good evidence that a trained pediatric anesthetist can

decrease the incidence of perioperative events [7,8]. Thirdly,

we have seen great advances in equipment and monitoring

over recent years and have developed minimum monitoring

standards to ensure the highest quality of patient care.

Of course, figures quoted for risk are very much dependent

on individual patient factors. It is abundantly clear from

the studies quoted above that children in the younger age

group, specifically below one year of age, have a greater

incidence of anesthetic mortality. American Society of

Anesthesiologists (ASA) status and comorbidities such as

prematurity, obstructive sleep apnea, and congenital abnormalities

will all adversely impact upon the risk. However

human error and equipment failure account for the majority

of negative outcomes and, if they could be eliminated,

would make approximately 90% of critical incidents preventable.

So, while medical optimization preoperatively

remains important the ability to reduce risk further lies

squarely with the anesthetist and his or her team and with

systems designed to minimize the scope for human error.

Key points

• The mortality associated with anesthesia is low,

but is higher at the extremes of age.

• The risks associated with anesthesia have been

greatly reduced by improvements in equipment

and monitoring techniques.

• Respiratory adverse events are the most

common perioperative problems.

• There are side effects or risks associated with

all modalities of analgesia. Recent data show

epidural analgesia to be associated with fewer

complications in children compared with adults.

• Intravenous fluids and blood products should

be prescribed with the same caution and

consideration as any other medication.

6

Pediatric Urology: Surgical Complications and Management

Edited by Duncan T. Wilcox, Prasad P. Godbole and Martin A. Koyle

© 2008 Blackwell Publishing Ltd. ISBN: 978-1-405-16268-5

Chapter 6 Perioperative Anesthetic and Analgesic Risks and Complications 37

Specific complications of general

anesthesia, their prevention and

management

Respiratory complications

Respiratory adverse events are the most common perioperative

problems faced by the anesthetist [9,10]. The

incidence of adverse events is higher in younger children

due to the relatively narrow infant airway coupled with

the high incidence of respiratory tract infections in this

population. The most common events are periods of arterial

desaturation, laryngospasm, and bronchospasm [11].

Laryngospasm is the reflex closure of the glottis by

adduction of the true or false vocal cords. It can persist

after cessation of the stimulus. Common causes include

local stimulation of the larynx, e.g. by saliva, blood, or

foreign body including a laryngoscope or endotracheal

tube. It can also occur in response to other stimulation,

e.g. surgery, movement, or stimulation of the

anus or cervix. The reflex is abolished in deeper planes

of anesthesia. Laryngospasm leads to partial or complete

airway obstruction, which presents with stridor

and causes hypoxemia and hypoventilation and in the

most severe cases negative pressure pulmonary edema.

Treatment consists of removing the stimulus, giving

100% oxygen and providing positive end expiratory

pressure (PEEP) via the breathing circuit. If the spasm

does not resolve with these maneuvres then a small dose

of intravenous induction agent or muscle relaxant can be

used to break the spasm.

Children with upper respiratory tract infections

(URIs) have a sevenfold increase in respiratory complications

compared to asymptomatic children [12].

Additional risk factors for developing adverse respiratory

events in children with URIs include: the use of an

endotracheal tube, age less than five years or a history

of prematurity (less than 37 weeks gestation), a history

of reactive airway disease or nasal congestion, parental

smoking, and surgery on the airway [13].

Airway complications

Control of a patient's airway is the most important

aspect of any general anesthetic. Difficulties can arise

due to inability to control a patient's airway and provide

adequate ventilation by bag-mask ventilation - a "can't

ventilate" situation - or when the airway can be managed

but intubation is difficult - a "can't intubate" situation.

The incidence of difficult laryngoscopy/intubation

varies between 1.5% and 13% and failed intubation has

been identified as one of the anesthesia-related causes of

death or permanent brain damage [14]. This happens

when a "can't intubate can't ventilate" situation arises.

There are certain childhood syndromes that are associated

with difficult airways and these should be highlighted

during preoperative assessment in order that the

anesthetic can be planned accordingly. These syndromes

include the Pierre Robin Sequence, Crouzon syndrome,

Apert syndrome, Pfeiffer syndrome, Treacher-Collins

syndrome, craniofacial microsomia, and Goldenhar

syndrome [15].

More minor adverse events associated with the airway

include dental damage and postoperative sore throat.

Oral tissue and dental damage are common complications

of general anesthesia and account for a significant

proportion of medicolegal claims against anesthetists in

adult practice [16]. These injuries tend to occur during

laryngoscopy and intubation. A dental history should be

obtained during the preoperative visit and children with

wobbly teeth (peak ages 6-8 years) should be warned of

potential loss! Postoperative sore throat has a reported

incidence of 12.1% 24 h after surgery in daycase adult

patients. The incidence is higher after intubation than

following insertion of a laryngeal mask airway, and

interestingly still occurs in some patients who have had

no instrumentation of their airway but merely received

airway support via a facemask [17].

Cardiovascular complications

Adverse cardiac events are only a quarter as common as

respiratory complications during anesthesia. Common

events include arrhythmias and bradycardias, and hypertension

or hypotension [11]. The results of the Paediatric

Perioperative Cardiac Arrest Registry (POCA) found an

estimated incidence of anesthesia-related cardiac arrest

of 1.4 per 10,000 with a mortality rate of 26% in those

affected [6]. Pharmacological (overdose) and underlying

cardiac disease were the most common causes; 55% of

events occurred in children under the age of one year.

Gastrointestinal complications

Vomiting is the most common postoperative adverse

effect associated with anesthesia. Incidence ranges from

9% to 43% [18]. Incidence increases with age, and peaks at

11-14 years corresponding with puberty. The incidence of

vomiting is also related to the site of surgery and is highest

following strabismus surgery, appendicectomy, ENT

surgery, and orchidopexy. High-risk patients include those

with a previous history of postoperative nausea and vomiting

and/or a history of motion sickness [19]. Anesthetic

technique can influence the risk of postoperative

38 Part II General Principles

vomiting. Techniques should be adjusted and preemptive

antiemetics given to those considered to be high risk.

The incidence of perioperative aspiration is low and

generally has a good outcome. It is reported as between

1 and 10 per 10,000 with a very low incidence of pnemonitis

or need for admission to intensive care [20].

Neurological complications

Peripheral nerve injury has a reported incidence of 1 per

1000 anesthetics in adults. The most commonly affected

are ulnar nerve 30%, brachial plexus 23%, and the

lumbosacral nerves 16% [21]. Poor positioning intraoperatively

is a common underlying factor. The usual

mechanism of injury to superficial nerves is secondary

to ischemia due to compression of the vasa vasorum by

surgical retractors, leg stirrups, or contact with other

equipment. Injury is more likely to occur during periods

of poor peripheral perfusion due to hypotension

and hypothermia. Injury may be less likely in children

because of the protection afforded by their increased

subcutaneous tissue compared with adults and their

lighter weight. The mechanism of injury to the brachial

plexus is usually traction caused by excess shoulder

abduction. Damage can be avoided by taking meticulous

care with patient positioning, using padding to protect

pressure points, and avoiding extreme joint positions.

Most nerve injuries recover over a period of months; all

need to be reviewed by a neurologist.

The commonest type of ocular complication is corneal

abrasion. This presents with blurring of vision and usually

resolves over 1-2 months [16]. Protective reflexes are

lost during anesthesia and eyes need to be taped shut to

protect the cornea. Care must be taken to avoid pressure

to the eyes and extra padding and protection is needed if

the patient is in the prone position for surgery.

Awareness during anesthesia is a state of consciousness

that is revealed by explicit or implicit memory of intraoperative

events. From adult data, the incidence of conscious

awareness with explicit recall and severe pain is estimated

to be 1 per 3000 general anesthetics. Conscious awareness

with explicit recall but without pain is more common

with an incidence between 0.1% and 0.7% [22].

The incidence of awareness is higher in cases where neuromuscular

blocking agents are used. Patients who experience

awareness are at risk of developing post-traumatic

stress disorder. The commonest cause of awareness is drug

error: either inadvertent paralysis of an awake patient or

failure of delivery of volatile anesthetic agents.

Maladaptive postoperative behaviors have been

reported to occur in up to 65% of children undergoing

anesthesia and surgery. Changes include anxiety, sleep

disturbance, night terrors, and a return to bed wetting.

Variables such as young age, degree of patient and parent

preoperative anxiety, child's anxiety at induction, type

of surgery, and level of postoperative pain have been

reported to predict the occurrence of behavioral changes

[23]. Prolonged upset occurs even after daycase surgery

and anesthesia. Up to 32% of children exhibit negative

behavioral changes one month after their operation

[24]. Sevoflurane is the volatile anesthetic agent that is

most widely used for inhalational induction. Patients

who have received sevoflurane are often agitated on their

emergence from anesthesia. A short-lived post-anesthetic

delirium is well described. However, this emergence

delirium associated with sevoflurane is still the subject

of some debate and does not seem to develop into prolonged

neurocognitive changes. The incidence of maladaptive

behaviors seems to be similar with sevoflurane,

halothane, and isoflurane [25,26].

Rare but serious complications

Anaphylactic and anaphylactoid reactions during

anesthesia are rare but potentially life-threatening allergic

events. The two types of reaction are clinically indistinguishable

and in their most severe manifestations

present with cardiovascular collapse, bronchospasm, and

laryngeal edema. Serum histamine and mast cell tryptase

levels help confirm the diagnosis [27]. Patients require

follow-up with skin-prick test and Radio Allergy Sorbent

Test (RASTs) for specific IgE antibodies to identify the

triggering agent. The incidence of anaphylaxis is approximately

1 per 6000 anesthetics [28]. The most commonly

incriminated agents are the neuromuscular blocking

agents (58% of reactions), latex (16.7%), and antibiotics

(15%). A history of atopy, asthma, and food allergy are

more frequent in cases of latex allergy [29].

Malignant hyperthermia (MH) is a rare autosomal

dominant condition that is triggered by volatile anesthetic

agent and suxamethonium. It has an incidence of 1 in

15,000 in children and 1 in 50-100,000 in adults [30]. It

still has a mortality of 10% and requires prompt and

efficient recognition and management [31]. Diagnosis

is made by muscle biopsy and relatives of the index case

must also be investigated. Anesthesia in susceptible individuals

requires careful planning and avoidance of potential

trigger agents.

Suxamethonium is a short acting depolarizing muscle

relaxant. It is metabolized by plasma cholinesterase.

Suxamethonium apnea occurs in patients with reduced

cholinesterase activity. This reduced activity arises either

Chapter 6 Perioperative Anesthetic and Analgesic Risks and Complications 39

as a result of genetic variability in cholinesterase type or

secondary to acquired conditions, such as liver disease

and cancer. It presents as prolonged nonreversible paralysis

at the end of the anesthetic. Management involves

continued sedation and ventilation until the drug has

been metabolized. This may involve several hours of

mechanical ventilation.

Specific complications of pain

management

There are very few, if any, urological procedures that are

not potentially painful to a greater or lesser extent. Many

are easily ameliorated with simple analgesic regime while

other require more sophisticated regional techniques in

addition.

Systemic analgesia

Of course, when any drug is administered to any patient

the potential for error exists. It is always possible to give

the wrong drug (in the case of an allergic history for

instance), the wrong dose, or to give a drug by the wrong

route. Assuming that we are talking about giving appropriate

drugs to appropriate patients, it is worth considering

the range of agents at our disposal.

Paracetamol

This drug's pharmacokinetics are better known than

those of any other drug in the pediatric pharmacopoeia. The

main risks in its use relate to the potential for overdose.

Suggested maxima are 25 mg\kg per 24 hours at under 30

weeks postconceptional age, 45 mg\kg at less than 34 weeks,

60 mg/kg in term neonates and infants and 90 mg/kg

thereafter [32]. Paracetamol is metabolized in the liver

mainly by glucuronidation and sulfation but if these pathways

become saturated the hepatotoxic oxidative metabolite

N-acetyl-p-benzoquinoneimine may accumulate. Risk

factors for this include dehydration and sepsis.

Nonsteroidal anti-inflammatory drugs (NSAIDs)

In a large study, the risk of administering a short-term

course of ibuprofen was low and similar to paracetamol

[33]. Childhood asthma does not seem to be affected by

NSAIDs in the way as adult asthma [34]. Impaired renal

function and bleeding tendency remain contraindications,

however. The use of NSAIDs below six months of

age is ill advised because of the risk of pulmonary hypertension

and alterations in cerebral and renal regional

blood flow that is so dependent on prostaglandins in this

age range. A growing body of literature describing the

use of NSAIDs for the closure of patent ductus arteriosus

in neonates has led to the reappraisal of the lower age

limit, and the current United Kingdom national formulary

now contains dose-guidance for analgesic use down

to one month of age if 5 kg [35].

Opioids

The main side effects of this group are respiratory

depression, nausea and vomiting, constipation, urinary

retention, itching, and sedation. From a safety perspective

clearly the most important of these is respiratory

depression.

Since we have moved away from intermittent intravenous

bolus and intramuscular injections, the risk has

diminished because with infusions and bedside-controlled

analgesia pumps the potential for high-peak plasma concentrations

is less.

The addition of high background infusion rates to

patient-controlled regimes increases the risk of sedation,

hypoxia, nausea, and vomiting [36]. However a low rate

of 4 mcg/kg/h has advantages in analgesia without this

untoward side effect profile [37].

Respiratory depression is readily treatable with

naloxone but due to its relatively short duration of

action this may need to be repeated or infused. Urinary

retention and itching have been successfully treated with

lower doses of naloxone while itching often responds to

chlorpheniramine.

Nausea and vomiting occurs in 30-45% of children on

patient-controlled analgesia pumps and can be reduced

by prophylactic anti-emetic administration [38,39].

Adding an anti-emetic to the morphine pump is not

effective [40].

Nurse controlled analgesia pumps for children 5

years of age or so is effective [41] but is recommended

for use by trained nurses rather than parents [42].

Regional analgesia and local anesthesia

Epidural

A recent national audit of pediatric epidural complications

has just been completed in the United Kingdom.

Birmingham Children's Hospital painstakingly gathered

information from 21 pediatric centers over a 5-year

period resulting in data from more than 10,000 epidurals.

Ninety-six serious clinical incidents were reported giving

an overall incidence of 0.9% [43].

Out of these 96 clinical incidents, 40 were judged by

an expert panel to be coincidental to the epidural. These

included complications such as pressure sores and compartment

syndrome. Of the remaining 56 incidents the

40 Part II General Principles

commonest was local infection. The relative incidences

in this group are given in Table 6.1.

It is clear from the above that epidurals are extremely

safe. However, there is still considerable debate within

anesthesia as to the merits of epidural analgesia over and

above systemic modalities of pain relief. This is not the

least because the consequences of a serious complication

following an epidural are so great.

Having said that only one patient from the United

Kingdom audit had persistent neurological problems more

than 12 months postepidural insertion. This compares very

favorably with adult epidural data in which the quoted

risk of permanent neurological injury is 2-7:10,000 [44].

Furthermore, systemic analgesic techniques are not without

complications themselves.

If we were to analyze why there has been a decline in

the administration of epidurals, we would most likely

attribute it at least in part to the rise of a risk-averse

culture. Complications arising from opioids are more

likely to be attributed to patient and drug characteristics.

Complications arising from the epidural can more

readily be apportioned, fairly or not, to poor technique

or operator error.

Caudal analgesia

Caudals are the commonest regional technique used in

pediatric anesthetic practice. They are relatively easy to

perform, especially in children 8 years or so, and are

effective. They provide analgesia for 3-10 h depending on

the drug combinations used [45]. There are reports on

large series of caudals with very few side effects [46,47].

Transient leg weakness and urinary retention should

always be anticipated.

The most common additives in the United Kingdom

are clonidine and ketamine. The former approximately

doubles the duration of a plain local caudal to 5-6 h and

with ketamine a further 4 h or so may be expected [45].

Clearly such prolonged block needs to be anticipated and

warned for if inadvertent injury is to be avoided.

Peripheral blocks

As with all blocks, the potential for local anesthetic

toxicity exists. The risk is greatest when the solution is

injected into vascular tissue or injected inadvertently

intravascularly. Most texts recommend an upper limit

of 2 mg/kg for bupivacaine (0.8 ml/kg of 0.25%). The

recent introduction of levorotatory bupivacaine has been

widely embraced into clinical practice since this form has

been shown to be equally effective yet less cardio toxic in

the event of inadvertent intravascular injection. If cardiotoxicity

does occur it may manifest it as ventricular

extrasystoles, which can progress to a particularly shockresistant

ventricular fibrillation.

Adrenaline-containing solutions should be avoided

near end arteries such as dorsal nerve block or ring block

to help avoid the risk of penile necrosis. Ilioinguinal

blocks are commonly performed for groin surgery and

are extremely safe and effective. The main local risk

for these is tracking of the local anesthetic next to the

femoral nerve with resultant leg weakness and possible

delayed discharge as a result. The increasing use of ultrasound

guidance in the accurate placement of blocks may

reduce this complication.

Complications associated with

intravenous fluids

Recent publications have highlighted the risks of administering

infusions of hypotonic solutions to both medical

and surgical pediatric patients [48]. The infusion of

hypotonic solutions (such as 0.18% sodium chloride

with 4% glucose or 5% glucose) is associated with the

development of acute hyponatremia. The most serious

complication of hyponatremia is hyponatremic encephalopathy,

which can lead to permanent neurological

damage and death. Over 50% of children with a serum

sodium of 125 mmol/l will develop hyponatremic

encephalopathy [49]. Hyponatremia is associated with

the movement of water into brain tissue, which can lead

to cerebral edema. The resultant increase in brain volume

can lead to brain herniation and death. Children

are at particular risk as they have a higher number of

brain cells and a larger brain to intracranial volume

ratio compared with adults [50]. In a recent review of 50

cases of hospital-acquired hyponatremic encephalopathy

Table 6.1 The incidences of serious complications following

pediatric epidurals (United Kingdom National Audit).

Complication Incidence (out of 10,633

epidurals)

Infection 28

Drug error 14

Nerve injury 6

Postdural puncture headache 6

Local anesthetic toxicity 1

Inadvertent spinal anesthetic 1

Chapter 6 Perioperative Anesthetic and Analgesic Risks and Complications 41

mortality was as high as 50%. More than half the cases

occurred in the postoperative setting in previously

healthy children undergoing minor surgery [51].

For half a century, fluid therapy in children has been

based on Holliday and Segar's formula, which proposed

to match children's water and electrolyte requirements

on a weight-based calculation using hypotonic solutions

[52]. The formula was derived following studies

of metabolism in active children. It has been argued that

the requirements of hospitalized relatively inactive children

are less.

Surgical patients are at particular risk of developing

hyponatremia. The postoperative period is associated

with a nonosmotic secretion of antidiuretic hormone

(ADH). ADH reduces the ability of the kidneys to

excrete free water leading to hyponatremia and oliguria.

Infusion of hypotonic solutions further exacerbates

the situation. Excess ADH secretion can be encountered

even after minor surgery [49]. Pain, stress, anxiety, nausea,

and vomiting, and morphine can all act stimuli for

its release.

Studies have shown that while infusions of hypotonic

solutions in the perioperative period are associated with

falls in plasma sodium, infusion of isotonic solutions

are associated with stable plasma sodium levels [53].

Holliday and Segar have recently changed their recommendations.

They suggest halving the average maintenance

volume, i.e. 50 ml/kg/day for the first day of the

infusion and monitoring serum sodium if the need for

intravenous fluids continues [54].

Fluid therapy in surgical patients should be designed

to provide for different requirements: fluid deficits,

maintenance fluid requirements, and volume of fluid

needed to maintain an adequate tissues perfusion (and

counteract the effects of anesthetics). Fluid deficits consist

of preoperative deficits (fasting, gastrointestinal,

renal, or cutaneous losses), hemorrhage and third space

losses. The National Patient Safety Agency in the United

Kingdom has recently produced a Patient Safety Alert

with regard to intravenous fluid therapy in children [55].

They recommend the immediate removal of sodium

chloride 0.18% with glucose 4% from use. In units where

this has occurred, there have been no further cases of

iatrogenic hyponatremia [56]. They emphasize that the

prescribing of fluids should be afforded the same considerations

as the prescription of other drugs with reference

to indications, contraindications, and dose [57] and

that prescriptions should be individualized [58]. They

recommend that intravascular volume depletion should

be managed using bolus doses of sodium chloride 0.9%

(isotonic), and that ongoing losses should be replaced

with a similar biochemical solution. In most cases this

would be an isotonic solution such as sodium chloride

0.9%, sodium chloride with glucose 5% or Hartman's

solution (or Ringer's lactate). They state that sodium

chloride 0.45% with glucose 5% or 2.5% can safely be

prescribed for the majority of children as maintenance

fluid. They urge closer patient monitoring with regular

weights and measurement of plasma sodium. They

also call for a review of drug prescription charts so that

maintenance fluids can be prescribed separately to other

intravenous fluids [55].

Complications associated with blood

transfusion

Children are more susceptible than adults to the harmful

effects of hypovolemia. Volume correction is therefore of

paramount importance and can be achieved with crystalloids

and artificial colloids. In general children tolerate

hemodilution well and perioperative levels of 6 g/dl are

acceptable in a hemodynamically stable child. There are

of course many potential complications of transfusion

namely hypocalcemia, hyperkalemia, hypomagnesemia,

metabolic acidosis, and hypothermia. However, all of

these are generally correctable [59]. What is of greater

concern to parents and children is the infective risk of

blood transfusion.

Infectious disease transmission risk

Infectious risks of transfusion have decreased dramatically

secondary to improved screening, detection of

infected agents, and advances in pathogen inactivation.

Nonetheless the risk of infection, especially of human

immunodeficiency virus and hepatitis C, is often of great

concern to the parents of children who may need blood

transfusion. The incidence varies between countries and

is dependent upon the prevalence of these infections

within the donor community and the resources used to

screen the blood products. Tables 6.2 and 6.3 illustrate

the current situation in the United States.

Besides viral pathogens, bacterial contamination can

occur. This is most commonly seen with platelets [60].

Standards for testing platelets for bacterial growth are

being developed. Other infectious diseases that can

potentially be transmitted by transfusion include Chagas

disease, Lyme disease, malaria, and Creutzfeldt-Jakob

disease (CJD). Although no specific nucleic acid or antigen

testing for these diseases exist, donor screening and

42 Part II General Principles

the deferral of those with potential symptoms helps

prevent transfusion-related transmission. In the United

Kingdom all blood products are leukocyte-depleted and

clotting products are sourced from the United States to

reduce the risk of transmission of new variant CJD.

Incompatibility and other immunologic

considerations

Clerical error is the most common cause of mismatched

transfusion. Severe acute hemolytic reactions

most often result from immunologic destruction of red

cells because of ABO incompatibility. Less frequently,

serologic incompatibilities not detected by standard

antibody screens can cause an acute hemolytic reaction.

Anaphylactoid reactions with bronchospasm, laryngeal

edema, and urticaria are dangerous but rare and typically

occur in IgA-deficient individuals. The mandatory use

of leukocyte-depleted products in the United Kingdom

has significantly reduced transfusion reactions and

immune modulation caused by cytokines and leukocytedegradation

products. Formation of antihuman leukocyte

antigen (HLA)-antibodies and febrile transfusion reactions

have also been virtually eliminated [61].

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Screening tests

Hepatitis B surface antigen (HBsAg)

Hepatitis B core antibody (anti-HBc)

Hepatitis C virus antibody (anti-HCV)

HIV-1 antibody (anti-HIV-1)

HIV-2 antibody (anti-HIV-2)

HTLV-I antibody (anti-HTLV-I)

HTLV-II antibody (anti-HTLV-II)

Nucleic acid amplification testing (NAT) for HIV-1 and HCV

Serologic test for syphilis

Nucleic acid amplification for West Nile virus

Source: Data from www.aabb.org.

