Endocrine Surgery in Children (eBook)

Daniel J. Ledbetter, MD, FACS, FAAPProfessor of SurgeryUniversity of WashingtonAttending SurgeonSeattle Children’s HospitalSeattle, Washington, USAPaul R. V. Johnson, MBChB, MD, FRCS(Eng), FRCS (Edin), FRCS (Paed.Surg), FAAPProfessor of Pediatric SurgeryUniversity of OxfordDirector of the Oxford Islet Transplant ProgrammeAcademic Pediatric Surgery UnitNuffield Department of Surgical SciencesJohn Radcliffe HospitalOxford, UK 

Endocrine Surgery in Children

 

 

Daniel J. Ledbetter, MD, FACS, FAAP

Professor of Surgery

University of Washington

Attending Surgeon

Seattle Children’s Hospital

Seattle, Washington, USA

 

Paul R. V. Johnson, MBChB, MD, FRCS(Eng), FRCS (Edin), FRCS (Paed.Surg), FAAP

Professor of Pediatric Surgery

University of Oxford

Director of the Oxford Islet Transplant Programme

Academic Pediatric Surgery Unit

Nuffield Department of Surgical Sciences

John Radcliffe Hospital

Oxford, UK

 

 

 

Foreword

 

Endocrine conditions requiring surgery in children are extremely rare. Surgeons undertaking this surgery need to be specifically trained and exposed to a large volume of cases in order to maintain their expertise. Therefore it is clear that the surgery needs to be concentrated in designated regional centres and carried out by a select number of paediatric surgeons. In addition, a close working relationship with the paediatric endocrinologists is essential for the overall wellbeing of the child.

While management of many of these conditions must remain within the armamentarium of the paediatric surgeon, for example neuroblastoma, hyperinsulinism, adrenal tumours and gonadal conditions, there is a tendency to engage adult endocrine surgeons with specific expertise in a particular organ, such as thyroid, parathyroid, pituitary, to perform the procedures in conjunction with the paediatric surgeons. In the latter situation, it is important that the overall care of the child should remain firmly in the province of the paediatric specialist.

This book devoted to the surgery of endocrine disorders in children fills a major gap in the paediatric surgical literature and brings together the full range of endocrine conditions encountered in the paediatric age range. The last publication devote to the surgery of endocrine disorders in children was part of the Progress in Pediatric Surgery series (now discontinued) published in 1991.

This publication includes contributions from international authorities in paediatric surgery and endocrinology, mainly from the United Kingdom and North America and should be viewed as the standard text for many years to come.

 

Lewis Spitz MD, PhD, FRCS(Edin, Eng, I), FRCHCP, FAAP, FCS(SA), FACS

Emeritus Nuffield Professor of Paediatric Surgery

Institute of Child Health, University College, London

Hon. Consultant Paediatric Surgeon

Great Ormond Street Hospital NHS Trust

London, UK

 

 

Preface

 

Endocrine surgical conditions during childhood are relatively rare. However, they represent an interesting and challenging group of conditions that all Pediatric Surgeons will encounter sometime during their careers. This book is one of the first to provide a specific overview of the range of different endocrine surgical conditions encountered in children, together with their management.  It is aimed at any surgeon operating on endocrine conditions in children and adolescents.

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The book is divided into sections based on the different endocrine organs. Each section begins with a chapter outlining the embryology, anatomy, and physiology of that organ, before subsequent chapters address the different surgical conditions that occur, together with their diagnosis, management, and outcomes. Basic science and ‘state of the art’ research perspectives are included as they relate to surgical decision-making and optimal clinical care. We have intentionally chosen a diverse group of authors who have experience and expertise in caring for children with endocrine surgical conditions in North America and in the UK. The authors include endocrinologists, adult endocrine surgeons, and paediatric surgeons, and represent many who are at the forefront of both clinical care and cutting edge research. One of the key messages that comes out throughout the book is that rare endocrine surgical conditions require a collaborative multi-disciplinary team approach to ensure that the children receive the very best management resulting in the most favourable outcomes.

 

It is our hope that this international, multidisciplinary perspective will give surgeons caring for children with endocrine conditions requiring surgery, additional insights that will lead to a better understanding of the conditions and ultimately improved patient care.

 

Dan Ledbetter and Paul Johnson

 

 

 

 

Dedication

 

To Sunny, Kelly, and Brian – the greatest joys of my life.

DJL

 

To Hilary, Thomas, and Tilly – for your unconditional love, support, and patience during the preparation of this book.

PRVJ

 

 

Acknowledgments

 

We want to thank the many people who have directly and indirectly contributed to this book. First, we would like to thank our mentors who stimulated our interests in pediatric endocrine surgery. These include the late David Tapper and the late Nick Dudley, both of whom demonstrated excellence in the operative care of children. Next, Diana Farmer, who persuaded the American College of Surgeons that a panel discussion of endocrine problems in children deserved a place on the program of its annual Clinical Congress. That panel discussion was the primary inspiration for this book. 

