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 To my wonderful wife, Susan, and Baby Grace, who make coming home early my daily goal.
I will always be grateful to my teachers and role models, Roy Witherington, J. Herbert Johnston,
Patrick C. Walsh, and Robert Douglas Jeffs.
John P. Gearhart, MD

To my wife, Kanda, and my children, Andy and Stephanie, who have been a constant source of love and support,
and to my brother Larry, who has shown the way. I am forever grateful for the encouragement and teachings of
my mentors—Alan Retik, Hardy Hendren, and Mike Mitchell—and the support of my present and past partners,
as well as my former residents and fellows. I dearly miss my first mentor, Dr. John Donohue, and my brother Skip,
both of whom we lost this past year.
Richard C. Rink, MD

To my wife, Jessica, my children, David and Caroline, and stepchildren, Emma and Sophie:
Thank you for your patience and love.

To the memory of my father, Claude, who left us in November 2008:

He held my hand along beautiful paths.

To Philip Ransley, David Frank, and Maggie Godley, who are my essential companions.

To Captain and his indestructible joy of life.

Pierre D. E. Mouriquand, MD
 Contributors
Mark C. Adams, MD Guy A. Bogaert, MD, PhD
Professor, Urology and Pediatrics, Monroe Carell Jr. Full Professor,
Children’s Hospital at Vanderbilt, Pediatric Urology, Katholieke Universiteit Leuven;
Vanderbilt University, Nashville, Tennessee Clinical Chief, Pediatric Urology, and Medical Manager,
Chapter 57: Augmentation Cystoplasty Ambulatory Surgery Center, Leuven University Hospitals,
Leuven, Belgium
Sharon Phillips Andreoli, MD Chapter 34: Urethral Duplication and Other Urethral
Professor of Pediatrics, Anomalies
James Whitcomb Riley Hospital for Children and Indiana
University School of Medicine; Claire Bouvattier, MD
Byron P. and Frances D. Hollett MCU, Paris Descartes University; PH,
Professor of Pediatrics, Cochin-Saint Vincent de Paul, Paris, France
Director, Division of Pediatric Nephrology, Chapter 35: Disorders of Sex Development: Endocrine
Indiana University School of Medicine, Aspects
Indianapolis, Indiana
Chapter 18: Glomerulonephritis in Children Berk Burgu, MD, FEAPU
Chapter 46: Acute Kidney Injury and Chronic Kidney Clinical Instructor,
Disease in Children Ankara University School of Medicine,
Department of Urology, Division of Pediatric Urology,
Darius J. Bägli, MDCM, FRCSC, FAAP, FACS Ankara, Turkey
Associate Professor, Department of Urology, Chapter 43: Cryptorchidism
University of Toronto; Chapter 51: Rhabdomyosarcoma
Associate Surgeon in Chief, Department of Surgery,
Hospital for Sick Children, Mark P. Cain, MD, FAAP
Toronto, Canada Professor of Urology, Department of Urology,
Chapter 53: Adrenal Tumors in Children Riley Hospital for Children,
Indiana University School of Medicine,
Linda A. Baker, MD Indianapolis, Indiana
Professor, Department of Urology, Pediatric Urology, and Chapter 56: Incontinent and Continent Urinary Diversion
Director of Pediatric Urology Research,
The University of Texas Southwestern Medical School at Anthony A. Caldamone, MD, MMS, FAAP, FACS
Dallas, Dallas, Texas Professor of Surgery (Urology) and Pediatrics,
Chapter 43: Cryptorchidism Alpert Medical School of Brown University;
Chief of Pediatric Urology, Hasbro Children’s Hospital,
G. M. Barker, MD, FRCS (PAEDS) Providence, Rhode Island
Consultant Urologist, Uppsala University Children’s Chapter 32: Prune-Belly Syndrome
Hospital, Uppsala, Sweden
Chapter 27: Pathophysiology of Bladder Dysfunction Anthony J. Casale, MD
Professor and Chairman, Department of Urology,
Lorenzo Biassoni, MSc, FRCP, FEBNM University of Louisville School of Medicine;
Honorary Senior Lecturer, Institute of Child Health, Chief of Urology, Kosair Children’s Hospital,
University College London; Consultant in Nuclear Medicine, University of Louisville Hospital, Louisville, Kentucky
Department of Radiology, Great Ormond Street Hospital for Chapter 55: Urinary Tract Trauma
Children, London, United Kingdom
Chapter 8: Radioisotope Imaging of the Kidney Marc Cendron, MD
and Urinary Tract Associate Professor of Surgery (Urology),
Harvard School of Medicine;
David Bloom, MD Attending Pediatric Urologist,
Chair, Department of Urology, and Jack Lapides Professor of The Boston Children’s Hospital,
Urology, University of Michigan Medical School, Boston, Massachussetts
C. S. Mott Children’s Hospital, Chapter 42: Disorders of the Penis and Scrotum
Ann Arbor, Michigan
Chapter 60: Injectable Bulking Agents in the Treatment of George Chiang, MD
Pediatric Urinary Incontinence Assistant Clinical Professor of Surgery (Urology),
University of San Diego;
Attending Pediatric Urologist,
Rady Children’s Hospital, San Diego, California
Chapter 42: Disorders of the Penis and Scrotum

vii
 viii Contributors

Lyn S. Chitty, PhD, MBBS, MRCOG Delphine Demède, MD

Reader in Genetics and Fetal Medicine, Consultant in Pediatric Urology,
Institute for Women’s Health and Unit of Clinical and Claude-Bernard University, Lyon, France;
Molecular Genetics, Institute of Child Health, University Consultant in Pediatric Urology, Hôpital
College Hospital London, London, United Kingdom Mère-EnfantsGHE
Chapter 4: Prenatal Diagnosis of Fetal Renal Bron, France
Abnormalities Chapter 41: Hypospadias

