This book provides in-depth practical advice on how to manage children with endocrine conditions that may benefit from surgery. It is more detailed than general pediatric surgery texts and more surgically oriented than endocrinology texts. The first section is devoted to the thyroid and parathyroid, with detailed discussion of thyroid nodules, thyroid cancer, hyperthyroidism, hyperparathyroidism, and multiple endocrine neoplasia. The second section on the pancreas focuses on nesidioblastosis, islet cell transplantation, the surgical treatment of diabetes, and surgical complications of diabetes. Adrenal disorders are then discussed, followed by a section on the evaluation and management of ovarian and testicular torsion and tumors. The closing section addresses miscellaneous topics such as gynecomastia in boys and growth restriction surgery. This book will serve as an invaluable reference for all practitioners and trainees who care for children with endocrine problems for which surgery is considered.
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
<index
</index
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, <edmonton, alberta,="" canada=""
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
“This is an excellent review of problems in children which are either endocrine in origin, or which have significant endocrine implications. … The intended audience is primarily pediatric surgeons caring for these patients, but the book will be a valuable asset for adult endocrine surgeons who may help care for pediatric patients as well as for endocrinologists. It also will be a valuable resource for trainees in surgery, pediatrics, and endocrinology who will help in the care of these patients.” (Vincent R. Adolph, Doody’s Book Reviews, November, 2017)
This book provides in-depth practical advice on how to manage children with endocrine conditions that may benefit from surgery. It is more detailed than general pediatric surgery texts and more surgically oriented than endocrinology texts. The first section is devoted to the thyroid and parathyroid, with detailed discussion of thyroid nodules, thyroid cancer, hyperthyroidism, hyperparathyroidism, and multiple endocrine neoplasia. The second section on the pancreas focuses on nesidioblastosis, islet cell transplantation, the surgical treatment of diabetes, and surgical complications of diabetes. Adrenal disorders, including neuroblastoma, pheochromocytoma, and adrenal masses, are then discussed, followed by a section on the evaluation and management of ovarian and testicular torsion and tumors. The closing section addresses miscellaneous topics such as gynecomastia in boys and growth restriction surgery. This book will serve as an invaluable reference for all practitioners and trainees who care for children with endocrine problems for which surgery is considered.
Provides in-depth practical advice on how to manage children with endocrine conditions that may benefit from surgery