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Virus Risk Days possible to

transmit disease,

i.e. false negative

screen

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Hepatitis B 1 per 137,000 59

Hepatitis C 1 per 1,000,000 82

Human 1 per 641,000 or less 51

T-lymphotrophic

virus I and II

HIV 1 per 1,900,000 22

Source: Data from www.aabb.org.

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Paediatric Update. Eur J Cancer 2001;37:2421-7.

III Open Surgery of the

Upper Urinary Tract

Pediatric Urology: Surgical Complications and Management

Edited by Duncan T. Wilcox, Prasad P. Godbole and Martin A. Koyle

© 2008 Blackwell Publishing Ltd. ISBN: 978-1-405-16268-5

47

Nephrectomy

Paul Crow and Mark Woodward

Introduction

The advent of laparoscopic surgery has made open

nephrectomy an infrequent undertaking in pediatric urology.

The most common indications for open nephrectomy

are now malignant disease (see Chapter 33) and

trauma (see Chapter 38).

Contraindications to elective, simple laparoscopic

nephrectomy are increasingly rare, with some centers now

reporting successful laparoscopic surgery for xanthogranulomatous

pyelonephritis [1]. These facts, together with

the absence of contemporary publications on elective

open simple nephrectomy, would seem to confirm that

laparoscopic surgery has now taken over as the gold

standard approach.

Although infrequently applied, a good working knowledge

of open approaches to the kidney remains important

to the pediatric urological surgeon. In particular,

the ability to convert rapidly from laparoscopic to open

nephrectomy, whether it is to control hemorrhage or for

nonprogression, remains an essential surgical skill. In

addition, familiarity with the choice of open approaches

to both normally positioned and ectopic kidneys is vital

on the rare occasion that laparoscopy is contraindicated.

This chapter will cover open approaches to the kidney,

concentrating on the anatomical basis, advantages, and

potential complications of each approach.

Surgical approaches to the kidney

The first intentional nephrectomy was performed by

Gustav Simon in 1869 in the treatment of an ureterovaginal

fistula. The first pediatric nephrectomy for a Wilms

tumor was performed in Leeds, by Richard Jessop in 1877.

Retroperitoneal flank approaches had a lower incidence

of postoperative peritonitis and were the approach of

choice in the first half of the 20th century. Advancements

in the surgical technique led to a revival of transabdominal

approaches in the 1950s. The modern surgeon has

numerous choices of approach, which can be tailored to

the needs of each individual case.

Retroperitoneal approaches

The majority of surgeons prefer a retroperitoneal

approach to simple nephrectomy, the principal advantage

being avoidance of the peritoneal cavity and the associated

risk of forming intraperitoneal adhesions. Open access to

the retroperitoneum can be achieved through loin, lumbotomy,

and anterior approaches. Readers are referred

Key points

• With the advent of laparoscopic surgery, open

simple nephrectomy is rarely performed.

• The retroperitoneal approach is preferable to the

transabdominal approach as it does not lead to

the formation of intraperitoneal adhesions.

• Conversion of laparoscopic to open

nephrectomy is usually achieved by joining or

extending port site incisions.

• Preoperative imaging provides valuable

anatomical information useful in planning

nephrectomy, particularly when the kidney is

ectopic.

• Careful preoperative planning and surgical

technique can minimize the morbidity associated

with hemorrhage or damage to perirenal

structures.

7

Pediatric Urology: Surgical Complications and Management

Edited by Duncan T. Wilcox, Prasad P. Godbole and Martin A. Koyle

© 2008 Blackwell Publishing Ltd. ISBN: 978-1-405-16268-5

48 Part III Open Surgery of the Upper Urinary Tract

to excellent operative texts of the various approaches

while the advantages and disadvantages of the various

approaches are described here.

Loin (flank) approach

Advantages Good access to renal parenchyma and

collecting system [2].

Good access in obese patients.

Disadvantages Exposure of renal pedicle inferior to

anterior approaches.

Relatively large incision with

higher incidence of wound pain

and muscle bulge.

Dorsal lumbotomy

Advantages Rapid access without cutting muscle [3].

Useful for bilateral procedures without

patient repositioning.

Less postoperative pain and bulge.

Fresh approach in those with previous

loin or abdominal surgery.

Disadvantages Exposure may be limited and

access to the kidney and pedicle

inferior, so more suitable

for low, small, or cystic kidneys.

Anterior subcostal/transverse

Advantages Good access to the renal pedicle.

Disadvantages Retraction of peritoneal cavity can limit

access.

Transabdominal approaches

The increased postoperative recovery time and risk of

intraperitoneal adhesion formation means that these

approaches are generally reserved for malignant or traumatic

cases (see Chapters 33 and 38). The advantage of

these approaches is that they allow excellent access to

the renal pedicle and great vessels. Transverse/subcostal

or midline incisions are most frequently employed,

although occasionally a thoracoabdominal incision may

be used.

The advantages/disadvantages of the transverse/subcostal

approach have been discussed; converting it to a

transabdominal procedure simply involves opening rather

than retracting the peritoneum. This approach allows

excellent access to the lateral and superior portion of the

kidney. If required the incision can be extended across the

midline, although this involves further muscle division.

A midline incision is generally preferred in traumatic

cases or when the patient has a narrow subcostal angle.

It allows for rapid access without muscle cutting via

incision of the linea alba and provides good access to

the entire peritoneal cavity. The incision is made from

the xiphoid to the caudal aspect of the umbilicus but is

easily extended inferiorly. Mass closure is performed.

A thoracoabdominal approach tends to be reserved for

large upper pole tumors [4] and has the obvious disadvantage

of entering the thoracic cavity and cutting costal

cartilage.

Independent of incision choice, once the peritoneal

cavity has been entered, the colon can be reflected medially

by incising its lateral peritoneal attachment. On the

left side this is facilitated by dividing the splenocolic ligaments,

which also prevents undue traction on the spleen.

On the right side the duodenum is reflected along with

the colon. Exposure may be maintained with the use of

a ring retractor. Vascular anatomy is variable, but on the

right side the renal vein normally receives no tributaries,

but is short and care must be taken not to damage the

vena cava. If retraction of the right renal vein is difficult,

the renal artery can be taken between the vena cava and

aorta as opposed to lateral to the cava. On the left, the

renal vein is long and typically receives gonadal, adrenal,

and lumbar tributaries. These are ligated and divided to

facilitate retraction of the renal vein and ligation of the

renal artery. The kidney is mobilized by sharp dissection

starting laterally to provide better access to posterior

hilar area where friable veins may be present.

Surgical approaches in specific situations

Conversion from laparoscopic to open

nephrectomy

The choice of conversion technique depends on the

reason for conversion and the laparoscopic approach

employed. Emergency conversion for severe hemorrhage

should be via whichever route the surgeon feels will

allow fastest vascular control. If possible, pressure should

be applied to the bleeding point with a pledget or open

swab pushed through a port site. If the reason for conversion

is less precipitous, then the surgeon can be more

circumspect about the incision. In retroperitonoscopy,

conversion is usually achieved by joining the port sites in

the form of a subcostal loin incision. In transabdominal

laparoscopy, the port site best placed to give access to the

area of difficulty is extended.

Approaches to ectopic kidneys [5]

Ectopic kidney position can be a result of abnormal

migration (e.g. pelvic kidney) or abnormal fusion

Chapter 7 Nephrectomy 49

(e.g. horseshoe kidney and crossed fused renal ectopia).

The choice of incision will depend on the position,

size, and vascular supply of the renal unit to be

removed, as determined by the preoperative imaging.

An extraperitoneal iliac fossa approach is often the best

for a pelvic kidney. An oblique skin incision is deepened

by dividing the external oblique aponeurosis inline

with its fibers. The internal oblique and transversus

abdominis muscles are divided by muscle splitting. The

peritoneum is bluntly mobilized and retracted medially

to give access to the retroperitoneal space and the

kidney. The ectopic renal vessels can derive from the lower

aorta or iliac vessels and the anatomy must be carefully

defined before the vessels are taken and kidney removed.

An anterior subcostal extraperitoneal incision is usually

the favored approach for a horseshoe kidney heminephrectomy,

although a transverse supraumbilical

transabdominal approach is better if access to both sides

of the kidney is required. A midline incision is often preferred

in older children and adolescents. The vascular

supply varies considerably between individuals and preoperative

CT reconstruction can provide valuable information.

The lower poles and isthmus frequently receive blood

from the common iliac vessels, which can be intimately

related to the gonadal vessels and ureters. Again, careful

dissection is required to define the vascular anatomy. The

isthmus can be thin and fibrous, and if so can be divided

between clamps and the edges oversewn. In a thicker isthmus

the renal capsule is incised and the arcuate vessels are

ligated individually, with any calyceal breaches repaired.

Complications

Complications are uncommon in simple open nephrectomy

in children. While there are numerous contemporary

publications outlining complication rates following

laparoscopic nephrectomy, there are no recent series with

outcome data from open nephrectomy. In this section,

the more frequently encountered and the potentially

more serious complications are discussed together with

the methods by which they can be avoided and treated.

Intraoperative complications

Hemorrhage

Intraoperative hemorrhage is very unusual in open

nephrectomy in children, and blood is usually only crossmatched

if the surgeon anticipates difficulties, e.g. XGP

nephrectomy. Preoperative contrast CT is rarely required.

If hemorrhage is encountered, it is vital that the anesthetist

is immediately made aware of the problem. As a

general rule, brisk hemorrhage is best initially dealt with

by applying pressure while ensuring that suction and vascular

equipment is available. Proper exposure of the area

allows for precise vascular control and is more reliable

than blind diathermy or clip application. Venous hemorrhage

tends to be more troublesome and difficult to

locate than arterial bleeding. The sites of venous bleeding

are to some extent predictable, with four areas most commonly

encountered:

1 Lumbar veins entering the posterolateral aspect of the

vena cava at each vertebral level. They may be damaged by

traction on the cava and should be identified and ligated if

the cava needs to be mobilized. If a lumbar vein is avulsed,

compression should be applied to the cava above and

below. The cava is then rolled medially and the ostium

clamped with Allis clamps. The defect is then closed with a

vascular suture. The proximal end of the lumbar vein can

retract back into the psoas muscle leading to troublesome

hemorrhage. If the vein cannot be grasped with a clip, the

area of hemorrhage is oversewn.

2 Right gonadal vein entering anterolateral surface of

the vena cava. Similarly, this thin-walled vein is at risk

during mobilization or traction of the cava and avulsion

can be repaired as described above.

3 Lumbar veins drain into the left renal vein just lateral

to the aorta and into the vena cava close to the entry of

the right renal vein.

4 Right adrenal vein draining into the vena cava.

Venous injury is less commonly encountered if dissection

is undertaken in the relatively bloodless plane

immediately adjacent to the cava's wall. If the cava itself

is damaged, repair is best effected with pressure above

and below the tear, using Allis or Satinsky clamps to

facilitate the placement of a vascular suture.

Hemorrhage can also rarely be encountered as a result

of splenic or hepatic injury. Traction is the most common

mechanism of injury to the spleen and this can be

prevented by taking down the lienorenal and splenocolic

ligaments early in the procedure. Small, superficial

tears in either organ can be controlled with pressure and

application of hemostatic gauze (Surgicel). Deeper lacerations

may require repair with mattress sutures, over

Surgicel bolsters, if necessary. More extensive damage to

the spleen can be managed by placing it in a bag or mesh

to apply external pressure. Splenectomy is rarely necessary

and only used as a last resort.

Bowel injury

The duodenum is particularly at risk in right nephrectomy

and colon can be damaged on either side. Careful

mobilization (Kocherization) of the duodenum reduces

50 Part III Open Surgery of the Upper Urinary Tract

the risk of unwitting injury from retraction or diathermy.

If the duodenum is breached, it should be

sutured directly, after debriding the area in the case of

diathermy injury. The same holds for colonic injury,

with a defunctioning stoma reserved for large or severely

contaminated injury.

Pancreatic injury

If recognized intraoperatively, injury to the tail of pancreas

is best managed with partial amputation to avoid

pancreatic fistula.

Pneumothorax

The pleura is not infrequently breached, both deliberately

and inadvertently, during nephrectomy. Small defects

can be closed with running sutures taking care to avoid

the lung. Once the sutures are loosely in place, the lung is

inflated to push out the fluid and air that has accumulated

in the pleural space, before tightening the suture line. This

process can be facilitated by placing a tube in the pleural

space, which is removed when the lung is fully inflated.

Larger defects are closed as fully as possible, leaving chest

drain in situ. A postoperative chest radiograph should be

taken to confirm resolution of the pneumothorax.

Early postoperative complications

(30 days)

Pancreatic fistula

This presents in a similar fashion to acute pancreatitis

and with fluid discharge from the wound. US or CT

scan reveals a retroperitoneal collection and fluid analysis

shows high pH and amylase. Treatment is done by

percutaneous drainage to prevent pseudocyst formation.

The majority of fistulae close but extended periods of

drainage and dietary support may be required.

Ileus

More commonly encountered following transabdominal

incisions, most cases will resolve with nasogastric

drainage and careful fluid and electrolyte replacement.

Although the majority are due to bowel handling, care

must be taken not to miss a more serious etiology such

as bowel injury, hemorrhage, or pancreatic fistula. Ileus

lasting more than a few days or accompanied by systemic

sepsis should be treated with suspicion.

Wound infection and dehiscence

Superficial wound infections are managed by opening

superficial layers and appropriate dressings. Unless

accompanied by systemic infection or spreading cellulitis,

many do not require use of antibiotics. Wound dehiscence

is rarely encountered with modern suture materials

and wound closure techniques and generally reflects

poor surgical technique. Deep wound dehiscence, particularly

in transabdominal incisions, requires early surgical

intervention but most can simply be resutured.

Chest infection

Atelectasis is common after nephrectomy, particularly

with flank incisions. Both the operative and nonoperative

sides can be affected by the surgery itself and the

flexed intraoperative position. The risk of chest infection

is increased by inadequate postoperative analgesia,

leading to poor chest expansion, expectoration, and

mobilization.

Secondary hemorrhage and hematoma

The presentation depends on the briskness of the bleed.

Severe hemorrhage is usually obvious, presenting with

signs of shock and abdominal distension. Dry wound

drains do not rule out the diagnosis and the hemoglobin

does not necessarily fall in the acute phase of

bleeding. Immediate surgical intervention is required in

conjunction with resuscitation with intravenous fluid

and blood. Slower hemorrhage may be less obvious and

lead to hematoma formation, particularly in retroperitoneal

incisions. Abdominal distension, abdominal wall

bruising, and fall in hemoglobin are common findings.

Treatment can be conservative or by radiological or surgical

drainage depending on the extent of the collection.

Renal insufficiency

Preoperative renograms are always obtained to give differential

function, so unpredicted postoperative renal

impairment is exceptionally rare following nephrectomy

for unilateral renal disease. If nephrectomy is contemplated

in bilateral renal disease, a pediatric nephrologist

is usually involved, and it may be necessary to assess GFR

formally prior to surgery. Where unexpected renal insufficiency

is encountered postoperatively, acute reversible

causes need to be actively ruled out and again, a nephrology

opinion sought.

Late postoperative complications

(30 days)

Pain

Chronic wound pain can be encountered after any incision

but is more common after loin approaches. In the

majority of cases the wound appears well healed and

there is no readily appreciable cause for the pain. In

Chapter 7 Nephrectomy 51

many individuals the pain is due to local nerve injury.

If simple analgesia is not effective, input from the pain

team should be sought.

Wound bulge

Loin incisions are frequently accompanied by postoperative

wound bulge, especially in infants. This does not

usually represent a hernia but rather a localized muscle

weakness secondary to muscle stretching or subcostal

nerve neurapraxia which tends to resolve spontaneously.

Incisional hernia can complicate any of the approaches

and when encountered, surgical repair should be considered.

Mesh can be used depending on the site and size of

the defect.

Conclusion

Most pediatric urologists will increasingly rarely perform

open simple nephrectomy. A good understanding of the

surgical technique and the issues that surround the procedure

remains important, particularly to laparoscopic

surgeons who may have to convert to an open approach

as a matter of urgency.

References

1 Kapoor R, Vijjan V, Singh K et al. Is laparoscopic nephrectomy

the preferred approach in xanthogranulomatous

pyelonephritis? Urology 2006;68:952-5.

2 Woodruff LM. Eleventh rib, extrapleural approach to the

kidney. J Urol 1955;73:183.

3 Gardiner RA, Naunton-Morgan TC, Whitefield HN et al.

The modified lumbotomy versus the oblique loin incision

for renal surgery. Br J Urol 1979;51:256.

4 Clarke BG, Rudy HA, Leadbetter WF. Thoracoabdominal

incision for surgery of renal, adrenal and testicular neoplasms.

Surg Gynecol Obstet 1958;106:363.

5 Hinman F. Atlas of Paediatric Urologic Surgery. Hinman text

book: published by Saunders (W.B.) Co Ltd (Sep 1994), pp.

135-40.

52

Partial Nephrectomy

Marc-David Leclair and Yves Héloury

Introduction

Partial nephrectomy may be performed in children

with either duplex kidney or single system. Indications

of partial nephrectomy in a normal nonduplicated urinary

tract are merely represented by renal tumors in

very selected cases, like Wilms tumor arising in a solitary

kidney, in bilateral kidneys, or in a context of predisposing

syndrome. Oncological results and complications

of nephron sparing surgery in these cases will not be

detailed in this chapter.

Duplication of the ureter and the renal pelvis is one

of the most common malformations of the upper urinary

tract. Ureteral duplication occurs with an incidence

of 0.8%, predominantly in females, and may be bilateral

in 20-40% of the cases [1]. Although most duplicated

systems remain asymptomatic, there is an increased incidence

of childhood urinary tract infections, as might

be expected with the associated increased incidence of

reflux and obstruction. Duplex kidney may also be associated

with renal hypoplasia or dysplasia, in correlation

with the abnormal location of the ureteral orifice [2].

Partial nephrectomy is a well-established treatment of

nonfunctioning moieties in duplicated renal collecting

systems. The most frequent indications for upper pole

heminephrectomy include nonfunctional upper moiety

with ectopic ureter or ureterocele. Indications for lower

pole heminephrectomy are mainly represented by damaged

lower-moieties with massive vesicoureteric reflux

(VUR), or rarely pyeloureteric junction (PUJ) obstruction

of the lower collecting system.

Outcomes from operation

Upper pole partial nephrectomy for

ectopic ureterocele

The primary treatment of ectopic ureterocele may

involve initial endoscopic decompression by intravesical

puncture, and subsequent total reconstruction with

ureterocele excision, reconstruction of the detrusor, and

reimplantation of the ureter (usually the ipsilateral lower

pole ureter) combined with partial nephrectomy of the

dysplastic upper moiety. Such trigonal reconstructions

can be challenging, especially when performed early

in infancy; therefore, a simplified approach based on a

primary upper pole heminephrectomy was developed,

considering that in most of duplex kidneys with ectopic

ureterocele, the dysplastic upper pole unit usually does

not have sufficient function to warrant salvage.

The "simplified approach" with primary upper pole

heminephrectomy was deemed to allow ureterocele

decompression, and to facilitate later staged approach to

bladder-level surgery. In some cases, it could be expected

that ureterocele decompression would obviate the need

Key points

• Partial nephrectomy in children has a low

complication rate.

• Most important complications are urinary leak

and functional impairment of the remaining

moiety.

• Very few patients will require further surgery for

treatment of a symptomatic ureteric stump.

8

Pediatric Urology: Surgical Complications and Management

Edited by Duncan T. Wilcox, Prasad P. Godbole and Martin A. Koyle

© 2008 Blackwell Publishing Ltd. ISBN: 978-1-405-16268-5

Chapter 8 Partial Nephrectomy 53

for subsequent bladder procedure. Experience with the

simplified approach suggests that the overall need for

eventual bladder surgery is very much related to the presence

of VUR at diagnosis. Partial nephrectomy alone can

be the definitive treatment in 85% of children with ectopic

ureterocele with no associated VUR [3,4]. However, it is

rare that extravesical ureterocele on duplex kidneys show

no reflux. In addition, new onset VUR may be observed in

25-40% of cases after upper tract surgery [4-6]. Conversely,

preoperative contralateral or ipsilateral lower pole VUR,

particularly when high-grade, appears to increase the likelihood

of subsequent bladder-level surgery. Husmann et al.

reported a reoperation rate of 96% with high-grade VUR

or involving more than one renal moiety [3,4]. Overall,

after upper pole partial nephrectomy, a significant proportion

of children will require subsequent ureterocelectomy

and ureteral reimplantation for definitive treatment of

persistent or new onset reflux and recurrent urinary tract

infections. The overall reoperation rate may vary between

series with ureterocele type, age at surgery, or degree

and number of renal moieties with VUR. Shekarriz and

co workers showed that almost half of patients treated with

upper pole heminephrectomy eventually require further

surgery after long-term follow-up [5].

Partial nephroureterectomy alone may result in urinary

incontinence in a small subset of patients [3]. In these rare

occasions, a large distended ureterocele may have created

an intrinsic muscular defect in the bladder neck to cause

incontinence after decompression. Exceptionally, a ureterocele

may prolapse to the perineum after upper pole partial

nephrectomy [7].

Upper pole partial nephrectomy for

ectopic ureter

Most ectopic ureters are associated with duplex kidneys

and poorly functioning upper moieties. Their surgical

management usually involves upper pole partial nephrectomy.

Rarely, the upper moiety shows enough function to

warrant conservation of the parenchyma, and a ureteropyelostomy

or ureteroureterostomy to drain the ectopic

upper collecting system into the lower system may be

appropriate [8,9].

Most duplex ectopic ureters occur in females, and those

ending distal to the external sphincter may cause incontinence.

The outcome of upper pole partial nephrectomy

performed in this context is usually straightforward, with

immediate relief of the symptom. Rarely, reflux of voided

urine into the residual ureteral stump may lead to a small

amount of dribbling incontinence after micturition or

recurrent infection [10].

Lower pole partial nephrectomy

Most of lower pole heminephrectomies are carried out

for nonfunctioning lower moieties due to renal scarring

by reflux nephropathy or associated dysplasia. Outcome

and risk of subsequent surgery usually depends on the

outcome of contralateral reflux.

Management of the retained ureteral

stump

It is generally accepted that in the absence of reflux, the

stump of the excised upper pole ureter can be left opened,

occasionally with a small feeding tube in the lumen to

ensure that the ureterocele effectively decompresses [10].

When there is associated reflux in the excised ureter (as

it is often the case after primary ureterocele endoscopic

incision), the stump should be ligated [11].

There has been much debate about the natural history

of the remaining ureteral stump after heminephrectomy

and the necessity of removing the lower part. Removal

of the defunctionalized ureter requires additional lower

abdominal incision and possible dissection into the

bladder neck and the urethra, particularly in ectopic

ureters. Moreover, despite separation of the two orifices

in the bladder, distal ureters in complete duplication are

in a common sheath and share common vasculature,

and close dissection of one of the ureters may lead to

ischemic injury of the other. On the other hand, stasis

of infected urine in the remaining stump is suspected

to increase the risk of recurrent urinary infections,

although documentation of isolated stump infection is

difficult to prove. Persad et al. showed that the ureteral

stump may behave like a bladder diverticulum and cause

symptoms mimicking pyelonephritis, and therefore

recommended that the whole ureter be excised [12]. In

the Great Ormond Street series of upper pole partial

nephrectomies performed on children detected prenatally,

the reoperation rate to deal with ureteric stump was

8%, and the authors concluded that the risk of injury to

the good ureter might outweigh the benefits of a complete

ureterectomy [13].

After partial nephrectomy for ureterocele, it is likely

that the risk for recurrent urinary tract infections related

to the stump is higher in incompletely drained ureteroceles

and refluxing ureteral stumps [6]. However, the

risk for a secondary surgery in ectopic ureteroceles does

not only depend on the fate of the ureteral stump, but

also relies on the outcome of bladder function and reflux

in the remaining lower renal unit.