Bernard M. Churchill, MD, FRCS(C), FAAP Steven G. Docimo, MD

Judith and Robert Winston Chair in Pediatric Urology, Professor and Director, Pediatric Urology, and
and Director of Wendy and Ken Ruby Fund for Academic Vice-Chairman, Department of Urology,
Excellence in Pediatric Urology, Department of Urology, The University of Pittsburgh Medical Center;
David Geffen School of Medicine, University of California at Vice President of Medical Affairs,
Los Angeles; Director, Clark-Morrison Children’s Urological Children’s Hospital of Pittsburgh, Pittsburgh, Pennsylvania
Center, The Mattel Children’s Hospital at the Ronald Reagan Chapter 43: Cryptorchidism
Medical Center, Los Angeles, California
Chapter 20: Ureteropelvic Junction Anomalies: Congenital Ben O’Neill Donovan, MD
Ureteropelvic Junction Problems in Children Pediatric Urology Fellow,
University of Oklahoma College of Medicine;
Bartley G. Cilento Jr., MD, MPH Pediatric Urology Fellow,
Assistant Professor of Surgery, Harvard Medical School; Oklahoma University Medical Center,
Assistant in Urology, Children’s Hospital Boston, Boston, Oklahoma City, Oklahoma
Massachusetts Chapter 54: Neonatal Urologic Emergencies
Chapter 31: Bladder Diverticula, Urachal Anomalies, and
Other Uncommon Anomalies of the Bladder Jack S. Elder, MD
Clinical Professor of Urology, Case School of Medicine,
Clare E. Close, MD Cleveland, Ohio; Chief, Department of Urology, Henry Ford
Associate Clinical Professor of Surgery and Pediatrics, Health System, and Associate Director, Vattikuti Urology
University of Nevada School of Medicine, Las Vegas, Nevada Institute, Department of Urology,
Chapter 33: Posterior Urethral Valves Children’s Hospital of Michigan,
Detroit, Michigan
Christopher S. Cooper, MD Chapter 58: Bladder Outlet Surgery for Congenital
Professor, University of Iowa Department of Urology; Incontinence
Associate Dean, Student Affairs and Curriculum, University
of Iowa Carver College of Medicine; Director, Pediatric Waldo C. Feng, MD, PhD, FACS, FAAP
Urology, University of Iowa and the Children’s Hospital of Clinical Professor, University of Nevada Medical School;
Iowa, Iowa City, Iowa Chief of Urology, Sunrise Hospital and Children’s Medical
Chapter 26: Ureteral Duplication, Ectopy, and Ureteroceles Center, Las Vegas, Nevada
Chapter 20: Ureteropelvic Junction Anomalies: Congenital
Sarah M. Creighton, MD, FRCOS Ureteropelvic Junction Problems in Children
Consultant Gynaecologist,
University College Hospital, Fernando A. Ferrer, MD
London, United Kingdom Vice-Chair, Department of Surgery, and Associate Professor
Chapter 37: Adolescent Urogynecology of Surgery (Urology) and Pediatrics (Oncology), University
of Connecticut School of Medicine, Farmington, Connecticut;
Peter M. Cuckow, MBBS, FRCS (Paeds) Surgeon-in-Chief and Director, Division of Pediatric Urology,
Senior Lecturer, University College London, Connecticut Children’s Medical Center, Hartford, Connecticut
The Institute of Urology, Chapter 49: Oncologic Principles of Pediatric Genitourinary
The Institute of Child Health; Tumors
Consultant Urologist, Great Ormond Street Hospital for
Children, NHS Trust, London, United Kingdom J. David Frank, MBBS, FRCS
Chapter 1: Embryology of the Urogenital Tract Former Consultant Paediatric Urologist,
Bristol Urological Institute, Southmead Hospital
Chapter 40: Foreskin Bristol, United Kingdom
M. Daudon, PhD Chapter 16: Abnormal Migration and Fusion of the
Biologist, Hópital Necker-Enfants Malades, Paris, France Kidneys
Chapter 48: Urolithiasis in Children Dominic Frimberger, MD
Associate Professor, Pediatric Urology,
William De Foor, MD, MPH, FAAP Department of Urology,
Associate Professor, Division of Pediatric Urology, Oklahoma University College of Medicine;
Cincinnati Children’s Hospital, Cincinnati, Ohio Attending Physician, The Children’s Hospital, Pediatric
Chapter 47: Pediatric Renal Transplantation: Medical and Urology Section, Oklahoma University, Oklahoma City,
Surgical Aspects Oklahoma
Chapter 54: Neonatal Urologic Emergencies
 Contributors ix

Leo C. T. Fung, MD, FACS, FRCS(C), FAAP Isky Gordon, FRCR, FRCP
Associate Professor, Department of Urologic Surgery, Professor of Paediatric Imaging,
University of Minnesota, Institute of Child Health, University College London,
Minneapolis, Minnesota London, United Kingdom
Chapter 10: Urodynamic Studies of the Upper Chapter 8: Radioisotope Imaging of the Kidney and Urinary
Urinary Tract Tract

M. F. Gagnadoux, MD Daniela Gorduza, MD

Senior Consultant in Pediatric Nephrology, Consultant in Pediatric Urology,
Hopital Enfants Malades, Paris, France Claude-Bernard University, Lyon I,
Chapter 48: Urolithiasis in Children France; Consultant in Pediatric Urology, Hôpital ­
Mère-EnfantsGHE,
John P. Gearhart, MD, FAAP, FACS, FRCS (Hon) (Ed) Bron, France
Professor and Chief of Pediatric Urology, Chapter 41: Hypospadias
Department of Urology,
Johns Hopkins University School of Medicine, J. Damien Grattan-Smith, MD
Baltimore, Maryland Assistant Professor of Radiology,
Chapter 30: The Bladder Exstrophy–Epispadias–Cloacal Emory University School of Medicine;
Exstrophy Complex Staff Radiologist,
Children's Healthcare of Atlanta,
Kenneth I. Glassberg, MD Atlanta, Georgia
Professor of Urology, Chapter 11: Magnetic Resonance Imaging of the
Columbia University, College of Physicians and Surgeons; Pediatric Urinary Tract
Director, Division of Pediatric Urology,
Morgan Stanley Children’s Hospital of New York– Saul P. Greenfield, MD
Presbyterian, New York, New York Clinical Professor of Urology, State University of New York
Chapter 17: Multicystic Dysplastic Kidney Disease at Buffalo School of Medicine and Biomedical Sciences;
Director, Pediatric Urology, Women and Children’s Hospital
Prasad P. Godbole, MBBS, FRCS, FRCS (Paeds), of Buffalo, Buffalo, New York
FEAPU Chapter 23: The Diagnosis and Medical Management of
Honorary Senior Lecturer, Primary Vesicoureteral Reflux
University of Sheffield;
Consultant Paediatric Urologist, Mohan S. Gundeti, MB, MS, DNBE, MCh (Urol),
Sheffield Children’s Hospital, NHS Foundation Trust, FEBU, FICS, FRCS (Urol), FEAPU
Sheffield, United Kingdom Assistant Professor of Urology in Surgery and Pediatrics,
Chapter 44: Patent Processus Vaginalis The University of Chicago and Pritzker School of Medicine;
Director, Pediatric Urology, and Chief Pediatric Urologist,
Margaret L. Godley, PhD, CBiol, MIBiol Comer Children’s Hospital, the University of Chicago
Clinical Scientist, Honorary Fellow in Pediatric Urology, Medical Center, Chicago, Illinois
Institute of Child Health, University College London and Chapter 50: Wilms’ Tumor
Great Ormond Street Hospital for Children, London, United
Kingdom George Haycock, MB, BChir, FRCP, FRCPCH, DCH
Chapter 22: Vesicoureteral Reflux: Pathophysiology and Emeritus Professor of Paediatrics, Guy’s, King’s, and
Experimental Studies Sr. Thomas’ Hospitals School of Medicine, King’s College,
University of London;
Jens Goebel, MD Emeritus Consultant Paediatrician and Paediatric
Associate Professor of Pediatrics, Cincinnati Children’s Nephrologist, Guy’s and Sr. Thomas’ NHS Foundation Trust,
Hospital, University of Cincinnati; Medical Director, Kidney London, United Kingdom
Transplantation, Cincinnati Children’s Hospital, Cincinnati, Chapter 2: Nephrourology from Fetushood to Adulthood
Ohio
Chapter 47: Pediatric Renal Transplantation: Medical and Anna-Lena Hellström, MD
Surgical Aspects Urotherapist and Professor,
Queen Silvia Children’s Hospital,
Ricardo González, MD Göteborg, Sweden
Professor of Urology, Chapter 28: Pragmatic Approach to the Evaluation and
Thomas Jefferson University, Management of Non-Neuropathic Daytime Voiding
Philadelphia, Pennsylvania; Disorders
Senior Consultant, University Children’s Hospital Zurich,
Zurich, Switzerland Terry W. Hensle, MD
Chapter 59: Artificial Urinary Sphincter Given Foundation Professor of Urology and Vice Chair,
Department of Urology,
Columbia University, College of Physicians and Surgeons,
New York, New York
Chapter 3: Renal Function, Fluids, Electrolytes, and
Nutrition from Birth to Adulthood
  Contributors

