Hirschsprung's Disease. Diagnosis and Treatment

Editors: 

Ramón Núñez Núñez (Servicio de Cirugía Pediátrica, Hospital Materno Infantil, Complejo Hospitalario Universitario, Badajoz, Spain), Manuel López-Alonso (Unidad de Manometría, Hospital Universitario Infantil Virgen del Rocío, Spain)

Book Description: 

This new book on Hirschsprung's disease (HD) contains a complete update on the various aspects of this disease. It is the collaboration of an international group of pædiatric surgeons well-known in their fields who are experts in this pathology and of other prestigious professionals – neurobiologists, physiologists, geneticists, pædiatricians, and histopathologists. Over the course of 28 chapters, these authors describe the latest advances in the diagnosis and treatment of this pathology. The first part is an update of the anorectal physiology, pathophysiology, genetics, and histopathology. The second part deals with the clinical manifestations and diagnosis in the newborn, with especial reference to manometric diagnosis during the neonatal period and to the morphological and radiological study. Other important chapters are devoted to differential diagnosis, preoperative care, indications for ostomy, and preparing the child for the pull-through. The third part describes the various classical surgical options (Swenson, Duhamel, Soave, Rehbein), and the new methods using laparoscopy and transanal pull-through, which have been the major breakthrough in the treatment of this condition. Total colonic aganglionosis and the critical review of the different surgical techniques occupy two magisterial chapters. The fourth part comprises an extensive exposition of enterocolitis in HD, the long-term results following surgery, other variants of HD, and two novel chapters in treatises on this disease (intestinal transplants and nutrition). In the Annex, parents of children with this disease describe their experience. The book ends with a final chapter devoted to informed consent. This book will be an invaluable reference for pædiatric surgeons and pædiatricians and gastroenterologists, and has a special interest for parents of children with Hirschsprung's disease.

Table of Contents: 

PART I: History, Development of the Enteric Nervous System, Physiology, Pathophysiology, Epidemiology, Classification, Genetics, and Histopathology of Hirschsprung's Disease;pp. 1 

• Chapter 1. Hirschsprung's Disease: Historical Review;pp. 3-14 
  (José Boix-Ochoa and Pilar Abad Calvo) 
• Chapter 2. Biological Development of the Enteric Nervous System;pp. 15-44 
  (Marie A. Breau and Sylvie Dufour) 
• Chapter 3. Anorectal Physiology;pp. 45-53 
  (Juan Ribas ) 
• Chapter 4. Pathophysiology of Hirschsprung's Disease;pp. 55-71 
  (Prem Puri and Anne-Marie O’Donnell) 
• Chapter 5. Epidemiology. Classification. Congenital Defects 
  Associated with Hirschsprung's Disease;pp. 73-85 
  (Emilio Blesa, Enrique Galán and Juan J. Cardesa) 
• Chapter 6. Genetics and Hirschsprung's Disease;pp. 87-105 
  (Enrique Galán, Emilio Blesa and Juan J. Cardesa) 
• Chapter 7. Histopathology of Hirschsprung's Disease;pp. 107-121 
  (William Meier-Ruge and Elizabeth Bruder) 

PART II: Hirschsprung's Disease: Clinical Features, Manometric Diagnosis, Radiological Diagnosis, Morphological Diagnosis, Differential Diagnosis, Medical Care, Enterostomies, and Bowel Preparation for Pull-Through;pp. 123 