After partial nephrectomy for duplex ectopic ureter,

Plaire et al. [14] reported a 12% rate of ectopic ureteric

54 Part III Open Surgery of the Upper Urinary Tract

stump excision, although most indications of secondary

procedure were related to VUR in either upper or

lower moiety. In this series, none of the ectopic ureters

presenting with incontinence required repeat surgery

[14]. Contradictory results from a study of 15 renal units

with ectopic ureter treated with partial nephrectomy

suggested that ectopic retained ureter, with or without

reflux, rarely necessitated stump removal [6]. The

removal of the lower segment of an ectopic ureter can

be technically difficult and may cause injury to bladder

continence mechanisms. Modern techniques of laparoscopic

or retroperitoneoscopic heminephrectomy offers

a unique exposition of the whole urinary tract, and allow

to carry out the dissection lower down to the bladder

level with excellent visualization. These minimally invasive

techniques should provide an excellent way to minimize

further stump-related complications without the

need for additional flank incision in ectopic ureters.

The natural history of the refluxing ureteral stump

remains to be investigated. Despite recommendations

that refluxing distal ureteric stumps should be removed

at nephrectomy [12], there is little evidence that it is

responsible for significant morbidity. In large published

series, secondary procedures to remove refluxing ureteral

stump are necessary in 5% of the cases [11,15-17]. It is

possible that the distal ureteral stump retain some peristaltic

activity that prevents urinary stasis [17].

Complications

There is very little data in pediatric urology literature

on complications of partial nephrectomies in duplex

kidneys. The postoperative course of this procedure is

usually uneventful, and its morbidity is probably much

lower than in adult surgery. Complications are rare, represented

mainly by urinary leaks and ischemic complications

of the remaining moiety.

Main published experience in the field comes from the

adult urological practice, where partial nephrectomies

are performed for renal tumor excision. The complications

observed in this context in adults are mainly urinary

fistula, infections, bleeding, and acute renal failure

when performed in solitary kidney. Up to 30% of nephron

sparing procedures can be associated with technicalor

renal-related complications, but most of them can be

satisfactorily managed nonoperatively or endourologically

and only few will require further open surgery [18].

Heminephrectomy in duplicated collected system

is obviously facilitated by the fact that there is usually

distinct segmental vascular supply with separate

branches to the lower and upper halves of the kidney. In

addition, the transected renal surface between the two

moieties is ideally a plane where no entry into the collecting

system should be necessary, hence minimizing

the risk of urinary leakage.

Urinoma

Urinoma or urinary leak is reported on very few occasions

in most published series. In our personal series of

75 heminephrectomies (30 open and 45 retroperitoneoscopic

partial nephrectomies), a calyceal breach was recognized

and sutured intraoperatively in four cases (three

open partial nephrectomies, and one laparoscopic partial

nephrectomy converted to open for suturing).

Urinoma represents an accumulation of urine around

the remaining moiety, and may be explained either by

some residual functioning parenchyma or a contained

urine leak from a small calyceal breach in the collecting

system of the remaining moiety [19] not recognized

intraoperatively. Urinary leak diagnosed postoperatively

usually remains asymptomatic and requires no treatment

as it usually resolves spontaneously [20].

There is little evidence that minimally invasive surgery

actually modifies the incidence of postoperative

urinoma. Classic principles of open partial nephrectomy

recommended preserving a strip of renal capsule,

sutured for covering of the remaining moiety. This

maneuvre, which is not routinely performed in endosurgical

procedures, was deemed to decrease the risk of

urine leak. One series reported a 20% rate of postoperative

urinoma with laparoscopy [21], but subsequent

comparative studies failed to demonstrate a clear difference

with open surgery [22]. Even if the risk of postoperative

urine leakage is slightly higher with endosurgical

procedures, this difference may not be clinically relevant

as this complication usually resolves by itself.

Cysts

Ultrasound postoperative follow-up often shows asymptomatic

residual cysts in contact with the transected

parenchyma [13,16,20,21,23,24]. This very well known

event is usually asymptomatic. In a series of 60 open

heminephrectomies, Gundeti et al. reported that such

cysts could be observed in up to 18% of the cases [25],

and half of them were still present more than 2 years

after surgery. Percutaneous aspiration, although unnecessary

in most of the cases have been reported. Results of

fluid analysis are consistent with the diagnosis of seroma,

likely secondary to disruption of lymphatics during

Chapter 8 Partial Nephrectomy 55

dissection [20]. Other explanations could be a collection

of fluid under the renal capsule, or some retained

glomeruli with no drainage system [25]. It seems that

this complication is being observed more frequently

with laparoscopic or retroperitoneoscopic approach [23]

although the reason for that remains unclear.

Ischemia/atrophy/functional loss of the

remaining pole

Basic surgical principles of heminephrectomy focus on

careful identification of both moieties vessels to prevent

unintended injury. This involves initial dissection of the

renal hilum to clearly identify the blood supply to the

moiety that needs to be kept. This dissection carries an

inherent risk of injury to the remaining pole vasculature,

leading to subsequent atrophy of the remaining renal

unit [26,27].

Even in the absence of erroneous division of vascular

branches and when no ischemic changes are noted intraoperatively,

progressive atrophy of the remaining moiety

may be observed on postoperative follow-up in up to 5%

of the cases [26,28]. Excessive traction on the kidney and

its pedicle may also be responsible for intimal injury and

subsequent thrombosis [29]. Precautions during open

surgery, with minimal traction on a kidney left in situ

could help to prevent this outcome [29]. However, this

complication is still being observed with laparoscopic or

retroperitoneoscopic approach, where mobilization of the

kidney is usually limited. In their series of 23 retroperitoneal

laparoscopic partial nephrectomies, Wallis et al.

observed a functional loss of the remaining moiety in

two patients, aged 7 and 9 months [20]. This finding

underlined that laparoscopic heminephrectomy remains

technically challenging, especially in young infants. We

had a similar experience, with one case of functional

loss of a nonrefluxing lower moiety in the postoperative

follow-up of an upper pole partial nephrectomy in a series

of 45 retroperitoneoscopic heminephrectomies [30].

This complication occurred among the first cases of our

experience, and led us to a strict policy of conversion to

open surgery when clear identification of renal vasculature

cannot be ascertained.

Renal function outcome of the remaining moiety after

partial nephrectomy has been previously reported [25].

This important study assessed changes in differential

renal function on MAG 3 or DMSA nuclear renograms

pre- and postoperatively, and showed an overall significant

decrease of 7% in renal function of the remaining

moiety, including 5/60 cases in whom decrease of renal

function was more than 10%. Possible explanations

included removal of healthy renal parenchyma, small

function attributed to the removed moiety, and intraoperative

ischemic injury to the remnant kidney.

Injury of the remaining collecting system

Clear identification of the collecting system anatomy

should be ascertained before proceeding to section of the

ureter. This is usually facilitated by an important difference

in size between the two ureters. Indeed, indications

for partial nephrectomy are mainly represented by gross

uretero-hydronephrosis of obstructive origin (upper

pole partial nephrectomy) or high-grade reflux (lower

pole partial nephrectomy). However, erroneous section

of the wrong ureter may happen and needs to be recognized

and repaired intraoperatively.

Torsion of the remaining moiety

Torsion of the remaining renal unit may occur, after

complete dissection of peritoneal attaches contributes to

abnormal mobility of the kidney in the renal bed. This

exceptional complication is similar to the torsion sometimes

occurring on renal transplants [31]. Some authors

have advocated routine nephropexy of the renal remnant,

with a suture fixing the capsule to the adjacent musculature

[16]. To our knowledge, this complication has never

been reported after laparoscopic heminephrectomy, where

freeing of the remaining pole is probably more limited.

Hemorrhage

Intraoperative bleeding is obviously an important issue in

adult renal surgery without duplication of the collecting

system. Conversely, blood loss is usually minimal in pediatric

partial nephrectomy, with most series showing intraoperative

bleeding 50 ml [23]. Usually, most blood losses

come from the transected parenchymal surface. Bleeding

originating from a nonligated ureteral stump (ureterocele)

has also been reported [32]. No difference in blood loss

has been shown between open or minimally invasive partial

nephrectomy. With the widespread use of modern section

and coagulation devices such as Harmonic Scalpel®, it

is likely that the amount of blood loss will become insignificant

in the outcome of these children.

Preventing and managing

complications

Urinary leak, cysts

Postoperative urinoma is common and usually requires

no treatment. The majority of urinary leak documented

56 Part III Open Surgery of the Upper Urinary Tract

by increased drain output will resolve spontaneously

if there is no obstruction of urinary drainage from the

involved renal unit [33]. Persistent urinary leak can benefit

from bladder drainage with a transurethral Foley

catheter [20]. In the rare event of a symptomatic urinary

fistula not resolving spontaneously, the collecting system

of the remaining moiety should be drained, ideally with

an internal ureteral JJ stent. When an internal drainage

fails to address the problem, it may be necessary to place

a percutaneous drain [32], or even to close surgically the

calyceal breach.

Some authors advocate systematic placement of a

drain in contact with the transected parenchyma at the

end of the procedure although there is no clear evidence

that it would really decrease the incidence of urinoma

formation [32] or postoperative cysts.

Injury to the collecting system

Although clear identification of ureters is usually easy

in the context of partial nephrectomy, there are few

situations where understanding of collecting systems

anatomy will be more challenging, like small ectopic

nonobstructed ureter draining a tiny upper moiety, or

lower pole low-grade reflux. In these situations, it may

be necessary to start the procedure with a cystoscopy to

insert endoscopically a ureteral stent. This stent can be

inserted either in the ureter that will need to be kept, or

in the one to be removed, with the plan to inject methylene

blue intraoperatively to facilitate identification of an

open calyx.

Inadvertent opening of the pelvis or ureter of the

remaining moiety will need to be carefully closed, and

can be drained with bladder drainage, internal JJ stent,

and/or direct suction drain.

Ischemia and atrophy of the remaining

moiety

Direct observation of ischemic changes on the remaining

moiety during heminephrectomy is a rare event, and

intraoperative decision will be difficult to make. If further

dissection shows evidence of definitive section of

the remaining pole vessels, it is likely there is no other

option than total nephrectomy. In every other situation,

the remaining moiety should be left in place and monitored

carefully.

Localized ischemia is probably relatively frequent

at the level of the transection in the parenchyma, as

the section may not be exactly performed between the

two moieties and is preferably done on the side that is

removed. Indeed, mild fever is very frequently observed

in our experience after partial nephrectomy one or two

days postoperatively and may be related to the phenomenon

of local ischemia.

Renal atrophy and functional loss of the remaining

moiety diagnosed on long-term ultrasound or functional

follow-up should not need reoperation in most of the

cases, unless a complication like arterial hypertension

occurs. Therefore, follow-up of an atrophied renal remnant

should be limited to annual monitoring of arterial

blood pressure.

Partial nephrectomy is an important procedure in the

surgical armamentarium of pediatric surgeons dealing

with complete ureteral duplication. This technique may

be technically demanding, especially with the onset of

modern minimally invasive approaches. However, there

are remarkably few complications following this procedure,

apart from the risk of injury to the remaining

moiety. The outcome of this procedure in the pediatric

population is mainly determined by the underlying condition

and the relevance of the indication.

References

1 Campbell MF. Anomalies of the ureter. In Urology, 3rd edn.

Edited by MF Campbell, JH Harrison. Philadelphia: WB

Saunders, 1970: Vol. 2, Chapter 37, pp. 1487-542.

2 Mackie GG, Stephens FD. Duplex kidneys: A correlation of

renal dysplasia with position of the ureteral orifice. J Urol

1975;114:274-80.

3 Husmann DA, Ewalt DH, Glenski WJ, Bernier PA.

Ureterocele associated with ureteral duplication and a non

functioning upper pole segment: Management by partial

nephrectomy alone. J Urol 1995;154:723-6.

4 Husmann D, Strand B, Ewalt D, Clement M, Kramer S, Allen

T. Management of ectopic ureterocele associated with renal

duplication: A comparison of partial nephrectomy and

endoscopic decompression. J Urol 1999;162:1406-9.

5 Shekarriz B, Upadhyay J, Fleming P, Gonzales R, Spencer-

Barthold J. Long-term outcome based on the initial surgical

approach to ureterocele. J Urol 1999;162:1072-6.

6 De Caluwé D, Chertin B, Puri P. Fate of the retained ureteral

stump after upper pole heminephrectomy in duplex kidneys.

J Urol 2002;168:679-80.

7 Ben Meir D, Livne PM. Prolapsed ureterocele after upper

pole heminephrectomy. Urology 2002;60:1111.

8 Mandell J, Bauer SB, Colodny AH, Lebowitz RL, Retik

AB. Ureteral ectopia in infants and children. J Urol

1981;126:219-22.

9 El Ghoneimi A, Miranda J, Truong T, Monfort G. Ectopic

ureter with complete ureteric duplication: Conservative surgical

management. J Pediatr Surg 1996;31:467-72.

10 Cooper CS, Snyder HM. Ureteral duplication, ectopy, and

ureteroceles. In Pediatric Urology, Edited by JP Gearhart,

Chapter 8 Partial Nephrectomy 57

RC Rink, PDE Mouriquand. WB Saunders, Philadelphia

2001: Chapter 28, pp. 430-49.

11 Adroulakakis PA, Stephanidis A, Antoniou A,

Christophoridis C. Outcome of the distal ureteric stump

after heminephrectomy and subtotal ureterectomy for reflux

or obstruction. BJU Int 2001;88:586-9.

12 Persad R, Kamineni S, Mouriquand PDE. Recurrent symptoms

of urinary tract infection in eight patients with refluxing

ureteric stumps. Br J Urol 1994;74:720-2.

13 Ade-Ajayi N, Wilcox DT, Duffy PG, Ransley PG. Upper pole

heminephrectomy: Is complete ureterectomy necessary?

BJU Int 2001;88:77-9.

14 Plaire JC, Pope JC, Kropp BP, Adams MC, Keating MA, Rink

RC, Casale AJ. Management of ectopic ureters: Experience

with the upper tract approach. J Urol 1997;158:1245-7.

15 De Caluwé D, Chertin B, Puri P. Long-term outcome of the

retained ureteral stump after lower-pole heminephrectomy

in duplex kidneys. Eur Urol 2002;42:63-6.

16 Mor Y, Mouriquand PDE, Quimby GF, Soonawalla PF, Zaidi

SZ, Duffy PG, Ransley PG. Lower pole heminephrectomy:

Its role in treating non-functioning lower pole segments.

J Urol 1996;156:683-5.

17 Cain MP, Pope JC, Casale AJ, Adams MC, Keating MA, Rink

RC. Natural history of refluxing distal ureteral stump after

nephrectomy and partial ureterectomy for vesicoureteral

reflux. J Urol 1998;160:1026-8.

18 Campbell SC, Novick AC, Streem SB, Klein E, Licht M.

Complications of nephron sparing surgery for renal tumors.

J Urol 1994;151:1177-80.

19 Lee RS, Retik AB, Borer JG, Diamond DA, Peters CA.

Pediatric retroperitoneal laparoscopic partial nephrectomy:

Comparison with an age matched cohort of open surgery.

J Urol 2005;174:708-12.

20 Wallis MC, Khoury AE, Lorenzo AJ, Pippi-Salle JL,

Bägli DJ, Farhat WA. Outcome analysis of retroperitoneal

laparoscopic heminephrectomy in children. J Urol

2006;175:2277-82.

21 Valla JS, Breaud J, Carfagna L, Tursini S, Steyaert H.

Treatment of ureterocele on duplex ureter: Upper pole

nephrectomy by retroperitoneoscopy in children based on a

series of 24 cases. Eur Urol 2003;43:426-9.

22 El Ghoneimi A, Farhat W, Bolduc S, Bagli D, McLorie G,

Khoury A. Retroperitoneal laparoscoic vs open partial nephroureterectomy

in children. BJU Int 2003;91:532-5.

23 Robinson BC, Snow BW, Cartwright PC, de Vries CR,

Hamilton BD, Anderson JB. Comparison of laparoscopic

versus open partial nephrectomy in a pediatric series. J Urol

2003;169:638-40.

24 Borzi PA, Yeung CK. Selective approach for transperitoneal

and extraperitoneal endoscopic nephrectomy in children.

J Urol 2004;171:814-16.

25 Gundeti MS, Ransley PG, Duffy PG, Cuckow PM, Wilcox

DT. Renal outcome following heminephrectomy for duplex

kidney. J Urol 2005;173:1743-4.

26 Decter RM, Roth DR, Gonzales ET. Individualized treatment

of ureteroceles. J Urol 1989;142:535-7.

27 Jednak R, Kryger JV, Barthold JS, Gonzales R. A simplified

technique of upper pole heminephrectomy for duplex

kidney. J Urol 2000;164:1326-8.

28 Belman AB, Filmer RB, King LR. Surgical management of

duplication of the collecting system. J Urol 1974;112:316-21.

29 Mor Y, Ramon J, Raviv G, Jonas P, Goldwasser B. A 20-year

experience with treatment of ectopic ureteroceles. J Urol

1992;147:1592-4.

30 Leclair MD, Supply E, Vidal I, Heloury Y. Laparoscopic partial

nephrectomy in infants and children. How difficult is

it? XVIIIth ESPU annual congress, April. Brugge, Belgium,

2007.

31 Roza AM, Johnson CP, Adams M. Acute torsion of the renal

transplant after combined kidney-pancreas transplant.

Transplantation 1999;67:486-8.

32 Piaggio L, Franc-Guimond J, Figueroa TE, Barthold JS,

Gonzales R. Comparison of laparoscopic and open partial

nephrectomy for duplication anomalies in children. J Urol

2006;175:2269-73.

33 Novick AC, Streem SB. Surgery of the kidney. In Campbell's

Urology, 6th edn. Edited by PC Walsh, AB Retik, TA Stamey.

Elsevier Science, Philadelphia 1992: Chapter 97, p. 2973.

58

Ureteropelvic Junction

Obstruction

Jenny Yiee and Duncan T. Wilcox

Introduction

Ureteropelvic junction obstruction is a common diagnosis

within pediatric urology, which has been increasing with the

introduction of prenatal ultrasound. Treatment options for

ureteropelvic junction obstruction encompass the urologic

spectrum. Watchful waiting, balloon dilation, endopyelotomy,

laparoscopic pyeloplasty, robotic pyeloplasty, and

open pyeloplasty are all current approaches. Controversies

in the management of ureteropelvic junction obstruction

include indication for surgical intervention versus watchful

waiting, timing of operations, surgical approach, the use of

drains, and management of complications. This chapter will

attempt to elucidate factors affecting patient outcomes and

provide an approach in the management of complications.

Surgical techniques

Kuster first described a "uretero-pyeloneostomy" as a

direct anastomosis of the ureter to the renal pelvis in

1891. In 1892 Fenger adapted for urology the Heineke-

Mikulicz, a general surgical technique for pyloric stenosis.

The Fenger technique splits a stenosed Ureteropelvic

junction (UPJ) longitudinally to close transversely. In an

attempt to achieve a smooth pelvic-ureteral transition

with minimal excess tissue, the Foley Y-plasty evolved.

This procedure advances a Y-shaped incision to close as

a V [1]. A variety of flaps then ensued such as the spiral

flap by Culp-DeWeerd [2,3], the vertical flap by Prince-

Scardino [4], the advancing V-flap by Devine [5], and

the dismembered V-flap by Diamond-Nguyen [6].

The now common Anderson-Hynes dismembered

pyeloplasty was first described in 1949 by British plastic

and urologic surgeons J.C. Anderson and Wilfred Hynes

[7]. As evidenced by the myriad of other techniques

described above, its elevation to gold-standard status was

not immediate. In its original description, an L-shaped

wedge of redundant renal pelvis was excised. The vertical

arm of the L was closed primarily while the lower arm

was anastomosed to a spatulated ureter. The original

description did not include a stent, an issue under much

current debate. In older studies, the Davis intubated

ureterotomy, Anderson-Hynes, and Prince-Scardino

reported success rates 80% [4,8] (Figure 9.1).

Outcomes

As the indication to perform a pyeloplasty is varied, so is

the definition of outcome. These can include improved

Key points

• Gold-standard Anderson-Hynes success rate

95%.

• Age, presentation, and preoperative criteria do

not affect outcome.

• Most common complications are urinary leak

and urinary infection.

• Use of stents and/or nephrostomy tubes may

decrease complications and improve outcomes.

• Urine leaks and obstruction should be managed

with immediate diversion with stent preferred.

• Failures can be treated with endopyelotomy, but

repeat pyeloplasty remains the gold standard.

9

Pediatric Urology: Surgical Complications and Management

Edited by Duncan T. Wilcox, Prasad P. Godbole and Martin A. Koyle

© 2008 Blackwell Publishing Ltd. ISBN: 978-1-405-16268-5

Chapter 9 Ureteropelvic Junction Obstruction 59

symptoms (e.g. flank pain or urinary tract infection),

improved hydronephrosis on ultrasound, stabilization of

renal function on radionuclide scan, and improved T1/2

by radionuclide scan. Large historic series consisting

mostly, but not exclusively, of dismembered repairs have

reported successful repairs in as high as 210/214 (98%)

[9] and 152/153 (99%) [10].

In a more recent and large series of dismembered

repairs, Sheu et al. [11] retrospectively reviewed 102

patients with 109 kidneys. Their average age at operation

was 21.7 months with a mean follow-up of 44 months

(Table 9.1). All patients received a perinephric Penrose

drain, but no collecting system drains were used. Four

patients (3%) required reoperation. These consisted of

repeat pyeloplasties and one nephrectomy. Renal function

improved or remained stable in 99 (97%) patients.

Preoperatively 92.5% of patients had T1/2 20 min. This

decreased to 7.5% postoperatively.

Another large series, by Tal et al. [12], retrospectively

reviewed 103 patients. The mean age at operation was 12

months with mean follow-up of 32 months. The use of

collecting system drains were left to the discretion of the

surgeon. This series reported no incidences of reoperation,

sepsis, or mortality. Decreased hydronephrosis was seen

in 83 (80.6%) patients, improved drainage by renal scan

seen in 90 (87.4%) patients, and improved or preserved

renal function by renal scan seen in 92 (89.3%) patients

(Table 9.1).

Outcomes by preoperative criteria

Whether one can predict functional improvement, i.e.

who are the best operative candidates, remains a subject

of active investigation. Zaccara et al. [13] retrospectively

reviewed 69 patients who underwent stented Anderson-

Hynes repairs. In attempting to correlate age, anterior-

posterior diameter, parenchymal thickness, glomerular

filtration rate, and differential renal function with postoperative

improvement in differential renal function, no

association could be found. The authors concluded that

improvement based on renal scan criteria was a random

event.

Outcomes by presentation

It has been shown that the manner of presentation,

whether prenatal hydronephrosis, urinary infection, or

pain, has no affect on initial differential renal function

[14]. In assessing whether the mode of presentation

affects outcome, most series agree that initial presentation

has little effect.

In the series by Sutherland et al. [15], 108 patients

1 year old presented with a prenatal ultrasound in

86 (80%) patients and urinary infection in 9 (8.3%).

Conversely the presentation of children 1 year old was

dominated by pain in 47 (48%), urinary infection in

Figure 9.1 Historic success rates from 1961 and 1982. (Adapted from Prince and Scardino [4] and Tynes et al. [8].)

0

10

20

30

40

50

60

70

80

90

100

Prince-

Scardino

Foley Yplasty

Anderson-

Hynes

Advancing Vflap

Davis

ureterotomy

Culp-

DeWeerd

Failure

Fair

Excellent/Good

Overall success

Failure Fair Excellent/Good Overall success

60 Part III Open Surgery of the Upper Urinary Tract

29 (24%), incidental in 13 (11%), and hematuria in 12

(10%). Outcomes did not differ between the two groups.