David B. Joseph, MD, FACS, FAAP Erica H. Lambert, MD

Professor of Surgery, Fellow and Instructor,
University of Alabama at Birmingham; Vanderbilt Medical Center, Nashville, Tennessee
Chief of Pediatric Urology, Chapter 3: Renal Function, Fluids, Electrolytes, and
University of Alabama at Birmingham, Nutrition from Birth to Adulthood
Children’s Hospital, Birmingham, Alabama
Chapter 21: Ureterovisical Junction Anomalies: Henri Lottmann, MD, FEBU, FRCS (Eng), FEAPU
Megaureters Consultant in Pediatric Urology,
Appendix Hôpital Necker-Enfants Malades, Paris, France
Chapter 48: Urolithiasis in Children
Antoine E. Khoury, MD
Adjunct Assistant Professor, Department of Medicine, Padraig S. J. Malone, MCh, FRCSI, FRCS, FEAPU
University of Minnesota, St. Paul, Minnesota Consultant Paediatric Urologist,
Chapter 10: Urodynamic Studies of the Upper Urinary Southampton University Hospitals, NHS Trust,
Tract Southampton, United Kingdom
Chapter 61: Fecal Incontinence in Pediatric Urology
Andrew J. Kirsch, MD, FACS, FAAP
Clinical Professor of Urology, Ranjiv Mathews, MD, FAAP
Academic Fellowship Director, Associate Professor of Pediatric Urology,
Emory University School of Medicine; The Johns Hopkins School of Medicine,
Attending Pediatric Urologist, Baltimore, Maryland
Residency Director, Children’s Healthcare of Atlanta, Chapter 6: Endoscopy of the Lower Urinary Tract
Atlanta, Georgia
Chapter 11: Magnetic Resonance Imaging of the Pediatric Gordon A. McLorie, MD, FRCSC, FAAP, CAQ
Urinary Tract Professor of Urology,
Wake Forest University School of Medicine;
Stanley J. Kogan, MD Chief, Pediatric Urology,
Clinical Professor of Urology, Albert Einstein College of Brenner Children’s Hospital,
Medicine; Wake Forest University, Baptist Medical Center,
Chief, Pediatric Urology, Children’s Hospital at Montefiore, Winston-Salem, North Carolina
Bronx, New York Chapter 53: Adrenal Tumors in Children
Chapter 45: The Pediatric Varicocele
Maria Menezes, MBBS, MS, MRCS (Ed)
Kristin A. Kozakowski, MD National Children’s Hospital, Tallaght, Dublin, Ireland
Clinical Fellow, Division of Pediatric Urology, University Chapter 24: Endoscopic Treatment of Vesicoureteral Reflux
of Toronto; Clinical Fellow, Division of Pediatric Urology,
Hospital for Sick Children, Toronto, Canada; Resident, Peter D. Metcalfe, MD, FRCSC
Department of Urology, Columbia University, New York, Assistant Professor, Department of Surgery, University of
New York Alberta, Stollery Children’s Hospital, Edmonton, Alberta,
Chapter 17: Multicystic Dysplastic Kidney Disease Canada
Chapter 56: Incontinent and Continent
Bradley P. Kropp, MD Urinary Diversion
Professor, Department of Urology,
The University of Oklahoma Health Sciences Center, College Michael E. Mitchell, MD
of Medicine, Oklahoma University College of Medicine; Professor Emeritus, University of Washington, Seattle,
Attending Physician, Pediatric Urology, Washington; Professor and Chief of Pediatric Urology,
The Children’s Hospital of Oklahoma, Children’s Hospital of Wisconsin,
Oklahoma University Medical Center, Oklahoma City, Medical College of Wisconsin, Milwaukee, Wisconsin
Oklahoma
Chapter 33: Posterior Urethral Valves
Chapter 54: Neonatal Urologic Emergencies
Pierre D. E. Mouriquand, MD, FRCS (Eng), FEAPU
G. Läckgren, MD, PhD Professor of Pediatric Urology,
Professor and Consultant Urologist, Claude-Bernard University;
University Children’s Hospital Uppsala, Uppsala, Sweden Head of Pediatric Urology,
Chapter 27: Pathophysiology of Bladder Dysfunction Hospices Civils de Lyon, Hôpital Mère-Enfants,
Lyon, France
Yegappan Lakshmanan, MD, (FRCS Ed) Chapter 41: Hypospadias
Chief, Department of Pediatric Urology,
Children’s Hospital of Michigan, Pierre-Yves Mure, MD
Detroit, Michigan Professor of Pediatric Surgery,
Chapter 15: Tissue Engineering in Pediatric Urology Claude-Bernard University, Lyon, France;
Consultant in Pediatric Surgery,
Hôpital Mère-EnfantsGHE,
Bron, France
Chapter 41: Hypospadias
 Contributors xi