 

Next, we would like to thank Springer, who supported this book through its long gestation to publication. In particular we would like to thank Margaret Burns who guided the book throughout its development to the point of completion.

We would also like to thank all the contributing authors. They have shown remarkable patience throughout the production of the book and without them this book would clearly not have been possible.

 

We would like to thank all our excellent clinical colleagues in Anesthesia, Surgery, Pathology, Radiology, Endocrinology, and Oncology at both Seattle Children’s Hospital and the Children’s Hospital in Oxford who have helped care for the many and varied patients with endocrine problems who have needed surgery. This includes pediatric surgical colleagues in the Division of Pediatric General and Thoracic Surgery in Seattle (Robert Sawin, John Waldhausen, Pat Healey, Adam Goldin, Ken Gow, John Meehan, George Drugas, Jeff Avansino, Patrick Javid, and Kim Riehle) and in the Department of Paediatric Surgery , Endocrinology, and Endocrine Surgery in Oxford (Hugh Grant, Kokila Lakhoo, Silke Wagener, Alex Lee, Ian Willetts, Rosa Romero, Radu Mihai, Fiona Ryan, and Taffy Makayer). Finally, we would like to thank all the surgical trainees including residents, fellows, core trainees, and registrars who do much of the work in the hospital, but more importantly, ask many of the most important questions that inspire everyone to discover new and better ways to care for children.

 

 

 

Foreword

Preface

Dedication

Acknowledgments

Contributors

I. Thyroid

1. Thyroid Gland Embryology, Anatomy, and Physiology

Gerard Walls and Radu Mihai

2. Hyperthyroidism

Betty Panayota Messazos, Radu Mihai, and Fiona Jane Ryan

3. Thyroid Nodules in Children

Geoffrey K. Blair and Daniel J. Ledbetter

4. Thyroid Cancer in Children

Daniel J. Ledbetter

II. Parathyroid

5. Parathyroid Gland Embryology, Anatomy, and Physiology

Gerard Walls and Radu Mihai

6. Parathyroid Surgery in Children

Jocelyn Burke, Herbert Chen, and Ankush Gosain

III. Adrenal

7. Adrenal Gland Embryology, Anatomy, and Physiology

Sanjeev Vasudevan and Mary L. Brandt

8. Phaeochromocytoma

Michael J. Stechman and Gregory P. Sadler

9. Neuroblastoma

Keith Holmes

10. The Evaluation and Management of Adrenal Masses and Adrenocortical Tumors

Kenneth W. Gow

IV. Pancreas

11. Pancreas Embryology, Anatomy, and Physiology

Yousef El-Gohary and George K. Gittes

12. Congenital Hyperinsulinism

Khalid Hussain, Shireen A. Nah, and Agostino Pierro

13. Pancreatic Neuroendocrine Tumors

Paul R. V. Johnson

14. Islet Cell Transplantation

Antonio Bruni, Michael McCall, and A. M. James Shapiro

15. Diabetes in the Pediatric Surgical Patient

Ari J. Wassner and Michael S. D. Agus

V. Bariatric Surgery

16. Assessing and Selecting Patients for Bariatric Surgery

Thomas H. Inge

17. Bariatric Surgery in Children

Mansoor Ali Khan and Roger Ackroyd

VI. Ovary, Testicles, and Fertility

18. Pathogenesis and Treatment of Disorders of Sexual Development

Rafael V. Pieretti

19. Testis Embryology, Anatomy, and Physiology

John Hutson

20. Surgical Considerations of Testicular Maldescent

John Hutson

21. Testicular Torsion

Daniel W. Colliver and David F. M. Thomas

22. Testicular Tumors in Children

Yaser El-Hout and Darius J. Bägli

23. Ovarian Embryology, Anatomy, and Physiology Including Normal Menstrual Physiology

Nancy Sokkary and Jennifer E. Dietrich

24. Ovarian Tumors in Children

David F. M. Thomas

25. Ovarian Torsion

Anne C. Fischer

26. Fertility Preservation

Jennia Michaeli, Stephen Kennedy, and Ariel Revel

VII. Breast

27. Breast Embryology, Anatomy, and Physiology

Monica E. Lopez and Oluyinka O. Olutoye

28. Paediatric and Adolescent Breast Disorders Clare M. Rees, Kokila Lakhoo, and P. G. Roy

29. Gynecomastia

Tim Goodacre and Gavin McCoubrey

VIII. Miscellaneous

30. Multiple Endocrine Neoplasia Type 1 and Type 4

Gerard V. Walls

31. Multiple Endocrine Neoplasia Type 2

Eduardo A. Perez and Michael Skinner

IX. Pituitary

32. Pituitary Gland Embyology, Anatomy, and Physiology

Vaitsa Tziaferi and Mehul T. Dattani

33. Surgical Considerations of the Pituitary

Toba Niazi and Samuel R. Browd

 