Tal et al. [12] noted that patients presenting with a urinary

tract infection versus prenatal detection were more

likely to be females and older. This was associated with

an increased complication rate, but did not affect overall

outcomes as measured by ultrasound or renal scan.

Salem et al. [16] in their series of 95 patients observed

that symptomatic patients (e.g. urinary infection,

abdominal pain, or palpable mass) showed a significantly

greater improvement than asymptomatic patients

presenting with ultrasound findings alone. This significance,

however, did not persist on logistic regression

analysis. The lack of association between outcomes and

age or presentation supports that of a prior study by

Macneily et al. [14].

A significant difference that did persist on logistic

regression analysis was renal function improvement as

predicted by preoperative differential renal scan. Salem

et al. found that patients with preoperative differential

functions of 40% were significantly more likely to

improve at least 5% than those with 40% function.

This finding is likely influenced by the fact that wellfunctioning

kidneys have little room to improve.

Outcomes by age

A consequence of increasing diagnoses by prenatal ultrasound

is the evolution of patient age. Most series prior

to the 1980s consisted exclusively of symptomatic older

toddlers and children whereas most series from the turn

of the century are now populated by asymptomatic

infants. While early studies reported higher infection

and complication rates in those 1 year old [17], more

recent studies support successful outcomes in infants.

Sutherland et al. [15] reviewed their series of pyeloplasties

from 1974 to 1994. This yielded 234 renal units

who underwent Anderson-Hynes repairs via a flank incision.

The use of stents or nephrostomy tubes depended

on surgeon preference and did not correlate to age. One

hundred and eight of these patients were 1 year old

and 119 were 1 year old. Decreased dilation on postoperative

intravenous pyelogram or ultrasound was seen in

95% in those 1 year old and 96% in those 1 year old.

They concluded that pyeloplasties were effective regardless

of age.

Woo and Farnsworth [18] reported a series of 51 patients

all 1 year old with a mean operative age of 3.7 months.

All underwent dismembered pyeloplasties with variable

Table 9.1 Modern presentations and outcomes of Anderson-Hynes dismembered pyeloplasty.

Sheu et al. [11] Tal et al. [12]

No. patients 109 103

Mean age (months) 21.7 12

Mean follow-up (months) 44 32.4

Presentation (Tal) or Prenatal ultrasound 31% 77.5%

Surgical indication (Sheu) Differential function 40% 60% N/A

T1/2 20 min 22% N/A

Urinary infection 3% 7.8%

Flank pain 14% 4.9%

Postoperative imaging Decreased/stable hydronephrosis 98% 80.6%

Improved/stable renal function 97% 89.3%

T1/2 improved 76% 87.4%

Complications Fever N/A 31.1%

Urinary infection 3.7% 12.6%

Leakage 3.7% 7.8%

Outcome Repeat pyeloplasty 3% 0

Nephrectomy 1% 0

Source: Adapted from Sheu et al. [11] and Tal et al. [12].

Chapter 9 Ureteropelvic Junction Obstruction 61

drainage tubes employed. Their success rate was 94% as

defined by improved renal scan.

Salem et al. [16] reported a series of 95 patients who

received renal scans pre- and postoperatively. They found

no difference in functional improvement in age groups

ranging from 3 months to 5 years old. As a group

about one-third of patients showed improved function

and two-thirds showed stability.

If age does not affect outcome, two conclusions can

be made. One is that surgical anatomy and technique is

unhindered in the young infant and the other is whether

prompt surgery on young infants is indicated given

equivalent outcomes at a later age.

Outcomes by delayed repair

Surgical versus nonsurgical management of ureteropelvic

junction obstruction remains controversial. Immediate

surgical repair was favored in the past; however, a study by

Ransley et al. [19] revealed the possibility of conservative

management. In this nonrandomized study, only 23% of

children with an initial differential renal function, 40%

function, managed nonsurgically eventually deteriorated

to require surgical correction. Not all patients regained

or improved their renal function after pyeloplasty. This

prompts the question whether waiting until the development

of deterioration affects overall outcomes.

In a study by Apocalypse et al. [20], 77 children were

managed surgically or with watchful waiting. Of 38 children

initially conservatively managed, 12 (32%) eventually

required surgical intervention due to deterioration

on imaging or urinary tract infections. Though this

group experienced a transient decrease in renal function,

after repair there was no difference in renal function by

DMSA scan between the conservative, early surgery, and

delayed surgery groups.

Another study by Chertin et al. [21] retrospectively

reviewed 44 patients who underwent delayed pyeloplasty

for a 5% worsening of renal function by renal scan. All

patients (100%) showed improvement in hydronephrosis

by ultrasound. Forty-two patients (95%) showed improvement

of their renal function with 36 (82%) regaining initial

levels of renal function. They found no correlation

between age, degree of hydronephrosis, or initial renal

function and subsequent improvement of renal function.

Outcomes by surgical technique

Laparoscopic, robotic, and endoscopic techniques are

discussed in other chapters of this book. In open repairs,

the Anderson-Hynes is now used almost universally. One

variable to the dismembered pyeloplasty is the choice of

flank versus dorsal lumbar incision. Though the flank

incision is more common in modern days, the dorsal

lumbar incision has historic roots, being described as

early as 1870 [22]. The dorsal lumber approach offers

exposure of the renal pelvis and hilum at the expense of

the upper pole and distal ureter.

Wiener and Roth [23] described 33 consecutive children

undergoing an Anderson-Hynes pyeloplasty.

The first 17 patients received a flank incision with the

next 16 patients all receiving a dorsal lumber incision.

Overall success rates were similar. One patient in the

flank incision required reoperation while none required

reoperation in the dorsal lumbotomy group. Overall

complication rate in the flank incision group was 22%

with 2 stent placements and 2 urinary tract infections.

The complication rate for the dorsal lumbar incision

group was 12% with stents required for an urinoma and

worsening hydronephrosis. One statistically significant

finding was the decrease in operative time in those older

than 12 months by dorsal lumbotomy.

A similar study by Kumar and Smith [22] retrospectively

examined 91 infants. The choice of incision was

surgeon dependent. The authors concluded that the

dorsal lumbotomy was superior given the significantly

decreased hospital stay (3 versus 7 days), faster time to

oral intake (48 versus 83 h), perceived superiority of

exposure, and minimal learning curve.

Both groups support the dorsal lumbar incision

based on its excellent operative time, time to recovery

and decreased hospital stay. Both groups also note

that operative and recovery time are similar to those of

endopyelotomy while providing an improved success

rate. More recent studies show even shorter hospital

stays between 2 and 3 days [24].

Complications

Prevention of complications during surgery starts with

gentle tissue handling, preserving blood supply, and providing

an adequate, tension-free anastomosis. Other factors

such as identification of a crossing vessel, drainage

tubes, and surgical approach can also affect postoperative

recovery.

Most complications are present in the immediate postoperative

period. Failures usually present within 2 years,

however failures have been described as distantly as

8 years postoperatively in the pediatric population

62 Part III Open Surgery of the Upper Urinary Tract

[25-27]. A review of literature by Smith et al. [24] compiled

833 patients from prior studies with an overall

complication rate of 13%. Urinary leakage, urinary tract

infection, and wound infection make up almost 90% of

these complications (Figure 9.2).

Complications by use of drains

The use of indwelling stents, externalized stents, or nephrostomy

tubes is heterogenous. This practice is largely

dependent on surgeon preference. In most studies, a

perinephric Penrose drain is ubiquitous. The theoretical

advantages to collecting system drains include decreased

urinary extravasation, decreased urinoma formation,

decreased obstruction secondary to postoperative edema,

ability to assess radiographically via a nephrostomy, and

optimization of alignment of the anastamosis. Those who

do not use drains cite possible higher infection rates, dislodgement

of tubes, continued possibility of obstruction or

extravasation, need for further anesthesia in tube removal,

and the questionable quality of a dry anastamosis.

Woo and Farnsworth [18] presented 54 patients, 13 of

whom received no tubed drainage, 6 of whom received

nephrostomy only drainage, and 34 of whom received

stent only drainage. Results regarding need for repeat

pyeloplasty and incidence of urinary infection and leakage

favored the stent only group.

Repeat pyeloplasties were required in 2 (15%) of the

tubeless drainage group, 1 (17%) of the nephrostomy

group, and 0 of the stented group. Similarly there were

no incidents of urinary leakage among the stented group,

but occurred in 31-50% of the other two groups. The

authors noted that while there was one stented patient

(3%) with a urinary infection, this patient did not need

further surgical intervention. Conversely, of the 4 (31%)

patients in the tubeless group who developed a urinary

infection, 50% later went on to require a repeat pyeloplasty.

This observation led to the conclusion that an

infection in the face of leakage could have devastating

consequences on anastomotic scarring. Therefore these

authors advocated a stent in all patients.

In assessing the utility of both a stent and a nephrostomy,

Smith et al. [24] presented a retrospective, nonrandomized

series of patients. Fifty-two patients had

an externalized stent and nephrostomy tube while 65

patients were tubeless.

This series found a similar overall complication rate

between stent nephrostomy versus tubeless (13% and

17%, respectively). There was a trend toward a higher

urinary tract infection rate with tubes (6%) than without

(1.5%). While no patients with tubed drainage

required repeat pyeloplasty compared to three patients

in the tubeless group, this finding did not reach statistical

significance. A review of literature presented in

this chapter demonstrated significantly more follow-up

procedures needed in the tubeless group (9%) versus the

tubed repairs (4%).

In support of the routine use of nephrostomy tubes,

Austin et al. [28] presented findings on 132 patients.

All patients underwent postoperative nephrostograms

prior to nephrostomy removal. Notably, nephrostomy

tubes were capped early in the postoperative course on

postoperative day 0 or 1. Average length of follow-up

was 2.1 years. Though 9% of nephrostograms initially

showed extravasation, all nephrostograms later showed

patent anastomoses with no subsequent obstruction.

Their rate of urinary infection was 1.5%, comparable to

other series. These authors concluded that their low rate

of complications warranted the use of a nephrostomy

tube as a means to achieve temporary diversion, perform

radiographic assessment, and reduce extravasation.

In a randomized prospective trial, Arda et al. [29] divided

patients into externalized stent versus no stent groups.

Stents were removed on postoperative day 3 and Penrose

drains on postoperative day 4, unless urine leakage

Figure 9.2 Distribution of complications by type. Total

infection rate 108/833 (13%) patients. (Adapted from Smith

et al. [24], with permission from Elsevier.)

Urinary leak 43%

Urinary infection 27%

Wound infection 19%

Obstruction 5%

Urinoma 3%

Broken stent 1% Hematoma 2%

Chapter 9 Ureteropelvic Junction Obstruction 63

was observed. In patients with Penrose drains only, drains

were removed on postoperative day 3, unless urine leakage

was observed.

Arda et al. found no difference in hospital stay, urine

leakage, or favorable results between those with an externalized

stent and those without. A persistent urinary tract

infection was present in one patient in the nonstented

arm of the study. Based on this investigation, the authors

recommend the use of a stent only in selected patients

such as those with poor renal function, severe hydronephrosis,

a solitary kidney, or a revision pyeloplasty.

Table 9.2 shows combined data from the above studies.

There exists a trend toward decreased complications

of all types and a decreased need for repeat pyeloplasties

in those with any type of tubed drainage. Though these

data are not definitive, it does suggest that tubes do not

increase complications and may even improve outcomes.

Managing complications

As described above, the most common complications

of pyeloplasties include urinary leakage, infection, and

obstruction. The ultimate goals in managing complications

are to preserve renal function and to prevent repeat

surgery. Management of certain complications is a part

of general medical knowledge. For example, most physicians

would manage urinary tract infections with cultures

and tailored antibiotic care. Management of other

complications is not as straightforward. Specifically, how

should leakage, obstruction, or malfunctioning drainage

tubes be treated in order to minimize distress to the

patient while maximizing function?

Initial management

Initial management of urinary leakage, obstruction, urinoma,

or infection focusses on relieving symptoms and

trying to prevent scar formation. Urinary extravasation,

especially in the setting of infection, is thought to promote

scar and subsequent long-term failure. Therefore

all infections should be treated promptly, especially in

the setting of extravasation.

It is also agreed upon that urine should be diverted to

limit anastomotic scarring. Nephrostomies and stents

are thought by some to be equally adequate modes

of drainage, however several authors [15,18] believe

a stent is superior in maintaining a patent anastomosis.

Sutherland et al. noted that of their patients who

developed urinary leakage or obstruction, 3 of 4 (75%)

patients treated with a nephrostomy eventually needed a

repeat pyeloplasty whereas 0 of 5 patients treated with a

stent eventually needed a repeat pyeloplasty.

After diversion and sterile urine are achieved, the

patient may be reassessed in 6-8 weeks after an adequate

period of recovery (Figure 9.3).

Definitive management

If obstruction persists after a trial of urinary diversion,

definitive management should be attempted. Options

include balloon dilation, endopyelotomy, repeat pyeloplasty,

or ultimately nephrectomy.

Anatomic factors contributing to failure include scar

tissue, a redundant pelvis, and crossing vessels that had

been missed or are new since the prior surgery. Methods

used to maximize success in reoperation vary. Some

advocate antegrade or retrograde pyelograms prior to

any reoperation [30] to define the anatomy. Others

believe CT scans before the primary or salvage surgery

are warranted to evaluate for crossing vessels. Rohrmann

et al. utilize a transperitoneal approach to gain access

to virgin tissue planes. In the cases of an ureterocalicostomy,

employment of an omental wrap can decrease

leakage and enhance blood supply. Ironically, though

the use of stents and nephrostomy tubes is variable during

primary repairs, their role in salvage repairs is more

widely accepted.

The collective results of five case series show all 37

(100%) attempted repeat pyeloplasties were successful

(Table 9.3). An additional six patients underwent successful

ureterocalicostomy. Three patients underwent

nephrectomy as the primary salvage technique due to

Table 9.2 Combined rates of complications in tubed

drainage versus no tubed drainage.

Tubed No tubed

drainage drainage

No. patients 244 94

Repeat pyeloplasties (%) 1 (0.4) 5 (5)

Overall complications (%) 17 (7) 19 (20)

Urinary tract infections (%) 6 (2) 6 (6)

Urine leak/Obstruction/ 21 (9) 13 (14)

Urinoma (%)

Source: Adapted from Smith et al. [24], Austin et al. [28],

Ransley et al. [19], and Woo and Farnsworth [18].

64 Part III Open Surgery of the Upper Urinary Tract

Table 9.3 Presenting symptoms and outcomes of recurrent ureteropelvic junction obstruction.

Thomas Lim Rohrmann Persky Sutherland

et al. [35] et al. [27] et al. [30] et al. [26] et al. [15]

No. patients 103 127 336 N/A 227

No. failed (%) 7 (93) 3 (98) 9 10 (97) 6 8 (N/A) 9 (96)

referrals referrals

Presenting Pain/obstruction 6 (86) 2 (17) 4 (25) 1 (13) 5 (56)

symptom Abnormal imaging 1 (14) 6 (50) 2 (13) 7 (88) N/A

Prolonged leak 0 3 (25) 5 (31) 1 (13) 4 (44)

Initial Stent 4 4 0 0 4

treatment Nephrostomy 1 2 10 0 5

Balloon dilation (No. successful) 5 (1) 0 3 0 0

Endopyelotomy (No. successful) 1 (0) 0 0 0 0

Success with initial treatment (%) 1 (14) 0 0 0 6 (67)

Definitive Repeat pyeloplasty 3 10 13 8 3

treatment Ureterocalicostomy 3 0 3 0 0

Nephrectomy 0 3 0 0 0

Crossing vessel found (%) 2 (33) 2 (17) 0 0 0

Percentage of successful kidney salvage 100 75 100 100 100

renal function, intraoperative findings, and family preference.

Therefore, in 42 attempted salvage repairs of

recurrent ureteropelvic junction obstruction, all were

successful.

Though open surgery is the most definitive approach,

the use of endopyelotomy or balloon dilation is attractive

given decreased morbidity. Jabbour et al. [31] reported a

series of patients undergoing endopyelotomy after failed

pyeloplasty. Though children were a part of this group,

the mean age was 35 years old. The interval between

pyeloplasty and endopyelotomy averaged 57 months.

Mean follow-up at 88.5 months revealed that 63 of 72

Figure 9.3 Initial treatment algorithm for urinary leak, obstruction, or urinoma.

Stent

PCN No

Yes No

Leak/obstruction

Already has stent/PCN?

Place stent

Not successful Successful

Attempt stent

placement

Place PCN

Yes No

Urinoma

Signs of infection?

Observe

Increasing size?

Perc drain

/ stent

Perc drain

/ stent

Chapter 9 Ureteropelvic Junction Obstruction 65

patients (87.5%) had success as defined by resolution

of symptoms and improved intravenous pyelogram

findings. All patients who failed presented within 1

year. Failures were subsequently treated with nephrectomy

(44%), repeat pyeloplasty (33%), endopyelotomy

(11%), and ileal interposition (11%). The authors did

not advocate preoperative CT scan to assess for existing

vessels, but Clayman reports that doing so has

decreased his postoperative bleed rate from 6.9% to 0%

[32]. This technique provides an acceptable success rate

while obviating the need to re-explore a scarred system.

Unfortunately, this technique has yet to be well studied

in the pediatric population.

Balloon dilation in the treatment of primary pediatric

ureteropelvic junction obstruction has reported success

rates from 47-70% [33,34]. No studies dedicated

to balloon dilation for recurrent ureteropelvic junction

obstruction exist. Combined data from Thomas et al.

and Rohrmann et al. (Figure 9.3) suggest that balloon

dilation as a treatment for recurrent ureteropelvic junction

obstruction has only a 1/8 (12.5%) success rate.

Though morbidity is minimal with this intervention, its

low success rate does not make it a definitive treatment.

Conclusion

The Anderson-Hynes dismembered pyeloplasty offers a

95% success rate for a common diagnosis in pediatric

urology. The criteria for and timing of operation are often

surgeon dependent; however, age preoperative factors,

delayed surgery, and mode of presentation do not affect

outcomes. Outcomes are affected by choice of incision

with a lumbar dorsal incision providing a shorter hospital

stay and time to gastrointestinal recovery over the more

common flank incision. The use of stents and nephrostomy

tubes is also largely surgeon dependent, though

data may suggest that complication rates decrease with

tubed urinary drainage. When complications do occur,

any urinary extravasation or obstruction should be managed

with diversion. A stent is preferred when possible

over a nephrostomy tube. Pyeloplasty failures are definitively

treated with a repeat pyeloplasty, though adult literature

suggests endopyelotomy may be an option.

References

1 Foley F. A new plastic operation for stricture at the ureteropelvic

junction. J Urol 1937;38:643-72.

2 Culp OS, DeWerd J. A pelvic flap operation for certain types

of ureteropelvic obstruction: Observations after two years'

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pyeloplasty. Urology 1981;18:235-7.

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hydronephrosis in children: A review of 219 personal cases.

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pyeloplasty. J Pediatr Surg 1980;15:133-43.

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TC. Ureteropelvic junction obstruction in children: 10

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function improvement. Scand J Urol Nephrol

2000;34:165-8.

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pyeloplasty: Outcome analysis based on patient age and surgical

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failure. J Urol 1981;125:695-7.

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67

Ureteral Reimplant Surgery

Laurence S. Baskin and Gerald Mingin

Introduction

Vesicoureteral reflux (VUR) is among the most common

problems encountered in pediatric urologic practice.

Controversies in the management of reflux include observation

versus medical therapy, versus the need for surgical

intervention. Among the latter, there is disagreement as to

the timing and type of intervention, i.e. either endoscopic

or open. This chapter will focus on the surgical treatment

of reflux including open, laparoscopic, and robotic ureteral

reimplantation. Those factors affecting the success

of the surgery as well as the potential complications and

their management will be discussed.

Who needs surgical intervention and in

what form

Observation of VUR with and without antibiotic prophylaxis

is still the most common option in the initial treatment

scheme of children with VUR. With the advent of

safe endoscopic therapy it could be argued that reflux

should be corrected, or at least that option be offered

at the time of diagnosis. This would eliminate the need

for antibacterial prophylaxis with its concomitant poor

patient compliance and potential resistance issues. We

still advocate the more traditional approach of medical

management since endoscopic intervention still requires

a general anesthetic. In addition, Benoit et al. [1] have

recently supported medical management as being more

cost effective than endoscopic treatment. A complete discussion

of the merits of open versus endoscopic correction

is beyond the scope of this chapter. It appears that

both techniques have a role in the surgical armamentarium;

however, surgeon preference and bias is also an

important factor.

Surgical techniques

Surgical treatment of reflux has evolved rapidly over

the last decade and ranges from transvesical to extravesical,

laparoscopic, robotic, and endoscopic treatments.

Regardless of the treatment options, all procedures aim

to create a flap valve mechanism where the ureter lies in

a submucosal bladder tunnel where the ratio of the tunnel

length to ureteral diameter is 5:1 or greater. The most

widely used open technique is the Cohen cross-trigonal

reimplant [2]. The ureters are mobilized transvesically

and submucosal tunnels are created so that the new hiatus

is on the opposite side. Other often-used open techniques

include the Glenn-Anderson ureteral advancement [3]

and the Politano-Leadbetter ureteroneocystostomy [4].

Key points

• Open ureteral reimplantation has a success rate

of 98%.

• Outcome is affected by a failure to recognize

underlying voiding dysfunction or neurogenic

bladder.

• Most complications are transient; ureteral

obstruction is the most serious complication and

should be managed with renal drainage.

• Persistent reflux can be treated endoscopically

or with repeat open surgery.

10

Pediatric Urology: Surgical Complications and Management

Edited by Duncan T. Wilcox, Prasad P. Godbole and Martin A. Koyle

© 2008 Blackwell Publishing Ltd. ISBN: 978-1-405-16268-5

68 Part III Open Surgery of the Upper Urinary Tract

In the former the ureter is mobilized and a submucosal

tunnel is developed toward the bladder neck. The latter

technique utilizes the creation of a new hiatus cranial to

the original one.

The technique utilized for extravesicle reimplantation

is the Lich-Gregoir or one of its modifications [5,6]. Here

an incision is made in the serosal and muscular layers of

the bladder sufficient for the ureter to be placed into the

trough and the muscle reapproximated over the ureter.

When ureteral reimplantation is performed laparoscopically

or with robotic assistance, the technique is often a

variation on the Lich-Gregoir, however, the laparoscopic

cross-trigonal transvesical technique has been performed

successfully in some centers [7]. The techniques

used for laparoscopic or robotic laparoscopic-assisted

ureteral reimplantation are similar. Transurethral cystoscopy

is performed and the bladder is distended with

saline. The camera port is placed in the dome of the

bladder using an open technique or under direct vision

after percutaneous placement of a traction suture. The

saline is removed and the bladder is insufflated with CO2

to 10-12 mmHg. Ureteral stents are placed and a crosstrigonal

reimplant is performed as described in the open

procedure.

Outcomes of surgical technique

In open transvesicle repairs the Cohen cross-trigonal

procedure is the most widely utilized with a success rate

of 98% in primary reflux [8]. For secondary reflux, the

success rates range from 89% to 95% [9,10]. The results

are similar for the Politano-Leadbetter repair [11]. With

regard to the extravesicle Lich-Gregoir the overall success

rates are 96% for both unilateral and duplicated

systems [12]. Success with laparoscopic transvesicle repair

approaches 96% [7]. Robotic ureteral reimplantation is

still in its infancy with no large series reported to date.

However, anecdotally there appears to be approximately

80% successful correction rate for unilateral reflux.

Factors affecting the outcome of surgical

correction

Persistence of VUR is the most common complication

after reimplant surgery. Although this occurrence may

be due to technical error, more often than not it is due to

overt bladder pathology or failure to recognize underlying

bladder pathology. Pathology can be in the form of

neurogenic bladder, anatomic pathology, or voiding dysfunction.