Kenneth G. Nepple, MD Faridali Rashji, MD, FRCPC

Resident, Department of Urology, University of Iowa, Iowa Associate Professor, Department of Pediatric Radiology,
City, Iowa and Chief of Pediatric Radiology, Children’s Hospital of
Chapter 26: Ureteral Duplication, Ectopy, and Ureteroceles Oklahoma, Oklahoma University Medical Center, Oklahoma
City, Oklahoma
Tryggve Nevéus, MD, PhD Chapter 54: Neonatal Urologic Emergencies
Associate Professor, Department of Women’s and Children’s
Health, Uppsala University; Philip G. Ransley, MD
Consultant in Pediatric Nephrology, Senior Lecturer in Paediatric Urology,
Uppsala University Children’s Hospital, Uppsala, Sweden Institute of Child Health, University College London and
Chapter 29: Nocturnal Enuresis Great Ormond Street Hospital for Children;
Consultant Paediatric Urologist,
Hiep T. Nguyen, MD Great Ormond Street Hospital, London, United Kingdom
Assistant Professor of Surgery, Harvard Medical School; Chapter 22: Vesicoureteral Reflux: Pathophysiology and
Assistant in Urology, Children’s Hospital Boston, Boston, Experimental Studies
Massachusetts
Chapter 31: Bladder Diverticula, Urachal Anomalies, and William G. Reiner, MD
Other Uncommon Anomalies of the Bladder Professor of Urology and Child Psychiatry,
University of Oklahoma Health Sciences Center,
Rien J. M. Nijman, MD, PhD Oklahoma City, Oklahoma, and Johns Hopkins School of
Professor and Chair, Department of Urology, Medicine, Department of Urology, Baltimore, Maryland
University Medical Centre Groningen, Chapter 39: Psychological and Psychiatric Aspects of
Groningen, The Netherlands Genitourinary Conditions
Chapter 9: Urodynamic Studies of the Lower Urinary Tract
Alan B. Retik, MD
John Park, MD Professor of Surgery (Urology), Harvard Medical School;
Cheng-Yang Chang Endowed Professor of Pediatric Urology, Chief, Department of Urology, Children’s Hospital Boston,
University of Michigan Medical School; Boston, Massachusetts
Chief, Division of Pediatric Urology, Chapter 25: Surgery for Vesicoureteral Reflux
C. S. Mott Children’s Hospital, Ann Arbor, Michigan
Chapter 25: Surgery for Vesicoureteral Reflux Richard C. Rink, MD, FAAP, FACS
Robert A. Garrett Professor of Pediatric Urology,
Chapter 60: Injectable Bulking Agents in the Treatment of Indiana University School of Medicine;
Pediatric Urinary Incontinence Chief, Pediatric Urology,
James Whitcomb Riley Hospital for Children, Indiana
Craig A. Peters, MD University Medical Center,
John E. Cole Professor of Urology, Indianapolis, Indiana
University of Virginia;
Chief, Division of Pediatric Urology, Chapter 36: Surgical Management of Female Genital
University of Virginia Health System Anomalies, Disorders of Sexual Development, Urogenital
Charlottesville, Virginia Sinus, and Cloacal Anomalies
Chapter 19: Congenital Urine Flow Impairments of the Chapter 57: Augmentation Cystoplasty
Upper Urinary Tract: Pathophysiology and Experimental
Studies Jonathan H. Ross, MD
Chief, Division of Pediatric Urology,
Lisandro Piaggio, MD Rainbow Babies and Children’s Hospital,
Pediatric Urologist and Chief, Division of Pediatric Urology, Cleveland, Ohio
Hospital Italiano de Buenos Aires, Buenos Aires, Argentina Chapter 52: Testicular Tumors
Chapter 59: Artificial Urinary Sphincter
Joao Luiz Pippi Salle, MD, PhD, FAAP, FRCSC
Prem Puri, MS, FRCS, FRCS (Ed), FACS, FAAP (Hon) Professor, Department of Surgery (Urology),
Newman Clinical Research Professor, University of Toronto;
School of Medicine and Medical Science, University College Head, Division of Urology,
Dublin; Consultant Paediatric Surgeon and Director of Hospital for Sick Children,
Research, Children’s Research Centre, Our Lady’s Children’s Toronto, Canada
Hospital, Dublin, Ireland Chapter 58: Bladder Outlet Surgery for Congenital
Chapter 24: Endoscopic Treatment of Vesicoureteral Reflux Incontinence

J. Todd Purves, MD, PhD Caroline Sanders, BSc Hons, PGD, RCN, RN
Assistant Professor, Department of Urology, Pediatrics, Cell Consultant Nurse,
Biology, and Anatomy, Medical University of South Carolina, Alder Hey Children’s Hospital, NHS Foundation Trust,
Charleston, South Carolina Liverpool, United Kingdom
Chapter 15: Tissue Engineering in Pediatric Urology Chapter 14: Nursing Intervention in Pediatric Urology
Chapter 30: The Bladder Exstrophy–Epispadias–Cloacal
Exstrophy Complex
 xii Contributors

Sovrin M. Shah, MD Mark D. Stringer, MS, FRCP, FRCS, FRCS Ed

Assistant Professor of Urology, Professor of Anatomy,
Albert Einstein College of Medicine, Bronx, New York; Department of Anatomy and Structural Biology,
Physician-in-Charge, Otago School of Medical Sciences,
Female Urology, Voiding Dysfunction, and Pelvic University of Otago, Dunedin, New Zealand
Reconstructive Surgery, Chapter 44: Patent Processus Vaginalis
Beth Israel Medical Center, New York, New York
Chapter 17: Multicystic Dysplastic Kidney Disease Tatum Tarin, MD
Urology Resident,
Curtis Sheldon, MD Stanford University, Department of Urology,
Assistant Professor of Surgery, University of Cincinnati; Stanford, California
Director, Pediatric Urology, Cincinnati Children’s Hospital, Chapter 13: Pediatric Urinary Tract Infections
Cincinnati, Ohio
Chapter 47: Pediatric Renal Transplantation: Medical and A. A. Thakre, MD
Surgical Aspects Consultant Pediatric Department Urologist, Division of
Pediatric Urology, Department of Pediatric Surgery, Civil
Rajesh Shinghal, MD Hospital, R. J. Medical College, Gujarat University;
Clinical Instructor, Director, Centre for Pediatric Urology, Minimally Invasive
Stanford University Medical Center, Stanford, California; Pediatric Urology Centre, Children’s Continence Centre,
Associate Chief of Urology, Santa Clara Valley Medical Welcare Speciality Hospital, Ahmedabad,
Center, San Jose, California Gujarat, India
Chapter 13: Pediatric Urinary Tract Infections Chapter 7: Laparoscopy in Pediatric Urology