 

 

 

Contributors

 

Roger Ackroyd, MBChB, MD(Distinction), FRCS(Eng), FRCS(Ed), FRCS(Gen Surg)

Department of General Surgery, Northern General Hospital, Sheffield, South Yorkshire, UK

 

Michael S. D. Agus, MD

Department of Pediatrics, Boston Children’s Hospital, Harvard Medical School, Boston, MA  USA

 

Darius J. Bägli, MDCM, FRCSC, FAAP, FACS

Department of Surgery, Division of Urology, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada

 

Geoffrey K. Blair, MD, FRCSC

Division of Pediatric Surgery, British Columbia’s Children’s Hospital, Vancouver, British Columbia, Canada

 

Mary L. Brandt, MD

Division of Pediatric Surgery, Department of Surgery, Baylor College of Medicine, Texas Children’s Hospital, Houston, TX, USA

 

Samuel R. Browd, MD, PhD, Facs, Faans, Faap

Department of Neurological Surgery, University of Washington, Seattle, WA, USA

 

Antonio Bruni, BSc

Li Ka Shing Centre for Health Research Innovation, Edmonton, Alberta, Canada

 

Jocelyn Burke, MD

Department of Surgery, University of Wisconsin School of Medicine and Public Health, Madison, WI, USA

 

Herbert Chen, MD

Department of Surgery, Division of General Surgery, University of Wisconsin School of Medicine and Public Health, Madison, WI, USA

 

Daniel W. Colliver, FRCS

Department of Paediatric Surgery and Urology, Queens Medical Centre, Nottingham, UK

 

Mehul T. Dattani, MBBS, MD, FRCPCH, FRCP

Developmental Endocrinology Research Group, Clinical and Molecular Genetics Unit, UCL Institute of Child Health and Great Ormond Street Hospital for Children, London, UK

 

Jennifer E. Dietrich, MD, MSc

Department of Pediatric and Adolescent Gynecology, Texas Children’s Hospital, Houston, TX, USA

 

Yousef El-Gohary, MD, MRCS (Glasg)

Department of Surgery, Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA

 

Yaser El-Hout, MD

Division of Urology, Department of Surgery, American University of Beirut Medical Center, Beirut, Lebanon

 

Anne C. Fischer, MD, PhD

Department of Pediatric Surgery, Tenet Florida Physician Services, West Palm Beach, FL, USA 

 

George K. Gittes, MD

Department of Pediatric Surgery and Surgery, Children's Hospital of Pittsburgh; Division of Pediatric Surgery, University of Pittsburgh, School of Medicine, Children's Hospital of Pittsburgh, Pittsburgh, PA, USA

 

Tim Goodacre, FRCS, BSc, MB, BS 

Nuffield Department of Surgery, University of Oxford, Oxford, UK

 

Ankush Gosain, MD, PhD

Department of Surgery, Division of Pediatric Surgery, University of Wisconsin School of Medicine and Public Health, Madison, WI, USA

 

Kenneth W. Gow, MD

Department of General and Thoracic Surgery, Seattle Children’s Hospital and the University of Washington, Seattle, WA, USA

 

Keith Holmes, ChM, FRCS, DCH

Department of Paediatric Surgery, St. George’s Hosptial, London, UK  

 

Khalid Hussain, MBChB, MRCP, MSc

Developmental Endocrinology Research Group, Clinical and Molecular Genetics Unit, Institute of Child Health/Great Ormond Street Hospital for Children, University College London, London, UK

 

John Hutson, MD

Department of Paediatrics, University of Melbourne; Department of Urology, Royal Children’s Hospital, Melbourne; Douglas Stephens Surgical Research Laboratory, Murdoch Childrens Research Institute, Melbourne Royal Children’s Hospital, Parkville, Victoria, Australia

Thomas H. Inge, MD, PhD, FACS, FAAP

Department of Surgery, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, USA

 

Paul R. V. Johnson, MBChB, MD, FRCS(Eng), FRCS (Edin), FRCS (Paed.Surg), FAAP

Department of Pediatric Surgery, University of Oxford; Oxford Islet Transplant Programme, Academic Pediatric Surgery Unit, Nuffield Department of Surgical Sciences, John Radcliffe Hospital, Oxford, UK

 

Stephen Kennedy, MA (Oxon), MD, MRCOG

Nuffield Department of Obstetrics and Gynaecology, University of Oxford, John Radcliffe Hospital, Oxford, UK

 