It also may be due to a large ureteral diameter

where an adequate tunnel length is not achieved.

In most cases the cause of a neurogenic bladder will

be obvious such as a myelomeningocele or spinal cord

injury. A more subtle presentation would be an older

child with an occult presentation of tethered cord. In

most cases reflux will resolve or improve by directly

decreasing bladder pressure via intermittent catheterization,

anticholinergic therapy, or bladder augmentation.

In the rare infant experiencing recurrent infection

especially with ongoing renal scarring or progressive

hydronephrosis despite adequate medical management,

vesicostomy is a temporary option that assures bladder

drainage. Anatomical causes of bladder dysfunction that

may worsen the results of reimplantation include the

presence of a ureterocele, or posterior urethral valves.

Voiding dysfunction or dysfunctional elimination by

far accounts for the majority of treatment failures. These

patients can be identified based on a history that elicits

symptoms such as infection, incontinence, urgency, frequency,

and constipation. Aggressive treatment, including

strict adherence to voiding and bowel regiments will

lead to resolution of reflux as well as associated lower

urinary tract infection. If these children have failed reimplantation

surgery, correction of the underlying voiding

dysfunction will often eliminate the need for further

intervention.

Megaureter

The management of megaureter is dependent on whether

the dilation is associated with primary obstruction or

reflux. Management also takes into account whether the

patient is asymptomatic or not.

In the absence of infection, increasing hydroureteronephrosis

or failure to demonstrate obstruction on

a nuclear scan conservative management is the rule.

Conservative management also applies to the refluxing

megaureter. If the patient is symptomatic the type of

intervention is age dependent. In children 6 months

of age or older, ureteral tailoring either excisional [13] or

tapered with intravesicle or extravesicle reimplant may

be considered [14]. The decision to tapper is based on

whether a 5:1 ratio of tunnel length to ureteral diameter

can be obtained. In younger children ureteral reimplantation

with excessive tailoring can potentially jeopardize

the vascularity of the distal ureter. The success rates vary

depending on associated obstruction versus reflux and

the approach utilized for reimplantation. Correction of

obstructed megaureters had a higher success rate than

Chapter 10 Ureteral Reimplant Surgery 69

refluxing ureters: 90% versus 74%. Ureters reimplanted

using an intravesicle technique had a higher success

rate when compared to the extravesicle technique: 86%

versus 76% [15]. This difference may be attributed to

differences in collagen to smooth muscle ratios at the

ureterovesicle junction.

If the child is not a candidate for definitive correction

due to age/size, nephrostomy tube placement or cutanous

ureterostomy are possible options. Nephrostomy tubes

are not well tolerated in children and placement has lead

to a significant complication rate (4-8%) [16] including

hemorrahage, septicemia, and puncture of the pleura

or peritoneum and for this reason cutanous ureterostomy

often is a better option. In this procedure the distal

ureter can be dissected off of the bladder and brought

out to the skin through a small Pfannenstiel incision.

This allows for decompression of the ureter. Infection

and stomal stenosis are associated complications [17].

Recently, the use of a refluxing ureteral reimplant in

children with megaureter has been described. In this

procedure, the ureter is implanted as to allow free reflux

of urine. The advantage is that obstruction is traded for

reflux [18].

Preventative measures to avoid

complications

There are a number of technical precepts, which if

followed will help to ensure a successful result. Chief

among these is adequate detrusor muscle backing.

Whether an open, laparoscopic, or robotic approach is

used in the submucosal tunnel or trough length should

be at least 4-5 times the ureteral diameter. It is important

that the chosen technique be done in such a fashion

that avoids excessive trauma to the tissue in order to prevent

secondary obstruction.

Thus, gentle tissue handling cannot be overemphasized.

This includes careful dissection of the ureter from

the bladder so as to avoid devascularization of the distal

ureter. The mucosa of the orifice should not be touched

thereby preventing edema. The judicious use of retraction

sutures facilitates minimal touch techniques. These

sutures will also allow the surgeon to maintain proper

orientation and avoid torsion of the ureter.

Importance is placed on a tension-free anastomosis,

while avoiding placement of the new hiatus laterally in

order to prevent kinking of the ureter. The anastomosis

should be fixed securely. We advocate placement of

a suture through bladder mucosa and muscle taking

ureteral serosa and mucosa to ensure that the ureter does

not retract into the submucosal tunnel. Finally, the original

hiatus is closed to avoid a bladder diverticulum, taking

care not to occlude the ureter.

Two additional technical points are controversial, the

need for routine ureteral stenting, and the placement of

a penrose drain in the space of Retzius. Stenting should

at least be considered when the distal ureter has been

transected or excessive handling has occurred and in

infants and those with a thickened and scarred bladder.

Stenting the ureter may be advantageous in a laparoscopic/

robotic approach were the stiffness could help with easy

identification of the ureter. The placement of a penrose

drain has been advocated for the prevention of urinary

extravasation [19] but equally good results have been

reported calling into question the necessity of drain

placement [20]. We do not advocate routine perivesicle

drains. In the rare event of leakage from the suture line

a urethral catheter can be placed and re-exploration

therefore is rarely necessary.

Complications

Operative complications are divided into two groups,

those that occur in the immediate postoperative period

and those that can occur up to several years out.

Early complications

Early complications occur in the first few days postsurgery

and are usually transient. They include low urine output,

hematuria, bladder spasm, retention, voiding dysfunction,

and infection. Preoperative dehydration, obstruction at

the level of the ureter (edema) or bladder outlet (clot or

catheter balloon) cause decreased urine output. Initially,

the position of the catheter and absence of clots should be

assured with gentle bladder irrigation. Inadequate hydration

usually is then uncovered as the culprit. Prevention

of this problem is paramount by assuring vigorous hydration

during the intraoperative period followed by 12 h

of fluid replacement at 1.5 times maintenance. Often

despite such aggressive fluid replacement, a fluid bolus of

10 ml/kg of isotonic saline may need to be administered.

If anuria or oliguria persists beyond 24-48 h despite the

above interventions, a renal bladder ultrasound should

be performed to look for urinary extravasation or

obstruction. Extravasation may be treated initially with

prolonged Foley catheter drainage, whereas complete

obstruction requires placement of ureteral stents or nephrostomy

tube drainage. Placement of ureteral stents such

70 Part III Open Surgery of the Upper Urinary Tract

as feeding tubes in the newly positioned ureteral orifices

often will require reopening the incision and bladder

with open ureteral catheterization especially if the crosstrigonal

technique has been used. Since this is typically

within 48-72 h of the original surgery, little wound healing

has taken place and the skin, fascia, and bladder incision

can be retraced with little effort. The new orifices

are typically edematous but will always accept a stent

initiating a brisk postobstructive diuresis. Nevertheless,

placement of nephrostomy tubes avoids a fresh incision

but has its own disadvantages. Nephrostomy tubes may

require more intensive postoperative care to prevent dislodging

and can be more uncomfortable for the child.

They do offer an option for antegrade stent placement in

the rare occasions where this may be necessary.

Hematuria and bladder spasms are seen with open

surgery and usually resolve within a week of surgery.

These symptoms can be distressing; however, reassurance

and selective use of anticholinergics are all that is

necessary.

Transient voiding dysfunction has been reported in

open surgery, whether this will hold true for robotic and

endoscopic treatment remains to be seen. Symptoms

include urge incontinence and nocturnal enuresis due

to inflammation, which resolves over several weeks.

Patients who have a bilateral extravesicle repair are at

an increased risk for postoperative voiding dysfunction.

Inefficient bladder emptying, requiring intermittent

catheterization was seen in 26% of patients after bilateral

reimplantation [21]. However, spontaneous voiding

resumed in all of these patients within 1 month. Patients

with a previous history of voiding dysfunction must be

encouraged to continue their voiding regiment.

It is not uncommon for patients to develop a febrile

urinary tract infection after surgery. This can be avoided

by obtaining a preoperative urine culture. Since a preoperative

urine culture is not always practical a urinalysis

or urine dip the morning of surgery will alert the physician

to the possibility of infection. Regardless, perioperative

antibiotic prophylaxis should be administered and at

discharge prior suppressive antibiotic regimens resumed.

Late complications

Ureteral obstruction is the most common late complication.

As mentioned previously, anuria or oliguria

persisting beyond 48 h mandates imaging to rule out

obstruction. Obstruction in open surgery is caused by

kinking due to excessive angulation or devascularization

of the distal ureter and occurs in 2-8% of cases, 8% of

European cases in IRS [22].

Avoidance of placing the new hiatus to laterally on the

bladder wall and care in closing the old hiatus should

avoid this problem. There are two additional circumstances

were obstruction is prone to occur. During an

extravesicle reimplant if a ureteral advancement suture is

placed, care must be taken to avoid excessive angulation

(Personal communication of Gerald Mingin). In the case

where the Politano-Leadbetter procedure is utilized the

ureter must be freely mobilized off the peritoneal reflection

to a distance of 6-8 cm. Failure to adequately mobilize

the ureter can also lead to excessive angulation. It is

recommended that this part of the procedure be done

under direct vision to avoid perforating the peritoneum,

ileum, or colon [23].

Complete obstruction necessitates nephrostomy tube

and/or ureteral stent drainage. Although there is the occasional

anecdotal report of resolution up to a year, most

patients will require reoperation. It is not unusual to see

temporary resolution with continuous Foley catheter

drainage. Angulation may not occur in the decompressed

bladder and is seen only with filling. However, this treatment

should not be a replacement for surgical revision.

Table 10.1 Treatment of anuria/oliguria.

Anuria/Oliguria Flush urethral catheter

Fluid bolus of 0.45 normal saline

Persistence beyond Renal bladder ultrasound

48 h

Urine extravasation Continued urethral catheterization

Hydroureteral Obstruction mandates placement

nephrosis of ureteral stents or a

percutanous nephrostomy tube

Long-term obstruction Redo-reimplantation

Postoperative reflux

Postoperative reflux may be due to persistent reflux in the

reimplanted ureter(s) or new onset contralateral reflux.

For open bladder surgery the reported incidence is up to

1.5% [24]. For unilateral extravesicle reimplantation this

number is reported to be as high as 5.5% [12]. In almost

all cases observation is the preferred treatment as spontaneous

resolution occurs over time. Rarely in the case of

ureterovesical fistula continued reflux warrants reoperation

with excision of the ureter distal to the fistula.

New onset contralateral reflux is observed in 19%

of open unilateral reimplants [25] and with Deflux

Chapter 10 Ureteral Reimplant Surgery 71

injections as well. Resolved contralateral reflux is a risk

factor for new onset occurrence and is seen in 45% of

patients where only a single side is surgically corrected

[26]. The majority of these patients will resolve over time

and observation is recommended. Most recommend that

at the time of the procedure bilateral treatment can be

performed even if one side has resolved spontaneously.

In a recent study of patients undergoing unilateral extravesicle

reimplantation contralateral reflux developed in

5.6% of patients with complete resolution in all patients

by 31 months [27]. Redo surgery after observation has a

high success rate.

However, great care must be exercised to avoid

ischemic ureteral injury. The surgeon must be prepared

to deal with a paucity of ureteral length. In these cases a

psoas hitch may prove useful if further length is needed,

a Boari flap can provide up to 14 cm in patients with

adequate bladder capacity [28]. In almost all situations

where length is compromised a transureterostomy can

be performed avoiding the need for bowel interposition

with the exception of history of stone disease.

Transureteroureterostomy has proved highly successful

in patients with failed ureteral reimplant surgery [29]. In

the case of a persistently dilated ureter, ureteral tapering

should be performed.

Finally, subureteral injection of dextranomer/hyaluronic

acid has been proposed as an alternative to open surgery

for failed ureteroneocystotomy. The success rate is 70%

with a single injection, but has been reported to ultimately

reach 100% after the second injection [30]. Endoscopic

treatment of VUR is discussed in Chapter 15.

Suggested follow-up postreimplantation

Patients should be kept on prophylactic antibiotics until

postoperative studies have been verified. A standard protocol

would include obtaining a renal ultrasound 4-6 weeks

postsurgery to ensure the absence of obstruction. Mild

dilation is expected due to transient edema. Moderate

hydronephrosis may be a sign of significant obstruction

and would require further testing. It is important that

preoperative cystogram be checked as we have often seen

de novo severe hydronephrosis in those with massive

dilatation of the collecting system with a preoperative

normal ultrasound. In case where obstruction must be

excluded, we prefer a nuclear lasix renogram to confirm

these findings. If standard open or extravesicle reimplantation

is performed especially for nondilating reflux, we

do not obtain a postoperative cystogram in light of the

success rate of these techniques. If the surgery is performed

robotically or endoscopically, the success rate is

not as yet predictable in most surgeons' hands and hence

a voiding urethral cystogram 3-4 months postsurgery is

recommended until results approaching those of open

surgery are achieved.

Decades long prospective studies on the outcome of

patients treated for VUR in childhood are lacking. A recent

retrospective study with an average follow-up time of 35

years looked at the outcomes in kidneys with no scaring,

unilateral scarring, and bilateral scarring. Information

on renal function was available on 55% of patients. Mild

renal damage (GFR 60-89 ml/min/1.73 m2) was found in

64% of patients. This was true of patients with either unilateral

scarring or no scarring. There was also an increased

tendency for hypertension in those patients with scarring.

Finally, a total of 83% of patients with bilateral scarring

had lowered kidney function, a quarter presented with

proteinuria and half with hypertension [31].

Screening for late occurring complications of VUR is

performed yearly and includes measurement of blood

pressure and a urinalysis to look for hypertension and

infection. Normal values for spot urine protein are

inconsistent. Based on the above measurement of urine,

protein is more likely to be of benefit in those individuals

with bilateral scarring.

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23 Tocci PE, Politano VA, Lynne, CM, Carrion HM. Unusual

complications of transvesicle ureteral reimplantation. J Urol

1976;115:731-5.

24 The American Urological Association pediatric vesicoureteral

reflux clinical guidelines panel. The Management of Primary

Vesicoureteral Reflux in Children. Baltimore, MD: American

Urological Association, 1997.

25 Hoenig DM, Diamond DA, Rabinowitz R, Caldamone AA.

Contralateral reflux after unilateral ureteral reimplantation.

J Urol 1996;156:196-7.

26 Ross JH, Kay R, Nasrallah P. Contralateral after unilateral

reimplantation in patients with a history of resolved contralateral

reflux. J Urol 1995;154:1171-2.

27 Minevich E, Wacksman J, Lewis AG, Sheldon C. Incidence of

contralateral reflux following unilateral extravesical detrusorrhaphy

(ureteroneocystotomy). J Urol 1998;159:2126-8.

28 Aronson W. Complications of Ureteral Surgery: Management

and Prevention in Complications of Urologic Surgery.

Philadelphia: W.B. Saunders, 2001.

29 Hendren WH, Hensle TW. Transureteroureterostomy: Experience

with 75 cases. J Urol 1980;123:826.

30 Jung C, DeMarco RT, Lowrance WT, Pope JC, Adams MC,

Dietrich MS et al. Suberteral injection of dextranomer/

hyaluronic acid copolymer for persistent vesicoureteral reflux

following urteroneocystostomy. J Urol 2007;177:312-15.

31 Lahdes-Vasama T, Niskanen K, Ronnholm K. Outcome of

kidneys in patients treated for vesicoureteral reflux during

childhood. Nephrol Dial Transplant 2006;21:2491-7.

73

Ureteroureterostomy

Job K. Chacko and Martin A. Koyle

Introduction

Ureteral duplication anomalies with complete ureteral

duplications can present in many different ways.

Vesicoureteral reflux (VUR) often associated with the

lower pole can be seen in conjunction with an upper

pole ureterocele and/or ectopic upper pole ureter. There

are a number of ways to surgically approach this including

incision/excision of the ureterocele, upper/lower pole

heminephrectomy, pyelopyelostomy, or common sheath

reimplant. One potentially underutilized technique is the

ipsilateral ureteroureterostomy (U-U). Evidence in the literature

suggests that U-U can be used with high success

with minimal morbidity and complication.

Another use of U-U is transureteroureterostomy (TUU)

for salvage procedures as well as diversion/undiversion.

This chapter will address these surgical techniques and

attempt to troubleshoot complications and provide steps

for management.

Surgical techniques

Ipsilateral ureteroureterostomy

The initial use of U-U was first described by Buchtel in

1965 [1]. The patient is placed supine on the operating

table. Most cases can be performed through a modified

Gibson incision or Pfannenstiel incision. If cystoscopy is

necessary for stent placement into recipient ureter, this

can be done with dorsal lithotomy and fluoroscopy. The

ureteral complex is located as it passes below the obliterated

umbilical artery and the ureters are separated

above the common distal blood supply. The donor ureter

is then transected and ligated distally if necessary. The

recipient ureter is then opened lengthwise to match the

diameter of the donor ureter. The ureteral anastomosis is

performed end-to-side with 7:0 polydioxane (PDS)

absorbable suture. An indwelling stent can be placed

prior to the anastomosis. Ureterocele excision and recipient

ureter reimplant can be performed through this same

incision. After the surgery is finished, the incision is closed

and a Penrose drain can be brought through the incision.

Transureteroureterostomy

The technique for TUU involves greater exposure

because of the need to mobilize the donor ureter to the

recipient ureter for the anastomosis. This usually involves

Key points

• Ureteroureterostomy is a safe, effective

procedure for managing ureteral duplication

anomalies.

• Urinary drainage with stents and/or drains

is crucial for preventing postoperative

complications.

• VUR can develop or persist after surgical

intervention and usually can be managed

conservatively.

• Problems with anastomosis patency are

uncommon.

• Transureteroureterostomy can be a

viable option for salvage procedures and

diversion/undiversion.

11

Pediatric Urology: Surgical Complications and Management

Edited by Duncan T. Wilcox, Prasad P. Godbole and Martin A. Koyle

© 2008 Blackwell Publishing Ltd. ISBN: 978-1-405-16268-5

74 Part III Open Surgery of the Upper Urinary Tract

a larger, midline transperitoneal incision. The posterior

peritoneum is incised to expose the retroperitoneum and

access to the ureters. The bowel is mobilized cephalad to

provide maximum exposure. The donor ureter is then

mobilized and ligated as distal as possible, taking care to

preserve the adventitia. The gonadal vessels often have

to be ligated for mobilization. The donor ureter is then

brought across to the recipient ureter above or below

the inferior mesenteric artery - this decision is based

intraoperatively on which will bring the donor ureter to

the recipient ureter off tension for a good anastomosis.

An end-to-side anastomosis is performed similar to the

ipsilateral U-U. An indwelling stent is placed prior to

completion of the anastomosis. External drainage with

Penrose or Jackson-Pratt drains can be placed to monitor

urinary leak from the anastomosis.

Outcomes from operations

Outcomes from ipsilateral U-U have historically been

very good. The largest series by Lashley et al. [2] of 100

ureteroureterostomies had an average patient age of 28

months with a hospital stay of 4.6 days. The anastomoses

patency was 94% and they found that the most common

complication was prolonged Penrose drain output.

Of 13 patients with prolonged drain output, one required

percutaneous drainage of the kidney, and one required

percutaneous drainage of urinoma. Other complications

included fever of unknown origin (2) and blood transfusions

(2). In addition, one patient each had ileus, retained

drain, gastroenteritis, febrile urinary tract infection (UTI),

and pneumonia. Six patients were considered failures.

Three patients had U-Us requiring revision secondary to

obstruction. Two patients had VUR that subsequently

underwent ureteral reimplants, and one patient had a

nondraining ureteral stump that required excision. None

of the complications were seen in these six patients with a

failed procedure.

Another series from Chacko et al. [3] of 41 U-Us had

an average age of 31 months and hospital stay average

of 1 day. The patency rate was 100%. Complications

seen were de novo VUR in two patients that had U-U

alone. One patient underwent ureteral reimplant, and

the other underwent subureteric injection. Two patients

with U-U and concomitant common sheath reimplant

had persistent VUR that was treated with observation

in one patient and subureteric injection in the other. In

children having concomitant U-U and reimplant, two

patients without indwelling stents developed transient

postoperative urinomas that required subsequent drainage.

Another patient presented with transient ipsilateral

urinary obstruction that required percutaneous drainage

that resolved spontaneously.

Bieri et al. [4] reported on 24 U-Us with an average

age of 4 years and a hospital stay of 3 days. Complications

noted were UTI in one patient and U-U revision for prolonged

drain output. Long-term patency rates were 100%.

Jelloul and Valayer [5] performed 19 U-Us on patients

with a mean age of 3.5 years that averaged 6 hospital day

stays. They did not note any complications at an average

follow up of 38 months.

U-U can be used as a primary and a salvage procedure.

Choi and Oh [6] reviewed their management of ureteral

duplication pathology. Eighteen U-Us were performed of

which 13 were primary U-Us. Five U-Us were performed

as salvage procedures for prior failed approaches in

which all succeeded. They noted that U-U had the lowest

failure rate of the different techniques they used. Overall

they observed an 89% success rate for U-U. Two patients

with primary U-Us required intervention. One patient

underwent remnant ureterocele excision, while another

had a ureterocele excision with ureteral reimplant for

VUR with infection.

Bochrath et al. [7] reported on 13 U-Us on patients

ranging from 2 to 14 years. The hospital stay ranged from

2 to 10 days with a median of 3 days. Complications

noted were VUR into three ureteral stumps, and persistent

VUR in one patient. The three patients with ureteral

stumps needed no further intervention as well as the

patient with persistent VUR.

Outcomes for ipsilateral U-Us are displayed in

Table 11.1.

Outcomes for TUUs in pediatrics have been reported

by a number of groups. An early study reviewing

TUU in children was performed by Halpern et al.

[8]. They performed TUU in 38 children - 14 TUUs

alone and 24 TUUs with cutaneous ureterostomy. The

majority of patients had underlying bladder pathology.

Complications included avulsion of the TUU resulting

in eventual death, two patients with ureteral necrosis

resulting in ileal conduit diversions.

Hodges et al. [9] reviewed their 25 year experience

with 100 patients. The age ranges were 1-83 years. Two

cases resulted in postoperative death from other sources

of pathology. Complications included prolonged urinary

drainage (2), inferior mesenteric syndrome (2), anastomotic

disruption (1), VUR (1), acute pyelonephritis (5).

Hendren and Hensle [10] described experience with

75 cases of TUUs. The main indications were for failed

Chapter 11 Ureteroureterostomy 75

ureteral reimplants or urinary undiversion. They noted

no deaths, anastomotic leaks, or nephrectomies. Three

patients developed reoperative complications. One

patient required a revision for an anastomosis that was

too anterior on the recipient ureter, causing excessive

angulation. Another patient had a kink in the recipient

ureter distal to the anastomosis that required resection.

The last patient needed revision of a bowel segment

used to drain a donor renal pelvis into a recipient ureter,

which was subsequently moved to drain into the opposite

recipient renal pelvis.

Rushton et al. [11] used TUU for 31 patients for urinary

diversions/undiversions and failed ureteral reimplants.

They noted neurogenic bladders in 26 patients and

4 others with bladder pathology. Complications included

two stent placements for transient obstruction and ureterocutaneous

drainage. Another patient developed

obstruction after tapered reimplant requiring cutaneous

ureterostomy. Lastly, one patient developed a large urinoma

due to ischemic necrosis of the upper ureter that

resulted in nephrectomy. Two complications including

partial small bowel obstruction and vesicocutaneous fistula

both resolved without intervention. There were no

complications with any of the TUU anastomoses.

A review of 69 TUUs by Mure et al. [12] for multiple

indications, for salvage and reconstruction, for diversion/

undiversion noted a low complication rate. Three

patients required reoperation - one for postoperative urinoma

and the other for common ureteral trunk ischemia

that required separate ureteral reimplantation into the

existing sigmoid conduit. The last patient required donor

nephrectomy for deterioration and infection. No complications

with the TUU anastomoses were noted.