Linda M. Dairiki Shortliffe, MD Julian Wan, MD

Stanley McCormick Memorial Professor and Chair, Clinical Associate Professor, University of Michigan,
Department of Urology, Stanford University School of Department of Urology, University of Michigan Medical
Medicine; Chief of Urology, Stanford University Medical School; Attending Pediatric Urologist,
Center; Chief of Pediatric Urology, Lucile Salter Packard C. S. Mott Children’s Hospital, Ann Arbor, Michigan
Children’s Hospital, Stanford, California Chapter 23: The Diagnosis and Medical Management of
Chapter 13: Pediatric Urinary Tract Infections Primary Vesicoureteral Reflux
Chapter 60: Injectable Bulking Agents in the Treatment of
Ulla Sillén, MD Pediatric Urinary Incontinence
Professor, Department of Pediatric Surgery and Urology,
Queen Silvia Children’s Hospital, Sahlgreenska Academy, S. M. Whitten, MBBS, MRCOG
University of Gothenburg; Head of Pediatric Urology, Consultant Obstetrician,
Pediatric Uronephrologic Center, Queen Silvia Children’s University College London, NHS Foundation Trust,
Hospital, Gothenburg, Sweden London, United Kingdom
Chapter 28: Pragmatic Approach to the Evaluation and Chapter 4: Prenatal Diagnosis of Fetal Renal Abnormalities
Management of Non-Neuropathic Daytime Voiding
Disorders Duncan T. Wilcox, MBBS, MD
Associate Professor, University of Colorado at Denver,
Howard M. Snyder III, MD Denver, Colorado;
Professor of Urology, University of Pennsylvania School Chair of Pediatric Urology,
of Medicine; Director of Surgical Teaching, Pediatric The Children's Hospital, Aurora, Colorado
Urology, Children’s Hospital of Philadelphia, Philadelphia,
Pennsylvania Chapter 51: Rhabdomyosarcoma
Chapter 26: Ureteral Duplication, Ectopy, and John R. Woodard, MD
Ureteroceles Formally Clinical Professor of Urology and Director of
Pediatric Urology, Emory University School of Medicine;
Arne Stenberg, PhD Formally Chief of Urology, Henrietta Egleston Hospital for
Associate Professor, Uppsala University; Head, Children, Atlanta, Georgia
Department of Pediatric Surgery and Urology,
Uppsala University Children’s Hospital, Uppsala, Sweden Chapter 32: Prune-Belly Syndrome
Chapter 29: Nocturnal Enuresis C.R.J. Woodhouse, MB, FRCS, FEBU
Professor of Adolescent Urology,
Louise C. Strawbridge, MRCOG University College; Consultant Adolescent Urologist,
Specialist Registrar, University College London Hospitals, London,
University College Hospital, United Kingdom
London, United Kingdom
Chapter 38: Ambiguous Genitalia in Male Adolescents
Chapter 37: Adolescent Urogynecology
 Contributors xiii

Mark Woodward, MD, FRCS (Paed) C. K. Yeung, MD

Consultant Paediatric Urologist, Department of Paediatric Honorary Clinical Professor, Paediatric Surgery and
Surgery, Bristol Royal Hospital for Children, Bristol, Paediatric Urology; Adjunct Professor, Institute of Chinese
United Kingdom Medicine, The Chinese University of Hong Kong,
Chapter 16: Abnormal Migration and Fusion of the Kidneys Centre of Clinical Trials on Chinese Medicine,
Prince of Wales Hospital, Hong Kong
Adrian S. Woolf, MD Chapter 7: Laparoscopy in Pediatric Urology
Professor of Nephrology and Head of Nephro-Urology Unit,
Institute of Child Health, University College London, Paul Zelkovic, MD
Great Ormond Street Hospital for Children, London, United Clinical Instructor,
Kingdom Department of Urology,
Chapter 12: Genes, Urinary Tract Development, and New York Medical College,
Human Disease Valhalla, New York
Chapter 45: The Pediatric Varicocele
Elizabeth B. Yerkes, MD
Assistant Professor of Urology, Northwestern University, J. Michael Zerin, MD
Feinberg School of Medicine; Professor of Radiology and Chief, Department of Pediatric
Attending Urologist, Imaging, Children’s Hospital of Michigan, Wayne State
Children's Memorial Hospital, Chicago, Illinois University School of Medicine, Detroit, Michigan
Chapter 36: Surgical Management of Female Genital Chapter 5: Morphologic Studies of the Urinary Tract
Anomalies, Disorders of Sexual Development, Urogenital
Sinus, and Cloacal Anomalies
 Foreword
The field of pediatric urology continues to change dramati- and neonatal emergencies. The second edition has been rede-
cally. Certainly there have been very significant advances signed in a full-color format that is more readable. In addition,
since the first edition of this book was published in 2001. In the content of the chapters has been updated with new topics,
this new edition, previous chapters have been updated and an techniques, and procedures. The text and images will also be
impressive number of new chapters has been added. The new available online with the purchase of the book.
edition ­ continues to be an international one edited by three In summary, the second edition continues to be an excel-
well-known pediatric urologists from the United States and lent, updated, and comprehensive textbook edited by world-
abroad. renowned pediatric urologists who have compiled a group of
The philosophy of the textbook is similar to that of the first international experts to contribute to the textbook. This book
edition—in other words, the editors and authors provide up- should be of great benefit to all physicians caring for infants
to-date information on the entire spectrum of pediatric urol- and children with pediatric urologic disorders.
ogy, including new chapters devoted to magnetic resonance
imaging of the pediatric urinary tract, tissue engineering, Alan B. Retik, MD

xv
 Preface
Pediatric urology has matured a great deal over the last decade, textbook without a special tribute to six of the “fathers” of
and, with the onset of the third millennium, this new textbook modern pediatric urology: Sir David Innes Williams from
is an important milestone not only for the third generation of Great Ormond Street Hospital, the late Mr. J. Herbert Johnston
pediatric urologists but also for all related specialists. This edi- from Alder Hey Hospital, Kelm Hjalmas, John W. Duckett,
tion is more than a comprehensive update; in it, the leading Jean Cendron, and Robert D. Jeffs.
specialists from around the world have shed new light on the The three editors would like to give their deep thanks for
various aspects of pediatric urology. the outstanding work done by all of the contributors in the
With the growing importance of new imaging, molecular production of this innovative textbook. They also wish to
biology, genetics, experimental surgery, minimally invasive express their gratitude to Mr. Scott Scheidt from Elsevier who
surgery, antenatal uronephrology, and evidence-based medi- has been behind each step of the new edition with incredible
cine in the field, different and more pertinent approaches to efficiency, from its revision and update to its delivery.
many familiar but also less common situations are detailed Finally, the editors would not have spent their evenings
here with a constant wish for clarity and honesty. and weekends for this textbook without the wonderful and
Such an enormous amount of data would not exist without permanent support of their families.
the outstanding work done by three generations of pediatric
urologists from all over the world, who have built our beauti- John P. Gearhart
ful specialty brick by brick, article after article, textbook after Richard C. Rink
textbook. They should all be thanked warmly. It is ­impossible Pierre D. E. Mouriquand
to mention all of them here, but we could not preface this