Mansoor

General Military Royal Centre for Defence Medicine; Department of Surgery and Trauma, Imperial College London. London, UK

 

Kokila Lakhoo, PhD, FRCS(Eng & Edin), FCS(SA), MRCPCH(UK), MBChB

Department of Paediatric Surgery, Children’s Hospital Oxford, Oxford Radcliffe Hospitals NHS Trust, Oxford, UK

 

Monica E. Lopez, MD

Department of Pediatric Surgery, Texas Children’s Hospital, Houston, TX

 

Daniel J. Ledbetter, MD, FACS, FAAP

Department of Surgery, University of Washington; Department of Surgery, Seattle Children’s Hospital, Seattle, WA, USA

 

Michael McCall, MD, PhD

Department of Surgery, Royal Alexandra Hospital, Edmonton, Alberta, Canada

 

Gavin McCoubrey

Department of Plastic Surgery, Salisbury NHS Foundation Trust, Salisbruy, Wiltshire, UK

 

Betty Panayota Messazos, MBBChir, MA, MRCPCH

Department of Paediatric Endocrinology, Oxford Children’s Hospital, Oxford University Hospitals NHS Trust, Oxford, UK

 

Jennia Michaeli, MD

Department of Obstetrics and Gynecology, Hadassah Medical Center and Hebrew University-Hadassah Medical School, Jerusalem, Israel

 

Radu Mihai, MD, PhD, FRCS

Department of Surgery, John Radcliffe Hospital, Oxford, UK

 

Shireen A. Nah, MBBS, MRCS, MS

The Surgery Unit, UCL Institute of Child Health, University College London and Great Ormond Street Hospital for Children, NHS Trust, London, UK

 

Toba Niazi, MD

Department of Clinical Neurological Surgery and Pediatrics, University of Miami, Miami, FL, USA

 

Oluyinka O. Olutoye, MD, PhD

Texas Children’s Fetal Center, Texas Children’s Hospital, Houston, TX, USA

 

Eduardo Perez, MD

Division of Pediatric Surgery, The Dewitt Daughtry Family Department of Surgery, University of Miami, Miller School of Medicine, Miami, FL, USA

 

Rafael V. Pieretti, MD

Department of Pediatric Surgery, MassGeneral Hospital for Children, Boston, MA, USA

 

Agostino Pierro, MD, FRCS (Eng), FRCS(Ed), FAAP

Department of Pediatric General and Thoracic Surgery, Hospital for Sick Children, Toronto, Ontario, Canada

 

Clare M. Rees, MD, MRCS, MBChB

Department of Paediatric Surgery, Children’s Hospital Oxford, Oxford Radcliffe Hospitals NHS Trust, Headington, UK

 

Ariel Revel, MD

Department of Obstetrics and Gynecology, Hadassah Medical Center and Hebrew University-Hadassah Medical School, Jerusalem, Israel

 

P. G. Roy, MBBS, MS (Gen Surg), MD, FRCS (Glas), FRCS (Gen Surg)

Department of Breast Surgery, Churchill Hospital, Oxford, UK

 

Fiona Jane Ryan, MD

Department of Paediatric Endocrinology, Oxford Children’s Hospital, Oxford, UK

 

Gregory P. Sadler, MD, FRCS

Department of Endocrine Surgery, John Radcliffe Hospital, Headley Way, Oxford, UK

 

A. M. James Shapiro, MD, PhD

Departments of Surgery, Medicine and Surgical Oncology, and Clinical Islet and Living Donor Liver Transplant Programs, University of Alberta,  

Michael A. Skinner, MD

Department of Pediatric Surgery, University of Texas Southwestern Medical Center; Children's Medical Center, Dallas, TX, USA

 

Nancy Sokkary, MD

Department of Pediatrics, UNM Children’s Hospital, The University of New Mexico, Albuquerque, NM, USA

 

Michael J. Stechman, MD, FRCS

Department of Endocrine Surgery, University Hospital of Wales, Heath Park, Cardiff, UK

 

David F. M. Thomas, FRCP, FRCPCH, FRCS

Department of Paediatric Urology, Leeds Teaching Hospitals and University of Leeds, Leeds, UK

 

Vaitsa Tziaferi, MD, MRCPCH, MSc

Department of Paediatric Endocrinology, Great Ormaond Street Hospital, London, UK

 

Sanjeev Vasudevan, MD

Department of Pediatric Surgery, Texas Children’s Hospital, Houston, TX, USA

 

Gerard Walls, MB, BS, MRCS

General and Endocrine Surgery, University Hospitals of Morecambe Bay, NHS Foundation Trust, Lancaster, UK

 

Ari J. Wassner, MD

Department of Pediatrics, Harvard Medical School, Boston Children’s Hospital, Boston, MA, USA