Lastly, Pesce et al. [13] had 70 patients requiring

TUUs with the majority (97%) for salvage procedures

for failed ureteral reimplants. Complications included

Table 11.1 Outcomes of ipsilateral ureteroureterostomies.

Study Number Age Hospital stay Follow-up Success Complications

rate (U-U

patency) (%)

Chacko et al. [3] 41 31 months 1 day 3-34 100 VUR (4); urinoma (2);

months transient obstruction (1)

(average

12 months)

Lashley et al. [2] 100 28 months 4.6 days 2.5-24 94 Prolonged Penrose output

months (13) - one required

percutaneous nephrostomy

tube, one required

percutaneous drain;

U-U obstruction (3); nondraining

stump with UTI (1);

fever (2); ileus (2); VUR (2);

retained drain (1); gastroenteritis

(1); pneumonia (1);

no yo-yo reflux

Choi et al. [6] 18 0-12 years - Median 89 VUR (1) required reimplant;

7.6 years excision ureterocele for UTI (1)

Bieri et al. [4] 24 4 years 3 days 41.4 months 100 UTI (1); U-U revision (1); no

yo-yo reflux

Jelloul et al. [5] 19 3.5 years 6 days 38 months 100 UTI (1); VUR (2); no yo-yo reflux

Bochrath et al. [7] 13 2-14 years 3 days 55 months 100 VUR stump (3); VUR (1); no

yo-yo reflux

VUR, vesicoureteral reflux.

76 Part III Open Surgery of the Upper Urinary Tract

one temporary obstruction requiring stent placement

and one distal obstruction of the recipient ureteral reimplant

treated with balloon dilation. Four patients with

neurogenic bladders developed complications unrelated

to the TUUs. There were no complications related to the

TUU anastomoses. Results are shown in Table 11.2.

Complications

Reported complications with ipsilateral U-U are depicted

in Figure 11.1. The most common problems were

prolonged drain output and VUR, whether de novo or

persistent versus VUR into the stump. Few of the patients

that had prolonged drain output required intervention

and were managed conservatively until it resolved.

VUR after surgery invariably is lower grade and often

can be managed with conservative measures including

subureteric injection and observation. Kaplan et al.

[14] reported on conservative management of patients

with complete duplication and VUR and of the observed

group, 48% had resolution of VUR. In addition, the high

success rates of subureteric injection with lower grade

VUR can and has been applied to VUR seen after U-U.

Table 11.2 Outcomes of transureteroureterosotomies.

Study Number Age Salvage/ Follow-up Success rate Complications

diversion- (U-U patency)

undiversion (%)

Pesce et al. [13] 70 2-13 years 68/2 4-21 years 100 Temporary obstruction

(average requiring PNT (1); distal

10.8 years) obstruction requiring dilation

(1); 4 patients with neurogenic

bladder: progression of pre-exisitng

renal disease (2); Renal stones

donor pelvis (1); distal ureteral

stenosis requiring reimplant (1)

Mure et al. [12] 69 1 month 22/47 Median 6 100 Urinoma (1); ureteral trunk

to 21 years years ischemia (1); nephrectomy

(mean 8.6 donor kidney (1)

years)

Rushton et al. [11] 31 5 weeks 8/23 1 year 100 Transient obstruction requiring

to 17 years stent (1); obstruction tapered

reimplant (1); ureterocutaneous

fistula requiring stent (1);

nephrectomy (1); partial small

bowel obstruction (1);

vesicocutaneous fistula (1);

30 patients with neurogenic

bladder or bladder pathology

Hendren and 75 Newborn to 35/40 - 98.6 Angulation at U-U (1); common

Hensle [10] 36 years stem obstruction (1); dilated

bowel to ureter problem (1)

Hodges et al. [9] 100 1-83 years 22/78 1 year 99 Prolonged urinary drainage (2);

inferior mesenteric syndrome

(2); U-U disruption (1); VUR

(1); pyelonephritis (5)

Halpern et al. [8] 38 4 months to 7/31 - 97 U-U avulsion resulting in death

10 years - (1); ureteral necrosis

requiring ileal conduit (2)

Chapter 11 Ureteroureterostomy 77

Lastly, VUR into the remaining stump after U-U can be

minimized by dissecting the stump to the level of the

bladder, which can be easily accomplished with the low

incision used for U-U.

Obstruction of the U-U anastomosis was uncommon

and in cases where revision was necessary, it was easily

accomplished with 100% success after the revision.

Urinoma formation was relatively uncommon and the

chance for leakage from the anastomosis can be minimized

with drainage. All studies advocated using some

form of urinary drainage, whether it was urinary stent,

drain, or both.

One concern many have with U-U is potentially leaving

behind a dysplastic upper pole. Smith et al. [15]

compared U-U versus upper pole nephrectomy. They

found that 9.5% of patients on pathology of the upper

pole had evidence of marked dysplasia and half of the

patients had no dysplasia or insignificant evidence of

pyelonephritis. The decision for U-U was based on the

appearance of the upper pole intraoperatively, and if it

appeared healthy and U-U was technically feasible, it

was performed. Husmann [16] reviewed consequences

of leaving tissue behind in multicystic dysplastic kidneys

and duplicated dysplastic segments and found them very

rarely associated with urinary infection, hypertension,

and renal tumors.

Another concern is ureteral disparity during U-U

anastomosis and the potential for yo-yo reflux between

the two ureters. This appears to be an academic concern

and no investigators have reported any problems with

ureteral disparity from donor to recipient ureter and no

instances of yo-yo reflux have occurred.

Ureteral anastomosis complications with TUU are

uncommon and all studies have shown excellent success.

Since the TUU is mainly used for salvage and more complex

reconstructive and creative surgeries, the chance

for complications invariably increases. Many cases are

reoperative procedures from the beginning and often

the patients have other comorbidities that can increase

the risk for complications. The most important point is

to anticipate problems and have a higher suspicion for

complications postoperatively.

Preventing complications

Preoperative

Preoperative evaluation is crucial because of the

variety of anomalies seen in complete ureteral duplication.

Information that is necessary consists of presence

or absence of ureterocele, location of the insertion

of the upper pole moiety and is it ectopic, obstructed,

or refluxing. In addition, lower pole VUR and contralateral

VUR need to be evaluated. Also, the character of

the upper pole parenchyma is important as well. Most of

these answers can be elucidated by a renal/bladder ultrasound

(RBUS) and voiding cystourethrogram (VCUG).

Anatomical definition not seen on VCUG can be evaluated

by intravenous pyelogram (IVP). Further evaluation

of upper pole function can be performed using nuclear

medicine imaging. An upper pole function of 10%

should be considered for U-U. Often times, the less

invasive RBUS can give an idea of the quality and

amount of upper pole parenchyma.

Intraoperative

Prior to making the incision in the lower abdomen,

cystoscopy with retrograde ureteropyelography can be

performed if preoperative imaging does not provide an

accurate map of the duplicated anatomy. It may be helpful

to identify ectopic ureteral orifices. If cystoscopy is not

warranted, ureteral stenting can be performed at the

time of the U-U anastomosis.

At the time of identifying the ureteral complex, care

must be taken when exposing the ureters to preserve

the blood supply. Minimal tissue handling is important

to prevent postoperative urine leak and stricture,

in addition to stenting/drain. Ureteral luminal disparity

from donor to recipient ureter has not been a problem,

Complications

VUR

24%

VUR stump

10%

Urinoma

9%

U-U revision

9%

Gastroenteritis

2%

UTI

7%

Fever

2%

Pneumonia

2%

Prolonged drain

output 31%

Ileus

2%

Transient

obstruction

2%

Figure 11.1 Complications of ipsilateral ureteroureterostomies.

78 Part III Open Surgery of the Upper Urinary Tract

but it is crucial to make an appropriate length incision

on the recipient ureter to match the dilated donor

ureter. The anastomosis should be performed with a

6-0 or 7-0 monofilament absorbable suture in a running

stitch. Using a lower abdominal incision allows for concurrent

procedures to be performed including ureterocele

excision, low resection of donor ureter stump in addition

to U-U with reimplant if VUR exists in the lower pole

ureter. If VUR exists on the contralateral side, reimplants

are best done intravesically to avoid potential bladder

neuropraxia from bilateral extravesical manipulation.

In patients with TUUs, the same principles of tissue

handling and urinary drainage apply. Other key points

are to create a tension-free anastomosis, as well as making

sure the donor ureter crosses the abdomen without

interference from the inferior mesenteric artery.

Postoperative

Patients postoperatively generally do well. It is important

to monitor drain output and remove it when it is dry to

prevent urinoma formation. Patients generally do well,

but if they present with UTI, pain, or fever, they warrant

urinalysis and culture and possible imaging with CT scan

or RBUS.

Normal routine follow-up should include RBUS at 6

weeks postoperatively. In addition, patients with other

renal and bladder comorbidities should have routine

creatinine and blood pressure monitoring.

Managing complications

Initial

Management of initial complications consists of evaluation

of symptoms. Early signs of problems can manifest

as fever, malaise, or urinary symptoms. All patients with

a documented, febrile UTI need evaluation for VUR

with a VCUG. Evaluation for urinoma or obstruction is

best evaluated by CT scan with contrast to assess size and

location of urine leak as well as hydronephrosis.

Definitive

Problems at the U-U can manifest in two forms: obstruction

or leak. Obstruction at the U-U site will manifest

as dilation in the donor renal segment. Management

consists of percutaneous nephrostomy drainage and

empiric antibiotics until infection is proven. After the

acute period resolves, antegrade nephrostogram should

be performed to assess patency of the anastomosis. If the

U-U is not open and draining, surgical intervention may

be necessary.

Urinary leak at the U-U site can occur and the risk

increases if stent and/or drains are not left in place postoperatively.

If urinoma does occur, percutaneous drainage

or open drainage is necessary. After resolution of the

urine leak, most studies show no problems with patency

with the U-U anastomosis. Of patients that did need

redo of the anastomosis, all of them resulted in patency

postoperatively.

VUR after surgery was a common complication. The

most definitive treatment historically performed was

ureteral reimplant. However, other management strategies

that have been successful, specifically observation or

subureteric injection, offer less morbidity. Yo-yo reflux in

all studies has not been an observed phenomenon and is

more of an academic concern.

Conclusion

Ipsilateral U-U is a safe and effective technique for managing

ureteral duplication anomalies. Postoperative

complications can be minimized based on patient selection

and recognizing pitfalls early and treating them

appropriately as they arise. For the right patient, it offers

good cosmesis, short recovery, and excellent success

rates.

TUU is also a safe and effective procedure for both

salvage procedures after failed ureteral reimplants and

urinary diversion/undiversion procedures in more complex

patients. Although postoperative problems are

uncommon, when they occur, they can be challenging

to manage. However, this should not deter the pediatric

urologist from using TUU as one of many methods for

managing these difficult cases.

References

1 Buchtel HA. Uretero-ureterostomy. J Urol 1965;93:153-7.

2 Lashley DB, McAleer IM, Kaplan GW. Ipsilateral ureterouretrostomy

for the treatment of vesicoureteral reflux or

obstruction associated with complete ureteral duplication.

J Urol 2001;165:552.

3 Chacko JK, Koyle MA, Mingin GC, Furness III PD:

Ipsilateral ureteroureterostomy in the surgical management

of the severely dilated ureter in ureteral duplication. J Urol

2007; 178 (4 pt 2): 1689-92.

4 Bieri M, Smith CK, Smith AY, Borden TA. Ipsilateral ureterouretrostomy

for single ureteral reflux or obstruction in a

duplicate system. J Urol 1998;159:1016.

Chapter 11 Ureteroureterostomy 79

5 Jelloul L, Valayer J. Ureteroureteral anastomosis in the treatment

of reflux associated with ureteral duplication. J Urol

1997;157:1863.

6 Choi H, Oh SJ. The management of children with complete

ureteric duplication: The use of ureterouretrostomy as a

primary and salvage procedure. BJU Int 2000;86:508.

7 Bochrath JM, Maizels M, Firlit CF. The use of ipsilateral

ureterouretrostomy to treat vesicoureteral reflux or obstruction

in children with duplex ureters. J Urol 1983;129:543.

8 Halpern GN, King LR, Belman AB. Transureteroureterostomy

in children. J Urol 1973;109:504.

9 Hodges CV, Barry JM, Fuchs EF, Pearse HD, Tank ES. Trans

ureteroureterostomy: 25-year experience with 100 patients.

J Urol 1980;123:834.

10 Hendren WH, Hensle TW. Transureteroureterostomy:

Experience with 75 cases. J Urol 1980;123:826.

11 Rushton HG, Parrott TS, Woodard JR. The expanded role

of transureteroureterostomy in pediatric urology. J Urol

1987;134:357.

12 Mure P, Mollard P, Mouriquand P. Transureteroureterostomy

in childhood and adolescence: Long-term results in 69 cases.

J Urol 2000;163:946.

13 Pesce C, Costa L, Campobasso P, Fabbro MA, Musi L.

Successful use of transureteroureterostomy in children: A

clinical study. Eur J Pediatr Surg 2001;11:395.

14 Kaplan WE, Nasrallah P, King LR. Reflux in complete duplication

in children. J Urol 1978;120:220.

15 Smith FL, Ritchie EL, Maizels M, Zaontz MR, Hseuh W,

Kaplan WE et al. Surgery for duplex kidneys with ectopic

ureters: Ipsilateral uretero-ureterostomy versus polar

nephrectomy. J Urol 1989;142:532.

16 Husmann DA. Renal dysplasia: The risks and consequences

of leaving dysplastic tissue in situ. Urology 1998;52:533.

IV Surgery of the

Bladder

Pediatric Urology: Surgical Complications and Management

Edited by Duncan T. Wilcox, Prasad P. Godbole and Martin A. Koyle

© 2008 Blackwell Publishing Ltd. ISBN: 978-1-405-16268-5

83

Epispadias-Exstrophy

Complex

Ahmad A. Elderwy and Richard Grady

Introduction

With Epispadias-Exstrophy Complex (EEC), the anterior

portion of the bladder and/or urethra and abdominal

wall structures are deficient and the pubis symphysis is

widely separated. Interestingly, classic bladder exstrophy

and epispadias often occur in isolation, while cloacal

exstrophy or exstrophy variants are usually associated

with anomalies of intestines, neurological system, upper

urinary tract, and skeletal system [1]. The complications

associated with this condition include those of the

untreated state as well as those that occur as a consequence

of surgical intervention. In this chapter, we will

discuss bladder exstrophy and epispadias.

Overview

Options to approach bladder exstrophy are discussed in

Table 12.1.

No treatment

Although bladder exstrophy is not a lethal anomaly in

infancy, these patients are often social pariahs because

of associated odor and hygiene problems. In addition,

66-75% of affected patients die by age 20 due to pyelonephritis

and renal failure [2]. The untreated exstrophic

patient has a 17.5% risk of bladder neoplasia after age

of 20 years, with high mortality rate. Early cystectomy is

not protective (Figure 12.1) [3].

Surgical intervention

Surgeon preference, patient anatomy, and availability of

tertiary care facilities all play a role in which operative

procedures are chosen.

Anatomical reconstruction of EEC

Bladder exstrophy repair

Early attempts (up to the 1970s) at bladder exstrophy closure

were dogged by high morbidity and poor long-term

Key points

• Epispadias-Exstrophy Complex (EEC) is a rare,

challenging birth defect.

• Preservation of kidney function and external

genitalia is a major concern.

• Complications associated with this condition are

common and can be severe.

• Familiarity and experience in the care of these

patients are essential for proper management.

12

Table 12.1 Options to approach bladder exstrophy.

1 No treatment

2 Anatomical reconstruction:

• Complete primary repair

• Staged repair

• Repair using radical mobilization of soft tissues

• Radical single-stage reconstruction

3 Urinary diversion (incontinent, anal sphincterbased

continence or continent reservoir) and genital

reconstruction with or without excision of bladder plate

Pediatric Urology: Surgical Complications and Management

Edited by Duncan T. Wilcox, Prasad P. Godbole and Martin A. Koyle

© 2008 Blackwell Publishing Ltd. ISBN: 978-1-405-16268-5

84 Part IV Surgery of the Bladder

outcomes for continence and renal function [4,5], and

many surgeons favored the use of primary urinary

diversion. However, the advent of the staged repair

approach demonstrated that anatomic reconstruction

of the exstrophic bladder was feasible and safe [6]. This

approach led to significant improvements in continence,

renal preservation as well as excellent cosmetic results

[7,8]. In literature, the rate of continence has varied.

Some patients require clean intermittent catheterization

(CIC) and bladder augmentation or diversion to achieve

dryness [9,10].

More recently, newer techniques of single-stage

exstrophy repair (including complete primary repair of

bladder exstrophy, CPRE) have been developed [11-14]

in addition to modifications of the staged repair (modern

staged repair of bladder exstrophy, MSRE) [15].

Neonatal exstrophy closure is recommended but salvage

single-stage reconstruction in delayed cases (6

months of age) is associated with lower continence rate

[16,17]. These new techniques of single-stage reconstruction

appear to be safer than that used in the past,

and enhance bladder capacity, stability, and compliance

more than staged approach [18].

Epispadias repair

Early efforts were associated with high rate of complications

[19]. Current methods (Cantwell-Ransley, and

Mitchell penile disassembly techniques) use dissection of

the corporal bodies and transposition of the tubularized

urethral plate to the ventral aspect of the penis with satisfactory

cosmesis and lower overall complications [19,20].

Urinary diversion

This approach may achieve some of the primary goals

of surgical intervention for EEC with fewer operations.

It is useful for patients who may not have reliable access

to health care facilities, and the patients who have not

achieved urinary dryness despite attempts at functional

reconstruction.

Ureterosigmoidostomy (USO) is associated with high

daytime urinary dryness rates of 92-97% without reliance

on catheters or external appliances. Despite the

reduction of complications for USO after improvements

in ureteral reimplantation, chronic metabolic

acidosis (up to 100%), chronic pyelonephritis (22%),

ureteral obstruction or reflux (up to 29%), significant

upper tract scarring and calculi (18%), and nighttime

incontinence (42%) necessitate secondary interventions

[21,22]. Furthermore, on longer-term follow-up, daily

fecal-urinary incontinence (26% and 48%, respectively)

with pelvic organ prolapse (48%), and delayed development

of neoplasms in the colon or the bladder remnant

(overall risk is 38%) have dampened enthusiasm for this

procedure [3,23]. Recently, low-pressure rectal reservoirs

have been proposed again as a treatment for bladder

exstrophy with acceptable day and night urinary dryness

(100% and 91%, respectively), and less complications.

Alkali supplementation and a regular follow-up surveillance

colonoscopy are still needed for these patients [24].

Incontinent urinary diversions were popular in the

past but had the significant disadvantage of an incontinent

abdominal stoma. Urinary conduits, especially ileal

loops, are not free of complications (renal deterioration

up to 41%). So, if necessary, colonic conduits with nonrefluxing

ureterocolic anastmosis are preferred [25,26].

The popularization of CIC has led to the development

over the past 15-20 years of continent urinary diversions

such as the Indiana pouch, which was developed for the

exstrophy population. Preservation of the native bladder

and bladder augmentation [27] allows the native

bladder to act as a convenient substrate for ureteral and

Mitrofanoff reimplantation.

Complications of anatomical

reconstruction of EEC

It is sometimes difficult to differentiate the failure to

reconstruct the primary pathophysiological defects of

Figure 12.1 Untreated bladder exstrophy (8 years old). Note

chronic changes of the bladder plate.

Chapter 12 Epispadias-Exstrophy Complex 85

EEC from complications associated with treatment. The

different approaches (CPER, MSRE, staged repair, etc.)

are designed to improve the long-term outcome, and

all rely on the same surgical principles, successful initial

closure, surgeon experience, and proper postoperative

management.

In most discussions of EEC, the subject of urinary

incontinence dominates the whole topic, to an extent

that the other disabilities resulting from the disorder

receive little attention [28]. Bladder exstrophy requires

a median of five operations per patient [29]. Successful

closure of isolated epispadias is achieved by single operation

in 80-91% [19,20] (Table 12.2).

Urinary complications

Bladder dehiscence has decreased from up to 13% to up

to 3% with recent techniques. It may be precipitated

by many factors including postoperative abdominal

distention, bladder prolapse, and the loss of ureteral

stents before postoperative day 7. Dehiscence necessitates

about a 6-month recovery period before a second

attempt at closure can be made. Tension-free reclosure

with osteotomy, preoperative testosterone, and combine

epispadias and bladder repair are important factors

in subsequent closures but up to a 40% failure rate

at reclosure is reported. Failure of the primary closure

markedly decreases the chance and onset for eventual

continence with volitional voiding from 30% if a patient

underwent two closures to less than 20% for who had

undergone more than two closure attempts. Primary use

of osteotomy may be protective against bladder dehiscence

[14,15,30,31].

Catheter malfunction is uncommon; catheter patency

should be confirmed at initial closure. Early loss of the

suprapubic catheter is a particular cause for concern. We

recommend replacing this with another tube or urethral

catheter as soon as possible if this happens within the

first 2 weeks following closure. Ureteric catheters malfunction

may necessitate early repositioning with open

surgery [12] or with fluoroscopy [32].

Urethrocutaneous fistula formation at the penopubic

junction is the most common surgical complication

of CPRE. In the setting of newborn exstrophy closure,

fistulas may occur in 14-35% of cases [14,29]. This fistula

rate is increased to 26-52% in delayed or redo cases

[16,17]. Two-layer closure covered with a single layer

small intestinal submucosa onlay help to prevent fistulae

[33]. With the staged exstrophy repair, postoperative fistula

develops in 21% of patients with intact plate versus

25% if para-exstrophy flaps are used [34].

Spontaneous fistula closure rate is expected in 25-100%

within 7.5 months. If not, surgical closure is indicated

Table 12.2 Complications following surgery for EEC.

Problem Early Late

Urinary Bladder dehiscence, urinary fistulae, BOO, UTI, urinary calculi, renal scarring, incontinence, stress

catheters malfunction, HUN incontinence, NE, CIC, bladder augmentation/diversion, renal

failure, bladder malignancy

Genital (male) Loss of glans or corpora, loss of Inadequate phallus, psychosexual delay, retrograde or difficult

penile skin ejaculation, subfertility, erectile dysfunction, sexual

reassignment*

Genital (female) Loss of urethra-vaginal septum, loss Genital cosmesis defects, vaginal stenosis, uterine prolapse,

of clitoris, vaginal deficiency miscarriage, elective cesarean section

Fascial Hematoma, wound infection, fascial Hernias, cosmesis defects in suprapubic area/umbilicus

dehiscence

Orthopedic Transient femoral nerve palsy Gait problems, abnormal hip dynamics, back pain

Others Latex allergy, abdominal distension, Rectal prolapse, fecal incontinence, short bowel syndrome,*

abdominal compartment fecal stoma/ACE,* multiple anesthesia, anxiety disorders,

syndrome,* death recurrence of exstrophy, death

HUN, hydroureteronephrosis; BOO, bladder outlet obstruction; UTI, urinary tract infection; NE, nocturnal enuresis; CIC, clean

intermittent catheterization.

*Specific complications with cloacal exstrophy.

86 Part IV Surgery of the Bladder

with urethrocystoscopy to evaluate the repair. Implications

of bladder neck (BN) fistula in reference to urinary continence

remain to be seen [17,33].

Urinary outlet obstruction is one of the potentially

dangerous failed outcomes of bladder closure because

it can cause renal deterioration, increase the risk for

chronic urinary tract infection (UTI), and may decrease

the chance to achieve urinary continence. This may be

somewhat subtle; however, routine ultrasonography of

the bladder and upper urinary tracts also should be performed

frequently after closure to detect hydronephrosis

(especially in combination with high postvoid residual)

that may indicate outlet obstruction and cystoscopy may

also be needed [35].