xvii
 P A R T I
BASICS

S E C T I O N 1
EMBRYOLOGY AND PHYSIOLOGY
OF THE URINARY TRACT

CHAPTER 1
EMBRYOLOGY OF THE UROGENITAL TRACT
Peter M. Cuckow

Human gestation starts with fertilization, defined by fusion Buccopharyngeal Amniotic cavity
of the nuclear material of a spermatozoon and a definitive membrane
oocyte, and continues until the birth of a fully developed Mesoderm
infant approximately 38 weeks later. During the first 10 weeks, Ectoderm
the body form and organ systems that are present at birth
develop (embryogenesis). The remaining 28 weeks are spent
in the maturation, growth, and development of function of
the body, enabling independent life after separation from the
placental support system. An understanding of embryogen-
esis and its disorders explains many of the anomalies encoun- Endoderm
tered in pediatric urologic practice and offers some clues to the
appropriate clinical approach to these conditions. Yolk sac

DEVELOPMENT OF EARLY URINARY TRACT Cloacal membrane

PRECURSORS Figure 1-1 An early cross section of the human embryonic disc. The
disc separates the ectoderm-lined amniotic cavity from the endoderm-
lined yolk sac. Ingrowth of cells from the primitive streak forms a third
After fertilization, the developing zygote, with its full dip-
layer between them, the mesoderm. This is deficient at the head and
loid complement of genetic material, travels down the fallo- tail ends, the locations, respectively, of the buccopharyngeal mem-
pian tube to reach the uterus. During the 5 to 6 days it takes brane and the cloacal membrane.
to complete this journey, the zygote divides to form a ball of
cells called a blastocyst. Further rapid divisions and the forma-
tion of two cavities on either side of an embryonic disc follow
implantation of the zygote into the endometrium (Fig. 1-1).
These cavities are called the amniotic cavity and the yolk sac.
The disc itself is initially formed from two layers of cells—the
ectoderm on its amniotic surface and the endoderm on its yolk
sac surface. At approximately 15 days, the inpouring of cells Head
from a differentiated midline area, called the primitive streak, Tail fold
forms a third layer of mesoderm throughout most of the disc.
This is further subdivided into three parallel areas, designated
laterally from the primitive streak as the paraxial, the inter- Yolk sac
mediate, and the lateral plate mesoderm. It is largely from the
intermediate mesoderm that the urinary and genital organ AllantoisCloacal
systems will develop. membrane
In two areas of the early embryonic disc, the endoderm Figure 1-2 The embryonic disc has folded, including an endo­derm
and ectoderm remain opposed. These form the buccopharyn- layer from the yolk sac that will form the hindgut and foregut. The
geal membrane at the head end and the cloacal membrane at cloacal membrane faces anteriorly, and the cloaca is defined distal to
the tail end (see Fig. 1-1). As the embryo continues to grow the confluence of the hindgut and allantois.


  part I: Basics

rapidly, its dorsal surface bulges into the amniotic cavity, and A priority of the embryo is to establish the seeds of its own
its head and tail ends fold forward to form the head and the reproduction. Thus, early in its development, primordial germ
tail folds, respectively. During this process, the lining or endo- cells are set aside in the wall of the yolk sac. These cells have
derm of the yolk sac is included within the two folds, where ameboid characteristics that enable them to migrate later in
it is the precursor of the foregut and the hindgut, respectively gestation to take part in gonadal differentiation and the for-
(Fig. 1-2). As folding of the tail end continues, the connecting mation of the genital tracts.
stalk and allantois are formed and displaced onto the front
surface of the embryo (see Fig. 1-2). The cloacal membrane is
also brought to the front of the tail fold, below the allantois. RENAL DEVELOPMENT
The allantois gains continuity with the developing hindgut
and defines the cloaca as the portion of hindgut distal to their From early in the 4th week of gestation, three nephric struc-
confluence (Fig. 1-3). The cloacal membrane is seen on the tures develop in succession from the intermediate mesoderm
surface of the embryo at the center of a depression called the that runs the length of the embryo. The first, or pronephros,
proctodeum. On either side of this are two surface elevations, appears in the cervical portion and rapidly regresses, with-
the urogenital folds, which join at their upper ends in the out forming any nephronlike structures (although it does
genital tubercle. Growth of the anterior abdominal wall above develop excretory function in amphibian larvae and some
the cloacal membrane, coupled with regression of the tail fold, fish). Subsequently, the appearance of tubular structures in the
causes its relative displacement toward the tail end of the midportion (thoracic and lumbar sections) of the intermedi-
embryo, facing downward (Figs. 1-4 and 1-5). ate mesoderm heralds the development of the mesonephros.

Allantois
Gonad
Gonad
Mesonephros
Genital
tubercle Mesonephric duct

Paramesonephric duct
Urogenital
sinus Blastema

Tail fold
Ureteric bud
Cloacal Urogenital
membrane Ureteric bud sinus
Cloaca
Müllerian tubercle
A Septum
B
Figure 1-3 A, The tail end of the human embryo during the 5th week of gestation (lateral view). The ureteric bud begins to grow posteriorly from
the distal part of the mesonephric duct. The urorectal septum advances forward to divide the cloaca into an anterior urogenital sinus and a poste-
rior rectum. As it does so, infolding of the lateral walls of the cloaca helps to complete the division. The gonad precursors are visible anteromedial
to the mesonephroi; their paired ducts descend lateral to the mesonephric ducts and join at the urogenital sinus to form the müllerian tubercle.
The cloacal membrane faces forward and upward. B, The same gestation looking from behind the urogenital sinus. The mesonephric ducts enter
the sinus posteriorly. The müllerian ducts come together and indent the sinus at the müllerian tubercle.

Mesonephros Gonad
Allantois

Mesonephric
duct

Genital Metanephros
tubercle
Cloacal
membrane

Urogenital sinus
Septum

A Rectum B
Figure 1-4 A, The tail end of the embryo during the 6th week of gestation (lateral view, müllerian and genital development not shown). The uro-
rectal septum advances toward the cloacal membrane. The kidneys are forming, and the origin of the ureteric bud approaches the urogenital sinus
as the end of the mesonephric duct is incorporated into its posterior wall. Growth of the anterior abdominal wall is accompanied by expansion of
the vesicourethral canal. The orientation of the cloacal membrane is beginning to change. B, Same gestation, posterior view.
 chapter 1: Embryology of the Urogenital Tract 

Gonad
Allantois
Kidney

Urogenital
membrane
Trigone
Anal
membrane

A B
Figure 1-5 A, The tail end of the embryo during the 8th week of gestation (lateral view, müllerian and genital development not shown). The kid-
ney ascends from the pelvis as the mesonephric duct, and its ureteric origins are further incorporated into the urogenital sinus. Cloacal septation is
complete, and the membranes, which have started to degenerate, are facing downward. B, Same gestation, from behind the urogenital sinus. The
trigone is formed with separation of the mesonephric ducts and ureteric orifices.