• Chapter 8. Hirschsprung's Disease: Clinical Features;pp. 125-136 
  (Ramón Núñez, Emilio Blesa and Rodolfo Cabrera) 
• Chapter 9. Manometric Diagnosis of Hirschsprung’s Disease during the Neonatal Period;pp. 137-158 
  (Manuel López-Alonso) 
• Chapter 10. Radiological Diagnosis of Hirschsprung’s Disease 
  during the Neonatal Period;pp. 159-168 
  (Manuel López-Alonso and J. Fernando Prieto) 
• Chapter 11. Morphological Diagnosis of Hirschsprung's Disease: 
  Rectal Suction Biopsy;pp. 169-181 
  (Dolores I. Segura and Manuel López-Alonso) 
• Chapter 12. Differential Diagnosis of Hirschsprung’s 
  Disease in the Neonatal Period;pp. 183-196 
  (José Mª Gil-Vernet, Marta De Diego and Jesús Broto) 
• Chapter 13. Preoperative Medical Care. Enterostomies. Bowel 
  Preparation for Pull-Through;pp. 197-218 
  (Ramón Núñez, Isabel Vargas and Mª José Estepa) 

PART III: Hirschsprung's Disease: Classical Surgical Treatment (Swenson, Duhamel, Soave, and Rehbein), Laparoscopy in Hirschsprung's Disease, Exclusive Transanal Pull-Through, Total Colonic Aganglionosis, A Critical Review of the Different Operations for Hirschsprung's Disease;pp. 219 

• Chapter 14. Swenson's Technique;pp. 221-228 
  (Luis Lassaleta) 
• Chapter 15. Duhamel's Technique;pp. 229-241 
  (Piyawan Chiengkriwate) 
• Chapter 16. Soave's Technique;pp. 243-253 
  (Antonio Hernández-Orgaz and Manuel López-Alonso) 
• Chapter 17. Rehbein's Technique;pp. 255-260 
  (José Mª Gil-Vernet, Jesús Broto and Marta De Diego) 
• Chapter 18. Laparoscopic-Assisted Transanal Pull-Through for Hirschsprung’s Disease;pp. 261-274 
  (Atsuyuki Yamataka and Akihiro Shimotakahara) 
• Chapter 19. One-stage Transanal Endorectal Colectomy and Pull-Through for Hirschsprung's Disease in Neonates and Infants;pp. 275-287 
  (Luis De La Torre-Mondragón and J. Arturo Ortega Salgado) 
• Chapter 20. Total Colonic Hirschsprung's Disease;pp. 289-303 
  (Barbara E. Wildhaber, Daniel Teitelbaum and Arnold G. Coran) 
• Chapter 21. A Critical Review of the Different Operations for 
  Hirschsprung's Disease;pp. 305-316 
  (Juan A. Tovar) 

PART IV: Enterocolitis in Hirschsprung's Disease, Long-Term Results, Variant Hirschsprung's Disease, Intestinal Transplant, and Nutrition in Hirschsprung's Disease;pp. 317 

• Chapter 22. Enterocolitis in Hirschsprung's Disease;pp. 319-348 
  (Ramón Núñez, Ana Torres and J.Ignacio Santamaría) 
• Chapter 23. Long-Term Results after Treatment of Hirschsprung's 
  Disease;pp. 349-371 
  (Scott A. Engum and Jay L.Grosfeld) 
• Chapter 24. Variant Hirschsprung’s Disease;pp. 373-384 
  (Prem Puri) 
• Chapter 25. Intestinal Transplant in Hirschsprung's Disease and Intestinal Pseudo-Obstruction;pp. 385-411 
  (Manuel López Santamaría, and Francisco Hernández Oliveros) 
• Chapter 26. Nutritional Status,situations of Nutrition in Hirschsprung's Disease;pp. 413-428 
  (Emilio Blesa and Luis Lassaletta) 
• ANNEX: Hirschsprung's Disease: The Parents' Experience 
  and Informed Consent for Hirschsprung's Disease;pp. 429 
• Chapter 27. Hirschsprung's Disease: The Parents' Experience;pp. 431-442 
  (Sheyla Leal, M. Carmen Vicente and Ángeles Del Hoyo) 
• Chapter 28. Informed Consent for Hirschsprung's Disease;pp. 443-462 
  (M. Ángeles López de la Torre) 

Index