Neourethral stricture or tortousity is often associated

with para-exstrophy skin flap use, one stage urethroplasty

by flap/graft interposition, meatal stenosis, or pubic suture

erosion. A stricture develops at the proximal anastomotic

site in 10-67% of patients with exstrophy who have

para-exstrophy skin flaps used at the initial closure [36].

We prefer to convert exstrophy cases with the inherently

short urethral plate (50-77%) into hypospadias to avoid

complications of flap/graft interposition [14]. Combined

bladder and epispadias repair for delayed or redo cases is

associated with up to 10.5% stricture rate [16,17].

Interim management of bladder outlet obstruction

(BOO) usually includes urethral dilation, internal

urethrotomy, CIC, surgical revision, or diversion. Longterm

urinary diversion (e.g. suprapubic catheter) increases

the likehood of bladder augmentation [36]. The popularization

of the Mitrofanoff principle has significantly

improved the management of those patients with tortuous

neourethra following urethral reconstruction.

Bladder and kidney infections. Patients are routinely

maintained on suppressive antibiotic therapy as vesicoureteral

reflux (VUR) occurs almost universally after

exstrophy closure. This is continued until VUR is corrected

or resolves spontaneously (up to 16%) [14,29].

Febrile UTIs occur in up to 22% of patients while 70%

experience one or more episodes of asymptomatic bacteriuria.

These patients should be appropriately evaluated

(to ensure that they have no evidence of outlet obstruction)

and aggressively treated [29]. Early ureteral reimplantation

or deflux injection is indicated if recurrent

febrile infections occur in the setting of adequate prophylaxis.

Cephalotrigonal technique is preferred for ureteral

reimplantation as a cross-trigone technique may complicate

future bladder neck reconstruction (BNR) [37].

Renal damage is related to outlet obstruction and

febrile UTIs. With improvement of techniques and

follow-up, it is decreased from 25% to up to 7.5%

[10,13,14,38].

Transient hydroureteronephrosis (HUN) occurs in up

to 23% of children following their initial surgery, which

may be related to gradual accommodation of the bladder

as it cycles urine in the presence of VUR. About half

of children with HUN may require CIC even though

there is no evidence of BOO. Spontaneous improvement

is expected in most, but this is unpredictable

[14,38]. If HUN persists, ureteral reimplantation, bladder

augmentation, or diversion may be indicated to provide

a low-pressure urine storage reservoir. Long-term

study of EEC patients following staged reconstruction

has found that about 24% had significant upper tract

damage in the form of renal scarring and/or moderate

or severe hydronephrosis. Serum creatinine remained

normal in 97%, mild renal insufficiency developed in

1.5%, and renal transplantation was performed in 1.5%

[10]. The storage of urine in intestinal reservoirs did not

change renal function for at least 10 years in 80% of the

patients. The remaining (20%) had some deterioration

in renal function, usually from identifiable and remediable

causes [39].

Incontinence. Universally accepted definitions for continence

remain a topic for discussion. For the purpose of

this chapter, we define urinary continence as 2- to 3-h

dry intervals with volitional voiding without bladder

augment or diversion.

Successful CPRE may be associated with primary daytime

continence (bladder emptying volitionally of with

the use of clean CIC) at age of toilet training [14,17].

However, many exstrophy patients are partially continent

after initial closure and show on urodynamics a low

leak point, wide BN and reasonable bladder compliance

and capacity (60-85 ml). For these patients, formal

BNR or endoscopic injection of the BN is indicated and

continence is achieved in up to 60-87%. Most surgeons

use for BNR either Young-Dees-Leadbetter or Mitchell

BNR (which also moves fibrotic tissue at the level of the

original BN away from the new BN) [14,15]. At the time

of BNR, we typically perform ureteral reimplantation (if

not done before) and construct a Mitrofanoff channel

[14]. The mean time to daytime continence after BNR

is 14 months (range 4-21) and the mean time to nighttime

continence is 22 months (range 11-33) [40]. These

children void volitionally and may catheterize one to two

times a day to ensure bladder emptying.

In some patients (5-52%), the bladder does not

grow to an adequate capacity (50-60 ml) and/or has

impaired compliance with upper tract deterioration. This

Chapter 12 Epispadias-Exstrophy Complex 87

may be due to postoperative complications (e.g. bladder

prolapse or dehiscence, BOO, recurrent UTIs, bladder

calculi, and long-term diversion), or can occur despite

technically successful reconstruction efforts (especially

if delayed or redo closure, delayed epispadias repair,

very small bladder plate at initial closure, or neuropathic

bladder). In this situation, concomitant BNR, bladder

augmentation with or without Mitrofanoff channel is

recommended [8,27,30].

Many adjuvant measures may be helpful to achieve

continence, control stress incontinence, and enuresis.

Again, urodynamics help to decide the needed

maneuver.

• Endourethral injection (1-3 sessions) of dextranomer

based implants (Deflux®) remained beneficial in 40% of

patients with 7-years follow-up [41]. A history of previous

surgery and gender had no significant effect on

the outcome. A maximum of three injections is predictive

with reasonable certainty of any benefit from the

procedure [42].

• Anticholinergic agents, low-dose desmopressin [43], or

imipramine [44] may also be helpful.

Despite near or total subjective continence and "good"

voiding in children who had undergone staged reconstruction

for exstrophy, of these patients 72% have

clinical problems related to emptying, which include

recurrent UTIs, epididymitis, and bladder calculi.

Objective urodynamic parameters confirm poor voiding

in most patients. One must question the normalcy of the

voiding pattern and price to achieve continence among

patients with exstrophy [45].

If urinary continence is not achieved within 2 years

following formal BNR and the above measures, future

success is elusive [40]. Treatment choice depends on

the results of urodynamic studies and other clinical and

social factors.

• Rarely, the urodynamic evaluation reveals a bladder

with adequate capacity and compliance with wide

BN and low detrusor leak point pressure. In these cases

BNR, artificial urinary sphincter, or other BN procedures

(wrap/sling/closure) can be performed. BNR revision

rarely achieves continence with volitional voiding [46].

The creation of a Mitrofanoff channel in this situation

is invaluable.

• Most of the patients demonstrate an inadequate bladder

capacity and a low detrusor leak point pressure

and usually require bladder augmentation or diversion.

Often, bladder augmentation is combined with a

BN procedure and Mitrofanoff channel to optimize the

chance for urinary dryness.

In long-term follow-up, about 60% of patients with

initially successful bladder closures and BNR have

required further surgery (augment/diversion) in their

second decade of life because of the gradual development

of poorly compliant, low-capacity bladders that

cause urinary incontinence [47]. This may be due to

fixed outlet resistance with multiple BN procedures. It

remains to be seen if CPRE will experience late continence

failures as these children age.

Bladder malignancy. Early reconstruction decreases

the risk of malignancy in the exstrophic bladder from

17.5% to 3.3% at a median age of 42 years [3]. Moreover,

patients who have undergone augmentation cystoplasty

using intestinal segments are at increased risk for malignant

degeneration [48]. Life-long surveillance with cystoscopy

and urine cytology is recommended. In the event of

malignancy, treatment options include radical cystectomy

with urinary diversion [49]. Four cases have been reported

with severe perineal pain after bladder augmentation that

was probably secondary to the abnormal retained bladder

remnants. Cystectomy cured the pain and may also have

removed a potential site of future malignant tumor [50].

The male genital complications

Atrophy of the corporal bodies, glans, and/or urethra. In

experienced hands, these complications are unusual.

It has been described after the initial stage of a staged

reconstruction [51,52] as well as CPRE (up to 5%)

[53,54]. These catastrophic complications can arise from

violation of tissue planes. Overly aggressive attempts at

mobilizing the corporal bodies from the pubis symphysis

and penile lengthening may result in corporal denervation

and/or devascularization [13] without additional

lengthening of the congenitally deficient exstrophic

penis [55]. Efforts to avoid this complication include

assessment of the glans penis for ischemia after pubic

rami reapproximation; if there is any color change, apply

papaverine, and replace the sutures higher in the pubis,

and/or consider osteotomy if not done.

With loss of the urethral plate and significant amounts

of penile skin, other sources of replacement tissues

(grafts/flaps) can be used for later reconstruction [56].

Ischemia of the glans and penile skin within 24-48 h

after closure is reversible in more than half of the cases

by observation. The use of vasodilators may be helpful.

Reoperation with higher replacement of pubic sutures

appears to be of no value in the setting of prolonged

ischemia [54]. Acute postoperative penile ischemia should

prompt immediate reoperation to release tight sutures,

restore circulation, and salvage the penis [13].

88 Part IV Surgery of the Bladder

A short phallus and/or persistent chordee. Complete

penile disassembly provides satisfactory cosmetic and

functional penile outcome [14]. In the Cantwell-Ransley

repair, up to 7% of patients require early penile straightening

surgery [34]. Revision rate for genitoplasty is 29%

after puberty [57]. In another study, erections were

curved in 34% with no curvature so severe as to prevent

sexual intercourse [58].

Penile degloving and division of suspensory ligament

can maximize the available penile length. A dorsal dermal

corporal graft, ventral corporal plication, or rotation

may additionally help lengthen as well as correct

any chordee/asymmetry. Scar excision can be closed in a

plastic fashion (Z-plasty) if enough penile skin is available.

Otherwise, rotational flaps, tissue expanders, or fullthickness

skin grafts can be used [52,59].

Sexual dysfunction. Adolescent males with exstrophy

are psychosexually delayed 2-4 years compared with

their peers [60]. Libido, erection, and orgasm are usually

intact. About 50-70% of men described intimate relationships

as serious and long-term [58,61,62].

Subfertility. Ejaculation is often present in up to

63-90% of men despite the extensive reconstructive

procedures done for these patients. Sperm quality and

quantity is impaired (at least in 40%) despite testes that

are believed to be intrinsically normal. This may be due

to partial obstruction, retrograde ejaculation, slow seminal

emission, or recurrent infections. Despite this, about

half of men do not require assisted reproductive techniques

to father children [58,61,62].

The female genital complications

Loss of urethrovaginal septum occurs in rare situations.

This usually occurs during dissection of urethral plate

from the underlying vagina or transaction and lengthening

of urethral plate with para-exstrophy flaps [56,63].

Mobilization of the BN, urethra, and vagina as a unit

helps prevention of this complication [14].

Further reconstruction of the female genitalia, if needed,

can be done during adolescent years. Mons-plasty with

hair-bearing skin and fat should be used to cover the

midline defect. Most patients required vaginal dilatation

or a cut-back/Y-V vaginoplasty to allow satisfactory

intercourse in the mature female. Initiation of sexual

activity tends to be delayed until early adulthood. All of

the female patients described intimate relationships as

serious and long term [63,64].

Uterine and vaginal prolapse occur in up to 50% of

patients. Early primary bladder reconstruction may

decrease this risk. Fixation of the uterus to the anterior

abdominal wall in childhood may be helpful to prevent

prolapse while allowing normal pregnancy [65]. Uterine

suspension procedures such as sacrocolpopexy can correct

uterine prolapse in the exstrophy patient and may

preserve fertility in young patients [66].

Obstetric implications. Fecundity is unimpaired in

female patients with exstrophy, but maintenance of a

pregnancy is significantly more difficult and requires

interdisciplinary cooperation. Successful pregnancy is

reported in 10-25% of patients [67]. Complications in

the past have included maternal deaths [2]. Nowadays,

these pregnancies are more often complicated by:

• Obstetric complications that include uterine or vaginal

prolapse (50%), preterm labor (40%), miscarriages

(28%), and malpresentation (25%). Bed rest is necessary

in the later stages of pregnancy for most of these patients

[68-70].

• Urinary complications that include transient secondary

urinary incontinence, recurrent UTIs (17-52%),

hydronephrosis requiring drainage (10%), Mitrofanoff

difficulties, and ileal prolapse (in those with ileal conduit).

Antibiotic prophylaxis is recommended and

patients may require indwelling catheters. The usual

voiding pattern can be resumed after delivery. Pregnancy

has no long-term effect on renal function and does not

compromise reconstruction [68-70].

Spontaneous vaginal deliveries (if not precluded

by malpresentation) are done in those women who

had undergone prior permanent urinary diversions.

Vaginal delivery may carry the risk of obstructed labor

(due to vaginal stenosis) and later prolapse. Most

recommend elective cesarean sections before term for

women with functional bladder closures to eliminate

stress on the pelvic floor and the urinary sphincter

mechanism [2,71].

Fascial abnormalities

• Fascial dehiscence may occur within 1 week after repair

in up to 3% of patients and require immediate repair.

This complication does not affect the integrity of the

bladder or urethral reconstruction [14] (Figure 12.2).

• Inguinal hernias have been reported in 56-82% of boys

and 11-15% of girls with bladder exstrophy, and 33% in

boys with complete epispadias. Incarcerated hernias affect

up to 50% of boys during the first year following their

initial procedure. The incidence of synchronous or asynchronous

bilaterality is about 80%. At the time of bladder

exstrophy closure, these hernias should be bilaterally

repaired using a preperitoneal approach to the internal

ring. The overall recurrence rate is 8-17% [72-74].

Chapter 12 Epispadias-Exstrophy Complex 89

Orthopedic complications

These are associated with the orthopedic management

of exstrophy in about 4% of patients [75]. These include

transient femoral nerve palsy, delayed union of osteotomy,

and osteomyelitis.

• Complications of traction. Pressure sores and compartment

syndrome with permanent muscle weakness can

occur with tight wrapping of legs [75]. Significant transient

hypertension was reported with inappropriately applied

traction [76]. We use a spica cast for 3 weeks for postoperative

immobilization of neonates, which facilitates early

discharge and nursing care with excellent results [14].

• Gait abnormalities in these children arise as a consequence

of underlying bone abnormalities. Many of these

children initially learn to ambulate with a wide waddling

gait that resolves as the children grow. Few develop

gait problems, hip dysplasia, or back pain [77]. A recent

study showed that early pelvic osteotomy has long-term

effects on patients' instinctive walking patterns and neutralizes

some of the effects of bladder exstrophy [78].

Other complications

• Latex allergy. One-third of patients with bladder

exstrophy developed latex symptoms and another third

have latex sensitization. Multiple surgical procedures

and atopy play a major role in the development of latex

hypersensitivity [79].

• Psychosocial concerns and long-term adjustment.

Children with exstrophy undergo multiple surgeries

and have potential problems with respect to urinary

continence, sexual function, and self-esteem problems.

Children who achieved continence after the age of 5

years are more likely to have problems with acting-out

behavior. They do not have clinical psychopathology

and improving outcomes may be achieved through a

focus on normal adaptation [80]. Current recommendations

include early psychiatric intervention and advised

to continue with long-term psychiatric support into

adult life [61]. There are a number of websites that can

prove very helpful to parents and patients (http://www.

bladderexstrophy.com/support.htm).

• Mortality. Currently, exstrophy repair carries a low

risk of mortality (up to 1.5% in the United States, all of

whom had been born prematurely) [81]. Late mortality

may be related to development of malignancy [3].

Conclusion

Although results of therapy are far from perfect, they

reflect remarkable accomplishments from many physicians.

There is great potential for further improvement

such that 1 day children born with exstrophy will be

treated early and completely and never know that they

had a major problem.

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in the bladder exstrophy population: Predictors of success?

Urology 2001;57:774-7.

41 Lottmann HB, Margaryan M, Lortat-Jacob S et al. Longterm

effects of dextranomer endoscopic injections for the

treatment of urinary incontinence: An update of a prospective

study of 61 patients. J Urol 2006;176:1762-6.

42 Burki T, Hamid R, Ransley PG et al. Injectable

polydimethylsiloxane for treating incontinence in children

with the exstrophy-epispadias complex: Long-term results.

BJU Int 2006;98:849-53.

43 Caione P, Nappo S, De Castro R et al. Low-dose desmopressin

in the treatment of nocturnal urinary incontinence in

the exstrophy-epispadias complex. BJU Int 1999;84:329-34.

44 Dave S, Grover VP, Agarwala S, Mitra DK, Bhatnagar V. The

role of imipramine therapy in bladder exstrophy after bladder

neck reconstruction. BJU Int 2002;89:557-60.

45 Yerkes EB, Adams MC, Rink RC, Pope JC IV, Brock JW, 3rd.

How well do patients with exstrophy actually void? J Urol

2000;164:1044-7.

46 Burki T, Hamid R, Duffy P et al. Long-term followup of

patients after redo bladder neck reconstruction for bladder

exstrophy complex. J Urol 2006;176:1138-41.

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47 Woodhouse CR, Redgrave NG. Late failure of the reconstructed

exstrophy bladder. Br J Urol 1996;77:590-2.

48 Fernandez-Arjona M, Herrero L, Romero JC et al.

Synchronous signet ring cell carcinoma and squamous cell

carcinoma arising in an augmented ileocystoplasty. Case

report and review of the literature. Eur Urol 1996;29:125-8.

49 Paulhac P, Maisonnette F, Bourg S et al. Adenocarcinoma in

the exstrophic bladder. Urology 1999;54:744.

50 Phelps SR, Malone PS. Severe perineal pain after enterocystoplasty

in bladder exstrophy. BJU Int 2004;93:835-7.

51 Woodhouse CR, Kellett MJ. Anatomy of the penis

and its deformities in exstrophy and epispadias. J Urol

1984;132:1122-4.

52 Amukele SA, Lee GW, Stock JA et al. 20-year experience

with iatrogenic penile injury. J Urol 2003;170:1691-4.

53 Hammouda HM. Results of complete penile disassembly for

epispadias repair in 42 patients. J Urol 2003;170:1963-5.

54 Husmann DA, Gearhart JP. Loss of the penile glans and/or

corpora following primary repair of bladder exstrophy

using the complete penile disassembly technique. J Urol

2004;172:1696-700.

55 Silver RI, Yang A, Ben-Chaim J et al. Penile length in adulthood

after exstrophy reconstruction. J Urol 1997;157:999-1003.

56 Gearhart JP, Baird AD. The failed complete repair of bladder

exstrophy: Insights and outcomes. J Urol 2005;174:1669-72.

57 VanderBrink BA, Stock JA, Hanna MK. Aesthetic aspects of

abdominal wall and external genital reconstructive surgery

in bladder exstrophy-epispadias complex. Curr Urol Rep

2006;7:149-58.

58 Avolio L, Koo HP, Bescript AC et al. The long-term outcome

in men with exstrophy/epispadias: Sexual function and

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59 Woodhouse CR. The management of erectile deformity

in adults with exstrophy and epispadias. J Urol

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60 Reiner WG, Gearhart JP, Jeffs R. Psychosexual dysfunction

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61 Ben-Chaim J, Jeffs RD, Reiner WG et al. The outcome

of patients with classic exstrophy in adult life. J Urol

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62 Woodhouse CR. Sexual function in boys born with exstrophy,

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63 Ben-Chaim J, Jeffs RD, Gearhart JP. Loss of urethrovaginal

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64 Woodhouse CR. The gynaecology of exstrophy. BJU Int

1999;83:34-8.

65 Stein R, Fisch M, Bauer H et al. Operative reconstruction

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66 Rose CH, Rowe TF, Cox SM et al. Uterine prolapse associated

with bladder exstrophy: Surgical management and subsequent

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67 Mathews RI, Gan M, Gearhart JP. Urogynaecological and

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69 Hensle TW, Bingham JB, Reiley EA et al. The urological care

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72 Husmann DA, McLorie GA, Churchill BM et al. Inguinal

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74 Connolly JA, Peppas DS, Jeffs RD et al. Prevalence and

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81 Nelson CP, Dunn RL, Wei JT, Gearhart JP. Surgical repair of

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2005;174:1099-1102.

92

Umbilical and Urachal

Anomalies

Paul F. Austin

The urachus, or median umbilical ligament, is a cordlike

structure that is continuous with the anterior dome

of the bladder inferiorly and extends in an extraperitoneal

fashion to the umbilicus superiorly. The urachus is

a normal embryonic remnant of the primitive bladder

dome and may be affected by disorders related to the

arrest of its normal involution. There are four clinical

entities relating to the incomplete involution of the

urachus during embryogenesis. These urachal anomalies

include a patent urachus, urachal cyst, urachal sinus, and

vesicourachal diverticulum (Figure 13.1).

Prevalence

Urachal anomalies are rare. In a large pediatric autopsy

series, the historical incidence of a patent urachus is 1

in 7610 cases and the incidence of a urachal cyst is 1 in

5000 [1]. Another example of the infrequency of urachal

anomalies includes a report of 315 cases accumulated

Key points

• The urachus is an embryonic remnant of the

allantois.

• The urachal anomaly subtypes are reflective of

the location of the incomplete involution of the

urachus.

• The medial umbilical ligaments are vestigial

urachal structures and useful landmarks for

identifying the ureters.

• Ultrasound and/or sinography are useful diagnostic

modalities for the majority of urachal anomalies.

13

(a) (b)

(c) (d)

Figure 13.1 Urachal anomalies: (a) patent urachus, (b) urachal

cyst, (c) urachal sinus, and (d) vesicourachal diverticulum.

Pediatric Urology: Surgical Complications and Management

Edited by Duncan T. Wilcox, Prasad P. Godbole and Martin A. Koyle

© 2008 Blackwell Publishing Ltd. ISBN: 978-1-405-16268-5

Chapter 13 Umbilical and Urachal Anomalies 93

over a 40-year period [2]. The most frequent urachal

abnormalities that typically present are either an urachal

sinus or an urachal cyst [3-5]. There is generally a 2:1

incidence of urachal anomalies in males compared to

females and urachal anomalies, usually present in early

childhood, may be clinically silent and remain unrecognized

until adulthood.

Embryology

The urachus is an embryonic remnant of the allantois

[6]. The allantois in embryos of reptiles, birds, and

some mammals has a respiratory function and may

act as a reservoir for urine during embryonic life. The

allantois remains very small in human embryos but is

involved with early blood formation and associated with

development of the urinary bladder [6]. As the bladder

enlarges, the allantois becomes the urachus and is represented

in adults as the median umbilical ligament.

The blood vessels of the allantois become the umbilical

arteries and veins. The obliterated umbilical arteries or

medial umbilical ligaments are important landmarks

to help locate the underlying ureters when performing

surgery on the bladder and ureter (e.g. extravesical

ureteroneocystomy).

The allantois arises from the yolk sac and extends

to the cloaca - the precursor to the bladder (urogenital

sinus) and the rectum (hindgut). With further development

and division of the urogenital sinus and hindgut

by the urorectal septum, the allantois is initially continuous

with the bladder but soon constricts and

becomes a thick, fibrous cord called the urachus. During

the 4th and 5th months of development, the urachus

narrows to a small caliber tube lined by transitional epithelium

[7].

Anatomy

The urachus lies within the space between the peritoneum

posteriorly and the transversalis fascia anteriorly

that is known as the space of Retzius [8]. The urachus

is bounded by the umbilicovesical fascia, which extends

laterally to each umbilical artery. Inferiorly, the fascial

layers spread out over the dome of the bladder to the

hypogastric artery posteriorly and to the pelvic diaphragm

anteriorly. Thus, a potential pyramidal shaped

space is created that is completely self-contained and

separate from the peritoneal cavity. These fascial planes

act to limit the spread of an urachal infection or neoplasm.

Knowledge of this anatomy becomes important

in the diagnosis and treatment of urachal diseases [9].

Clinical urachal anomalies

Urachal anomalies result from a failure of fibrosis and

involution of the urachus during embryonic development.

A variety of clinical urachal anomalies exist and

are dependent on where the failure of involution occurs

in the urachal tract between the bladder and the umbilicus

(Figure 13.1).

Diagnosis

The diagnosis of an urachal anomaly is made from

a combination of presenting history, physical exam,

and imaging. Periumbilical discharge suggests either

a patent urachus or an urachal sinus while a palpable

umbilical mass suggests an umbilical cyst. Patients may

present with abdominal, suprapubic, or periumbilical

pain. Periumbilical erythema and tenderness suggest an

underlying infection and patients with urachal anomalies

may present with dysuria, fever, and a urinary tract

infection.