Mesonephric or wolffian ducts form lateral to this region and 2o

grow downward to enter the lateral wall of the cloaca. These 1o
primitive renal units possess capillary tufts at the proximal
ends of simple nephrons and probably begin functioning at 3B
3o
between 6 and 10 weeks, producing small amounts of urine 1B
(see Fig. 1-4). The mesonephros forms the definitive kidney 2B
in amphibians and most fish. At approximately 10 weeks of
human gestation, the lower parts of the mesonephroi degener-
ate, leaving the upper nephrons, which will contribute to the
developing genital duct ­system.
At the beginning of the 5th week of gestation, a diverticu-
lum appears on the posteromedial aspect of the lower portion Figure 1-6 Development of the kidney and the final position of the
of the mesonephric ducts (see Fig. 1-4). This structure, the ureteric orifice in the bladder related to the starting position of the
ureteric bud on the mesonephric duct. A normally placed ureteric bud
ureteric bud, grows backward toward the lowest or sacral (1B) induces normal renal development and results in a normally posi-
portion of the intermediate mesoderm (called the metanephric tioned, nonrefluxing ureteric orifice (1O). A low origin (2B) may induce
blastema) and penetrates it late in the 5th week. The ureteric an abnormal kidney whose orifice (2O) is placed in a laterally ectopic
bud and metanephric blastema interact to induce nephrogen- position and is prone to reflux due to a short ureteric tunnel. A high
esis that continues throughout gestation and is complete just origin (3B) may also induce an abnormal kidney with an orifice that re-
before term at 36 weeks. The tip of the ureteric bud dilates to tains a closer association with the mesonephric duct (3O). (After Mackie
form the renal pelvis, and then it begins to branch dichoto- CG, Stephens FD, eds. Duplex kidneys: a correlation of renal dysplasia
mously. The first four generations coalesce to form the major with the position of the ureteric orifice. J Urol. 1975;114:274.)
calyces, and the sixth to eighth generations similarly fuse to
form the minor calyces. The next eight generations form the
definitive collecting duct system. Blastema cells collect around the amniotic cavity and is important for its lung and skeletal
the tip of each collecting duct and form nephrons, compris- development.
ing a Bowman capsule, proximal convoluted tubule, loop of
Henle, and distal convoluted tubule. Branches of the internal
iliac artery feed each nephron and form capillary tufts within
Anomalies of Ureteric Bud Development
the Bowman capsule. The branching of the ureteric bud is
complete by about 14 weeks, but new generations of nephrons
Renal Agenesis and Dysplasia
continue to be produced within the parenchyma throughout Failure of development of the ureteric bud on one side results in
the remainder of gestation. renal agenesis, which is found in approximately 0.1% of the pop-
The embryonic kidney has a lobulated external appear- ulation. Alternatively, misplacement of the ureteric bud on the
ance and ascends from its pelvic position during the 6th mesonephric duct prevents it from contacting the blastema with
to 9th weeks. During this process, lower branches of the potential to form a normal kidney (Fig. 1-6). Instead, the bud
vascular supply degenerate as upper branches form suc- misses and induces abnormal nephrogenesis, resulting in a dys-
cessively from the aorta, until it attains its definitive renal plastic kidney. Buds arising below the normal position arrive at
artery and lies in its final lumbar position. The pelvic kidney the urogenital sinus earlier in gestation and migrate further lat-
faces anteriorly and will rotate medially about 90 degrees erally, presenting a lateral ectopic orifice which is more prone to
during its ascent so that the hilum faces anteromedially in reflux owing to a shorter course through the bladder wall. This
the renal fossa. theory explains the association of reflux and renal dysplasia
Fetal urine is produced from the 10th week onward, (Fig. 1-7). A bud arising higher on the mesonephric duct arrives
but initially the plasma filtrate is little modified, because at the urogenital sinus later in gestation and has less time to
tubular function starts to develop only from the 14th week. migrate away from the mesonephric duct opening. Such a ure-
Throughout the latter part of gestation, the fetal kidneys ter may drain in a medially ectopic position on the trigone or
provide more than 90% of the amniotic fluid. An adequate into one of the mesonephric duct derivatives. The kidneys that
volume of this fluid allows the fetus to move freely within drained by these ectopic ureters are usually dysplastic.
  part I: Basics

A B
Figure 1-7 Cystogram (A) and technetium 99m dimercaptosuccinic acid (Tc99-DMSA) scan (B) characterizing unilateral reflux in a boy. This
condition manifested antenatally, and there was no history of urinary tract infection. The refluxing kidney is small and functions poorly, which is
typical of the dysplasia that accompanies reflux.

Duplex Kidney
BLADDER, TRIGONE, AND LOWER URETERIC
Duplex kidney, the most common of renal anomalies, arises DEVELOPMENT
when two ureteric buds occur on one side and induce
upper and lower renal moieties. If a single bud divides At about the same time that the ureteric buds appear
close to its origin, the result is an incomplete duplex kidney (28 days), the partitioning of the cloaca commences. An
with a common distal ureter (Fig. 1-8). If two separate buds ingrowth of mesoderm from the point of confluence of
form, the kidney is drained by two separate ureters. As it allantois and hindgut forms an advancing septum, which
reaches the urogenital sinus, the lower ureter migrates lat- progresses toward the cloacal membrane (see Fig. 1-3). This,
erally and crosses the upper ureter (the Weigert-Meyer law) aided by the ingrowth of lateral or Rathke folds on either
(see Fig. 1-8). The lower moiety of the kidney is therefore side, divides the cloaca into an anterior primitive urogenital
more prone to reflux. The upper ureter, because it arrives at sinus, which receives the mesonephric ducts, and a poste-
the urogenital sinus later, retains a closer association with rior rectum (see Fig. 1-4). This division is complete when the
the mesonephric duct opening and is prone to ectopia. The advancing edge of the urorectal septum reaches the cloacal
mechanism of ureterocele formation is unclear, but it may membrane during the 6th week, dividing it into an anterior
result from failure of involution of the Chwalla membrane urogenital and a posterior anal membrane. The urogenital
(Fig. 1-9). membrane breaks down during the 7th week, establishing
continuity between the developing urinary tract and the
amniotic cavity.
Anomalies of Renal Fusion, Position, and Rotation
The upper part of the primitive urogenital sinus between
the allantois and the mesonephric ducts is called the vesico-
Ectopic Kidneys
urethral canal; it will form the definitive bladder. Growth of
Kidneys that fail to ascend from the pelvis may also fail to the anterior abdominal wall between the allantois and the
rotate and have an anomalous blood supply derived from the urogenital membrane is accompanied by an increase in size
aorta or pelvic vessels. A common example of this is the pelvic and capacity of this bladder precursor. The allantois remains
kidney (Fig. 1-10). attached to the apex of the fetal bladder and extends into the
umbilical root; it loses its patency and persists as the median
umbilical ligament, otherwise known as the urachal remnant.
Fused Kidneys
By the 13th week, the interlacing circular and longitudinal
If the two kidneys come together during their development strands of the smooth muscle of the trigone are discernible.
in the pelvis, they may fuse. Most commonly, this results By 16 weeks, these are refined into discrete inner and outer
in a horseshoe kidney, in which fusion usually takes place longitudinal layers and a middle circular layer; at this time,
between the lower poles (Fig. 1-11). Occurring in 1 in 500 continence may be possible. The definitive urothelium is vis-
members of the population, this anomaly is usually asymp- ible by 21 weeks’ gestation.
tomatic and is characterized by malrotated calyces seen at During the process of cloacal septation, the mesonephric
urography. ducts distal to the ureteric bud origins (otherwise known as
 chapter 1: Embryology of the Urogenital Tract 

Mesonephric duct

Ureteric bud

Urogenital sinus

Metanephric blastema

Kidney
Urogenital sinus

Figure 1-9 Intravenous urogram showing a right duplex system in a

Vas girl. The ureter from the poorly functioning upper pole drains ectopi-
cally into a ureterocele that is seen as a filling defect in the bladder.
Figure 1-8 The embryogenesis of ureteric duplication. Partial du-
plication occurs when the ureteric bud divides after its origin to
form two collecting systems with ureters that join above the blad- middle of the 8th week, this Chwalla membrane disappears.
der to terminate in a single orifice (top left). If two separate buds
The muscularization of the ureter is probably induced by
arise, complete duplication is the result (top right). As the bottom
three images illustrate, the two ureteric origins are incorporated into drainage of the first secreted urine at about the 9th week of
the urogenital sinus at different times, so that they cross, causing the development. At 18 weeks of gestation, intrinsic narrowings
lower pole ureter to lie above and lateral to the upper pole ureter can be discerned at the ureteropelvic junction and the ure-
(the Weigert-Meyer law). terovesical junction.
The distal part of the primitive urogenital sinus will form
the definitive urogenital sinus. In females, this gives rise to
the common excretory ducts) widen and become incorpo- the entire urethra and the vestibule of the vagina. In males, it
rated into the posterior aspect of the primitive urogenital gives rise to the posterior urethra, and the anterior urethra is
sinus (see Fig. 1-4). As this continues, the posterior wall of formed from the closure of the urethral folds.
the canal widens. The ureteric orifices arrive on the surface
of this posterior wall early and become separated from the Anomalies of the Urogenital Membrane
mesonephric duct orifices. As further incorporation of the
lower mesonephric ducts occurs, the ureteric orifices move
and Cloacal Partitioning
superolaterally relative to the mesonephric duct orifices
(see Fig. 1-5). These stay close to the midline and descend
Exstrophy
into the developing posterior urethra. The epithelia of The exact embryologic origin of bladder exstrophy is unclear,
both ducts fuse in the midline, and the triangular area but it is thought to be failure of growth of the lower abdominal
between them and the ureteric orifices defines the trigone wall, between the allantois and the urogenital membrane, cou-
(see Fig. 1-5). pled with breakdown of the urogenital membrane. This leaves
During the ascent of the kidneys from the pelvis, the ure- a small, open bladder plate, a low-placed umbilical root, and
ters rapidly elongate. Initially, their lumen is not apparent, diastasis of the pubic bones. The genital tubercle is probably
but it develops cranially and caudally from the midpoint placed lower in these patients so the cloacal membrane rup-
until early in the 8th week, when only a membrane persists tures above it, leading to a penis with an open dorsal surface
between the lower ureter and the urogenital canal. By the that is continuous with the bladder plate.
  part I: Basics

Figure 1-10 Cystogram demonstrating a pelvic kidney with a ureter

that refluxes. Note the malrotated appearance of the calyces.

Figure 1-11 Intravenous urogram of a horseshoe kidney. The collect-

Cloacal Exstrophy ing system is malrotated, and the calyces face forward, giving a classic
appearance. The lower poles are joined across the midline.
If the septum and Rathke folds also fail to partition the clo-
aca, the bladder plate is separated into two halves by a cen-
tral hindgut plate. In this most severe variant of exstrophy,
the phallus is often divided in two halves that may be widely incompetent bladder outlets and poor capacity. Even after
separated. reconstructive surgery, the prognosis for urethral continence
is very poor (Fig.1-12).
Cloacal Anomalies
Incomplete septation of the cloaca leads to continued com- DEVELOPMENT OF THE INTERNAL GENITALIA
munication between the rectum and the urogenital sinus. The
urethra, vagina, and rectum join into a common distal chan- The gender of the individual has already been decided at
nel. The perineum is characterized by an imperforate anus fertilization, but during early genital development there is
and a single anterior opening. little discernible difference between the male and the female
embryo. As stated previously, the germ cells arise from the
yolk sac and migrate to the intermediate mesoderm. Here,
Anomalies of the Trigone they contribute to the genital ridges, which arise during the
5th week of gestation, anteromedial to the mesonephros (see
Renal Agenesis Fig. 1-3). Interaction between the germ cells and the surround-
If the ureteric bud fails to develop on one side, the ipsilateral ing tissue creates primitive sex cords within the developing
trigone also fails to develop, giving a characteristic endoscopic gonad, which is divided into an outer cortical layer and an
appearance. This is not usually associated with any continence inner medulla. At the same time, paramesonephric or mülle-
problems. rian ducts appear lateral to the mesonephric ducts and parallel
to them in their upper course. As they approach the primitive
urogenital sinus, these ducts converge and fuse in the midline,
Bilateral Single Ectopic Ureters forming an elevation in its posterior wall called the müllerian
If both ureters maintain their relationship with the mesoneph- tubercle (see Fig. 1-3).
ric duct, there is little separation between them, and the tri- From the 6th week, these early structures develop diver-
gone fails to develop. Patients who have this anomaly have gently in a male or a female direction. Ovaries arise ­principally