A variety of imaging may be used to make the diagnosis

of an urachal anomaly. The appearance of a fixed,

midline, cystic, extraperitoneal swelling between the

umbilicus and the bladder on ultrasonography (US) is

suggestive of an urachal anomaly [10]. During a workup

of abdominal or pelvic pain, computed tomography

(CT) may allow the diagnosis of an urachal anomaly [11]

Figure 13.3b. Several studies have advocated that US is

the test of choice if an urachal cyst is suspected with a

periumbilical mass and sinography is the best modality

to identify a patent urachus or urachal sinus [3,4,12].

Other imaging modalities may diagnose urachal anomalies

including a voiding cystourethrogram (VCUG) may

demonstrate an urachal diverticulum commonly seen in

patients with prune belly syndrome.

Analysis of the umbilical fluids may provide another

means of diagnosing an urachal anomaly. Fluid analysis

would include measuring the umbilical fluid for content

of urea and creatinine. Injecting methylene blue

transurethrally or indigo carmine intravenously and

observing a color change in the draining fluid; or conversely,

injecting indigo carmine into the fistulous tract

and looking for a color change in the urine may also

94 Part IV Surgery of the Bladder

provide the diagnosis [9]. Finally, cystoscopy has also

been reported to assist in the characterization of urachal

anomalies [7].

Outcomes and complications of urachal

anomalies

Congenital anomalies of the urachus represent an arrest

of the normal process of involution of the urachus and

may not present until adulthood. Common presenting

symptoms are periumbilical discharge, umbilical mass,

periumbilical pain, and dysuria [13]. Bladder prolapse or

eversion has been reported in a patent urachus [14,15]

mimicking an omphalocoele on antenatal scans. Sepsis

secondary to a patent urachus has also been reported

[16,17]. Urachal cysts may become infected. Usually the

infection is restricted to the space of Retzius but occasionally

the cyst may rupture intraperitoneally with

resultant bowel fistula formation [18]. A urachal sinus

usually presents with symptoms and signs of localized

sepsis. Occasionally, intra-abdominal contents may be

densely adherent to the inflammatory mass and may

be injured during resection. A vesicourachal diverticulum

rarely requires treatment unless it is large with

poor emptying due to a narrow neck or paradoxical

contraction.

In a report by Ueno and associates, the authors advocate

that patients with asymptomatic urachal remnants do

not require follow-up, and urachal remnants, especially

those under 1 year of age, do not require surgical resection

unless the patient has multiple symptomatic episodes

[19]. Their conclusions were based upon the finding of

only 1 patient out of 44 patients that developed recurrent

symptoms during follow-up (maximum follow-up was

32 months). The authors also cite the spontaneous

involution rate of the normal urachus during infancy

[20] and found that nearly one-third of their asymptomatic

patients had disappearance of their urachal

remnant.

One of the concerns of leaving an urachal remnant

is if untreated, urachal carcinoma may develop within

these anomalies. Urachal carcinoma is rare in children

(0.01%) [21] and accounts for 0.34% of all bladder

cancers [22,23]. The most common type is adenocarcinoma

although other histological types have been

reported [21,23-26]. The patients usually have a poor

prognosis due to late presentation with local invasion.

In a histologic review of 23 urachal remnants removed

over a 10-year period, Upadhyay and Kukkady found

normal urothelial lining in 17 urachal remnants, whereas

6 (25%) showed abnormal epithelium. This abnormal

epithelium included colonic epithelium, small intestinal

epithelium, and squamous epithelium which suggest

concern for malignant degeneration. Given this potential

risk of malignancy along with the minimal invasiveness

of laparoscopy, a case may be made for laparoscopy

as a treatment modality for asymptomatic urachal

remnants [27].

Diagnosis and management of urachal

anomalies

Patent urachus

A patent urachus usually presents itself at or soon after

birth when the umbilical cord is ligated and urine drains

from the umbilicus. Historically, lower urinary tract

obstruction has been considered a contributing factor in

its pathogenesis [28], but this is not seen in the majority

of cases. In fact, urethral tubularization occurs after the

urachal lumen obliterates during fetal development [29].

Subsequently, it suggests that infravesical obstruction has

little influence on urachal development.

As previously mentioned, the diagnosis is frequently

confirmed after injecting the patent urachal opening

with contrast during a sinogram or by analyzing

the umbilical fluid. Other conditions that may present

with a wet umbilicus include anomalies of the omphalomesenteric

duct (completely patent omphalomesenteric

duct, omphalomesenteric duct sinus, vitelline cyst,

Meckel's diverticulum) or an umbilical granuloma [30].

In the management of a patent urachus, observation

may be indicated in young infants without symptoms

because the involution of the urachus is not complete

at birth and spontaneous closure can occur in the first

few months of life [31]. If there is an associated bladder

outlet obstruction, management of the bladder outlet

obstruction is often adequate to cause involution of the

patent urachus.

When drainage is persistent, complete excision of the

urachus with a small cuff of bladder by an extraperitoneal

approach is recommended [32] (Figure 13.2).

Urachal cyst

The majority of urachal cysts develop in the lower

third of the urachus. Most urachal cysts go undetected

unless they become infected or enlarge to a size causing

mechanical symptoms [33]. Following enlargement

Chapter 13 Umbilical and Urachal Anomalies 95

(c)

(a) (b)

Figure 13.2 Patent urachus: (a) patent opening inferior to umbilical cord, (b) patent urachus visualized on cystogram and sinogram,

and (c) operative dissection of patent urachus.

of the cyst, symptoms include lower abdominal pain,

a feeling of heaviness, and urinary frequency. Urachal

cysts may become infected and develop into an urachal

abscess. The majority of these are infected with

Staphylococcus aureus [3,4] and these generally manifest

in adulthood. US is the most common diagnostic modality

to identify urachal cysts [4] (Figure 13.3a). CT scan is

beneficial when there is a large cystic abscess or there is

severe periumbilical cellulitis which may cause misinterpretation

on ultrasound [12] (Figure 13.3b).

Treatment of urachal cysts involves complete excision.

However, when infection is present, management by

perioperative drainage and antibiotics followed by subsequent

elective excision may represent the most effective

surgical option [34-36]. Excision of the urachal cyst

may be done openly or may be performed laparoscopically

[37,38].

Urachal sinus

An urachal sinus most likely represents an urachal cyst

that becomes infected and dissects to the umbilicus

(Figure 13.4). Additionally, an urachal sinus may drain

into the bladder or it may drain into either the umbilicus

or the bladder and is termed an alternating sinus. These

patients typically present in childhood with periumbilical

pain and tenderness and may have umbilical erythema,

excoriation, or reactive granulation tissue. A fistulogram is

usually diagnostic and will help delineate the extent of the

sinus tract [4,12]. After treatment of the acute infection,

surgical excision of the sinus tract is recommended.

96 Part IV Surgery of the Bladder

Vesicourachal diverticulum

A vesicourachal diverticulum is frequently seen in a child

with prune belly syndrome. A vesicourachal diverticulum

may be seen in the setting of lower urinary tract obstruction

(e.g. posterior urethral valves) but may also be discovered

incidentally during an imaging work-up (e.g.

VCUG for evaluation of vesicoureteral reflux). Patients

who have this urachal anomaly are usually asymptomatic.

A vesicourachal diverticulum is thought to occur when

there is incomplete obliteration and closure of the lower

portion of the urachus and the bladder apex. No treatment

is usually necessary since this anomaly is primarily

morphological and bears no functional consequences.

References

1 Rubin A. Handbook of Congenital Malformations.

Philadelphia: Saunders, 1967.

2 Blichert-Toft M, Nielsen OV. Congenital patient urachus

and acquired variants: Diagnosis and treatment. Review

of the literature and report of five cases. Acta Chir Scand

1971;137:807-14.

3 Mesrobian HG, Zacharias A, Balcom AH, Cohen RD. Ten

years of experience with isolated urachal anomalies in children.

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4 Cilento BG, Jr., Bauer SB, Retik AB, Peters CA, Atala A.

Urachal anomalies: Defining the best diagnostic modality.

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5 Choi YJ, Kim JM, Ahn SY, Oh JT, Han SW, Lee JS. Urachal

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6 Moore KL, Persaud TVN. The Developing Human: Clinically

Oriented Embryology, 5th edn. Philadelphia: Saunders, 1993.

7 Nix JT, Menville JG, Albert M, Wendt DL. Congenital patent

urachus. J Urol 1958;79:264-73.

(a) (b)

Figure 13.3 Urachal cyst: (a) ultrasound image of urachal cyst, note proximity to bladder (BL) and (b) CT scan evaluation of

abdominal pain revealing infected urachal cyst.

Bladder

Urachal

sinus

Figure 13.4 Urachal sinus: (a) urachal sinus presenting as a

protuberant umbilical mass with drainage and (b) operative

dissection of alternating urachal sinus. Note connection to

bladder and umbilicus.

(a)

Chapter 13 Umbilical and Urachal Anomalies 97

8 Hammond G, Yglesias L, Davis JE. The urachus, its anatomy

and associate fasciae. Anat Rec 1941;80:271-87.

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10 Holten I, Lomas F, Mouratidis B, Malecky G, Simpson E.

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remnant diseases: Spectrum of CT and US findings.

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13 Cilento BG, Jr., Bauer SB, Retik AB et al. Urachal anomalies:

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14 Lugo B, McNulty J, Emil S. Bladder prolapse through a

patent urachus: Fetal and neonatal features. J Pediatr Surg

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16 Takamura C, Ikegami M, Han YS et al. Patent urachus associated

with abdominal abscess: Report of a case. Hinyokika

Kiyo 1991;37:87-90.

17 Buckspan MB. Patent urachus and infected urachal cyst in

an adult: A case report. Can J Surg 1984;27:496.

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1952;84:252.

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Kanamaru H. Urachal anomalies: Ultrasonography and

management. J Pediatr Surg 2003;38:1203-7.

20 Zieger B, Sokol B, Rohrschneider WK, Darge K, Troger J.

Sonomorphology and involution of the normal urachus in

asymptomatic newborns. Pediatr Radiol 1998;28:156-61.

21 Clapuyt P, Saint-Martin C, De Batselier P et al. Urachal neuroblastoma:

First case report. Pediatr Radiol 1999;29:320.

22 Henly DR, Farrow GM, Zincke H. Urachal cancer: Role of

conservative surgery. Urology 1993;42:635.

23 Sheldon CA, Clayman RV, Gonzalez R et al. Malignant

urachal lesions. J Urol 1984;131:1.

24 Yokoyama S, Hayashida Y, Nagahama J et al. Rhabdomyosarcoma

of the urachus: A case report. Acta Cytol 1997;41:1293.

25 Defabiani N, Iselin CE, Khan HG et al. Benign teratoma of

the urachus. Br J Urol 1998;81:760.

26 D'Alessio A, Verdelli G, Bernardi M et al. Endodermal

sinus (yolk sac) tumor of the urachus. Eur J Pediatr Surg

1994;4:180.

27 Navarrete S, Sanchez Ismayel A, Sanchez Salas R, Sanchez R,

Navarrete Llopis S. Treatment of urachal anomalies: A minimally

invasive surgery technique. JSLS 2005;9:422-5.

28 Hinman F, Jr. Surgical disorders of the bladder and umbilicus

of urachal origin. Surg Gynecol Obstet 1961;113:605-14.

29 Schreck WR, Campbell WA, 3rd. The relation of bladder

outlet obstruction to urinary-umbilical fistula. J Urol

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3rd edn. Edited by B O'Donnell, SA Koff. Oxford/Boston:

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31 Zieger B, Sokol B, Rohrschneider WK et al. Sonomorphology

and involution of the normal urachus in asymptomatic

newborns. Pediatr Radiol 1998;28:156.

32 Nix JT, Menville JG, Albert M. Congenital patent urachus.

J Urol 1958;79:264.

33 MacNeily AE, Koleilat N, Kiruluta HG, Homsy YL. Urachal

abscesses: Protean manifestations, their recognition, and

management. Urology 1992;40:530-5.

34 McCollum MO, Macneily AE, Blair GK. Surgical implications

of urachal remnants: Presentation and management.

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36 Minevich E, Wacksman J, Lewis AG, Bukowski TP, Sheldon

CA. The infected urachal cyst: Primary excision versus a

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V Endoscopic Surgery of

the Urinary Tract

Pediatric Urology: Surgical Complications and Management

Edited by Duncan T. Wilcox, Prasad P. Godbole and Martin A. Koyle

© 2008 Blackwell Publishing Ltd. ISBN: 978-1-405-16268-5

101

Cystoscopy and Cystoscopic

Interventions

Divyesh Y. Desai

Introduction

Pediatric endoscopy has come a long way in the past two

decades, and advances and refinements in fiber optic technology,

along with miniaturization of equipment, allow

endoscopic visualization of almost the entire urinary tract.

Miniaturization has allowed the endoscopic appraisal of

most neonatal urethras including those of preterm babies

weighing 2000 g or more. There are a wide array of instruments

available today which allow for safe assessment and

intervention with minimal complications provided one

remains within the limitations of the available equipment.

Posterior urethral valves

Introduction

Posterior urethral valves remain the most common

cause of lower urinary tract outflow obstruction in

male infants with an estimated incidence of 1:5000

live births. The majority are suspected on antenatal

ultrasound screening and referred to specialist centers at

birth. Modern endoscopic equipment along with longterm

outcome data has dramatically changed the surgical

approach to valve treatment. In the past, many newborns

were treated with a vesicostomy primarily because of

the relatively large instrumentation available. Supravesical

diversions were in vogue due to their undisputed

short-term benefits on renal function; however, the longterm

outcome of these diversions show no benefit for

renal function and raise concern regarding the effect on

outcome on bladder function. This chapter will cover the

various approaches to the endoscopic ablation of posterior

urethral valves, their complications, and an approach

to avoiding these complications in the 21st century.

Surgical techniques and outcomes

In 1972, Whitaker et al. [1] reported the results of 112

patients in whom valves were ablated endoscopically

with an infant McCarthy panendoscope, using either a

bugbee (30) or a loop electrode (82) to destroy the valve

Key points

• Primary endoscopic resection of both anterior

and posterior urethral valves is the preferred

treatment option in majority of cases.

• Routine second look procedures ensure

completeness of resection and de-obstruction.

• With modern instrumentation and good surgical

technique, complications directly related to

endoscopic manipulation are rare, e.g. iatrogenic

urethral strictures.

• Endoscopic treatment of pediatric urethral

strictures is associated with a cure rate of 50%,

with best results achieved for the short segment

idiopathic bulbar strictures.

• Endoscopic ureterocele puncture is an effective

method of producing upper tract decompression

and can be curative for the single system

intravesical ureteroceles.

• JJ stenting as the initial treatment for primary

obstructive megaureter is curative in up to 50%

of cases but is associated with a high morbidity

(up to 70%).

14

Pediatric Urology: Surgical Complications and Management

Edited by Duncan T. Wilcox, Prasad P. Godbole and Martin A. Koyle

© 2008 Blackwell Publishing Ltd. ISBN: 978-1-405-16268-5

102 Part V Endoscopic Surgery of the Urinary Tract

membrane. In situations where the urethra (meatus and

submeatal region) would not accommodate the instrument,

a small perineal urethrostomy was made by cutting

down upon the tip of a small sound placed in the urethra,

and the panendoscope was introduced via the perineum

just distal to the external sphincter. The valve membrane

was ablated using coagulating diathermy current.

Many authors have expressed concern about iatrogenic

stricture disease with this technique. Whitaker does not

report on the incidence in his series; however, Myers and

Walker [2] reported a 50% incidence of strictures in his

series of valves ablated in infancy with a 25% incidence

in the group as a whole.

Whitaker and colleagues [1] reported a 33% incidence

of continuous incontinence with a further 55%

stress incontinence in their series of 112 patients. It is

difficult to attribute a cause to incontinence as some of

their patients had additional bladder neck surgery, and

the noncompliant valve bladder had not been ruled out.

An addendum to that publication reported a dramatic

improvement in daytime incontinence in five boys following

treatment with imipramine.

In 1973, Williams and associates [3] advocated ablating

the valves with a diathermy hook electrode under

radiological control. This technique avoided a perineal

urethrostomy and was successful in several cases, but

complications arose when the hook engaged adjacent

tissue like the bladder neck or verumontanum. Whitaker

and Sherwood [4] subsequently modified the hook,

which minimized the risk of adjacent tissue injury but

did not completely eliminate it.

Hendren [5] was a proponent of valve ablation under

direct vision and in the narrower urethra passed the

insulated wire electrode (3F) alongside an 8F endoscope,

a technique he described as "a little awkward." He ablated

the valves using cutting diathermy current and reported

no strictures with this technique. Several boys in his

series required further resection of the valves, and once

again the continence outcome was muddied by synchronous

or metachronous bladder neck surgery.

In 1986, Diamond and Ransley [6] described the

Fogarty balloon catheter ablation technique and noted that

while not universally successful, they achieved satisfactory

results in nine of ten carefully selected patients. The

bladder is filled with contrast material via a small feeding

tube, which is then removed. A no. 4 Fogarty balloon catheter

is passed and the balloon inflated within the bladder.

The catheter is then slowly withdrawn under fluoroscopic

control until the balloon engages the valve. A sharp tug

causes rupture of the anterior membrane. The balloon is

then deflated and the catheter is removed. Cromie and

associates [7] describe a similar procedure using a modified

venous valvulotome in which under fluoroscopic

control the valvulotome is used to engage and cut the

obstructing membrane. They reported completeness of

ablation in 13 of 15 patients treated. Both reports describe

a 0% stricture incidence with their respective techniques.

Other described techniques include Mohan's [8] urethral

valvotome in which the valves are engaged and

destroyed without any fluoroscopy control and under

local analgesia, relying on the instrument design to

"catch" the valves. Mohan reports good results in eight

boys treated using his instrument based on symptoms

and improved radiologically findings on repeat voiding

cystourethrogram (VCUG).

Percutaneous, endoscopic, antegrade diathermy valve

ablation has been popularized by Zaontz and Firlit [9]

with a subsequent description by Gibbons and associates

[10] of endoscopic antegrade diathermy valve ablation

through a vesicostomy.

A bugbee or insulated wire electrode passed through

an infant (5-7.5F) cystourethroscope is a useful technique

in very small infants. Similarly, laser ablation using

the pottasium titanyl phosphate (KTP) (or Nd-YAG)

laser via a small fiber passed through the working channel

of the infant cystoscope has been reported as a safe

technique in newborn infants with no stricture formation

up to 3 years follow-up. This technique relies on

coagulative necrosis of the membranous tissue and the

tip of the electrode or fiber is placed in direct contact

with the valve tissue and the current is applied. The

process is repeated at multiple sites.

Videoendoscopy using a 8.5F (5° lens) to 10F (0° lens)

resectoscope and a cold knife hook working element

allow safe and effective ablation of urethral lesions without

risk of thermal injury or significant bleeding and can

be employed in infants as small as 2000 g. At the present

time, this is the safest and most effective technique for

ablation of membranous lesions of the infant urethra and

is the gold standard against which other techniques will

be compared. The membrane is cut at the 5 and 7 o'clock

positions until its connection with the verumontanum is

lost. Some surgeons advocate additional routine incision

at the 12 o'clock position. The bladder is drained via a

6-8F feeding tube for 24-48 h and subsequently removed.

A review of the last 100 valve resections at the author's

institution using a bugbee/insulated wire or the cold

knife resection technique shows a 21% incidence of

re-resection at planned follow-up check cystoscopy and

a 0% incidence of urethral stricture disease.

Chapter 14 Cystoscopy and Cystoscopic Interventions 103

The instrumentation available for valve ablation at the

author's institution includes:

1 6-7.5F graduated Wolff cystourethroscope with a 3F

working channel

2 10, 11, or 13.5F resectoscopes with cold knife hook

working element

3 Bugbee electrodes, Colling's Knife, and resectoscope

loops.

In situations where the available instrumentation was too

large to be safely accommodated in the infant urethra,

valve ablation was deferred to a later date. In the interim,

the urethra was serially and passively dilated using increasing

calibre urethral catheters changed on a weekly basis.

Complications

Urethral stricture

Obstructive oliguria or anuria

True uretero-vesical junction obstruction is rare in boys

with posterior urethral valves (PUV). Temporary UVJ

obstruction, presumed to be due to entrapment of the

UVJ by the thickened detrusor following decompression

of the bladder, is seen not uncommonly in clinical practice.

The oliguria or in severe cases anuria typically lasts

for 24-48 h and resolves spontaneously. There may be

associated perirenal urinary extravasation and nephrostomy

drainage of one or both kidneys may occasionally

become necessary, however most cases can be managed

with a policy of watchful waiting.

Sarkis et al. [15], Noe and Jenkins, and Jordan and

Hoover have all reported individual cases and Sarkis recommends

use of non-self-retaining tubes to drain the

bladder to minimize the risk.

Urinary tract infection

It is not uncommon for these children to have impaired

renal function, making these neonates particularly vulnerable

to developing urine infection following urethral

instrumentation, VCUG, and valve ablation. In addition

they may acquire drug resistant organisms and with prolonged

antibiotic treatment, secondary fungal infections

which are extremely difficult to treat.

Renal impairment

The condition is associated with considerable morbidity

and accounts for 16% of children with end-stage renal

failure and for 25% of all children with end-stage renal

disease (ESRD) who undergo renal transplantation,

according to the UK Transplant Registry 2005. Early

renal failure is attributed to inherent renal dysplasia. The

etiology of late onset renal impairment is more complex,

and dysplasia, bladder dysfunction, and urinary tract

infections are all implicated.

The fragile renal function in the neonatal period is

exquisitely sensitive and vulnerable to fluid imbalance and

urinary infection. Adequate precautions along with input

from nephrological colleagues help to maintain optimum

function and nadir creatinine levels along with a formal

glomerular filteration rate (GFR) value at 1 year of age

helps predict long-term outcome in these children.

Urinary incontinence

At the present time, urinary incontinence as a consequence

of valve ablation and associated sphincter damage

is rare. In the past, a high incidence was reported: 33%

continuous and an additional 55% stress incontinence in

a series of 34 patients who had both valve ablation and

Table 14.1 Incidence of urethral stricture following

ablation of posterior urethral valves.

Author Age at Number Follow-up Stricture

valve (n) (year) (%)

ablation

(year)

Myers [2] 1 14 1-10 50

Myers [2] 1 14 1-10 0

Nijman [11] and 1 85 5-19 0

Mitra [12] 0-15 82 1-21 3.6

Crooks [13] 1.5-? 36 4-? 08

Churchill [14] 1 to 1 173 ? 12

Dense strictures following diathermy ablation are on the

decline and most recent series report a 5% incidence

of strictures. Diagnosed early they may be amenable to

dilatation or visual internal urethrotomy. Recurrent

strictures or long segment strictures will require definitive

anastomotic or augmented urethroplasty.

Incomplete valve ablation

Completeness of valve ablation must be confirmed on

follow-up VCUG and or cystourethroscopy. Incidence of

incomplete ablation varies and in our review was seen in

21% of cases. It is more likely following bugbee or insulated

wire ablation through a small cystoscope, as the vision

is limited due to relatively poor flow of irrigation fluid.

It is prudent to rescope the urethra within 6-12 weeks

of the initial ablation to check completeness of resection,

as the consequences of persistent obstruction are potentially

disastrous.

104 Part V Endoscopic Surgery of the Urinary Tract

bladder neck surgery [1]. The subsequent description of

the valve bladder and the abnormal urodynamic patterns

observed in these children clarified the etiopathogenesis

of incontinence in majority of these patients.

Parkhouse and associates [16] reported daytime urinary

incontinence at 5 years of age to associate with

poor long-term renal outcome. A further compounding

factor, which may be responsible, is polyuria, secondary

to impaired renal function.

Recent studies, which have prospectively looked at the

development of bladder function in these children, have