Hirschsprung's disease is one of the most important and most fascinating diseases in paediatric surgery. Our understanding of Hirschsprung's disease is developing rapidly, not only in relation to its pathophysiology and the development of new surgical techniques, but especially in relation to new genetic findings. A first comprehensive description of the pathophysiology, clinical symptoms, diagnosis and therapy of Hirschsprung's disease was outlined in 1970 by Theodor Ehrenpreis, Professor of Pediatric Surgery at the Karolinska Institute, Stockholm, Sweden, in a booklet entitled "Hirschsprung's Disease". The booklet of 176 pages was dedicated to Harald Hirschsprung (1830-1916) of Copenhagen, Denmark, and to Ovar Swenson of Chicago, Illinois, USA, the two pioneers in the study of Hirschsprung's disease. Harald Hirschsprung was a paediatrician, and Ovar Swenson a paediatric surgeon, who performed the first successful resection of an aganglionic bowel segment. That first book, published by Yearbook Medical Publishers, mainly discussed questions of postoperative continence based on the results of a large series of patients treated successfully at the Karolinska Institute.
In 1978 Ehrenpreis permitted one of the editors of the present edition to prepare an update of his internationally recognized book. Therefore, in 1982, a new book on Hirschsprung's disease by Alexander Holschneider was published by Hippokrates (Thieme-Stratton) with a foreword by Th. Ehrenpreis. It was a multiauthored textbook with particular prominence given to the results of an international clinical research study of the postoperative results in Hirschsprung's disease, undertaken from 1976 to 1978 by the author himself and a technical assistant, with special regard to the underlying surgical techniques. The follow-up studies were performed with the help of the Volkswagen Foundation in 16 paediatric surgical departments in Europe and the United States over a period of 3 years. The most interesting and unique aspect of this study was the fact that all clinical and electromanometri-cal investigations were performed by the same research team, independent of the staff of the individual hospital. As a result of this study concept, a most objective com parison of the results of Swenson's, Soave's, Duhamel's and Rehbein's techniques was achieved.
However, as our understanding of Hirschsprung's disease and associated motility disorders of the gut increased, a second edition of this book was published in 2000, this time by Harwood Academic Publishers, part of the Gordon and Breach Publishing Group. The title of this new book was changed to "Hirschsprung's Disease and Allied Disorders", because we included other enteric plexus disorders and smooth muscle disorders of the gut. The editors of this again multiauthored edition were Alexander Holschneider and Prem Puri. The book was divided into three parts: Physiology and Pathophysiology, Clinical Aspects, and Treatment and Results. As well as discussion of normal colonic motor function and the pathophysiology of classical Hirschsprung's disease, the book included special chapters on the development of the enteric nervous system, the functional anatomy of the enteric nervous system, animal models of aganglionosis, the molecular genetics of Hirschsprung's disease and the RET protein in human fetal development and in Hirschsprung's disease. New areas of special interest included intestinal neuronal dysplasia, particular forms of intestinal neuronal malformations, enterocolitis, megacystis-microcolon-intestinal hypoperistalsis syndrome, degenerative hollow visceral myopathy mimicking Hirschsprung's disease, and newer diagnostic techniques such as special neuronal markers, electron microscopy and anal sphincter achalasia. This second edition was the most comprehensive book ever published on Hirschsprung's disease and allied disorders.
With the passage of time, our understanding of enteric plexus disorders has exploded. Ehrenpreis in his preface of 1970 cited the President of the Swedish Nobel Prize Committee who stated that there are more scientists living today than during all past centuries. After having reviewed the recent literature on Hirschsprung's disease and allied disorders we are convinced that this is even more relevant today. Therefore, a new edition of Hirschsprung's disease and allied disorders was realized with the help of Springer. The previous chapters
"Clinical Generalities of Hirschsprung's Disease", "Disorders and Congenital Malformations associated with Hirschsprung's Disease", "Megacystis-Microcolon-Intes-tinal Hypoperistalsis Syndrome", "Degenerative Hollow Visceral Myopathy Mimicking Hirschsprung's Disease" and "Diagnosis of Hirschsprung's Disease and Allied Disorders" have been updated. A new separate chapter on "NAPDH-Diaphorase Histochemistry" has been introduced in the part "Diagnosis", next to the updated chapters "Histopathological Diagnosis and Differential Diagnosis of Hirschsprung's Disease", "Immunohistochemical Studies" and "Electron Microscopic Studies of Hirschsprung's Disease". For reasons of clarity, previously separated chapters such as the former chapters 5 and 6 "Molecular Genetics of Hirschsprung's Disease" and "Ret-Protein in Human Foetal Development and in Hirschsprung's Disease" have been brought together and concentrated in a new chapter. Chapter 3 "Functional Anatomy of the Enteric Nervous System" by M.D. Gershon and chapter 6 "Normal Colonic Motor Function and Relevant Structure" by J. Christensen have been reproduced. Chapter 12 "Particular Forms of Intestinal Neuronal Malformations" and chapter 14 "Megacolon in Adults" have become part of the new chapter 8 "Hirschsprung's Disease: Clinical Features" and chapter 18 "Neurocristopathies and Particular Associations with Hirschsprung's Disease". Chapter 17 "Intestinal Obstructions Mimicking Hirschsprung's Disease" has become chapter 21 "Adynamic Bowel Syndrome".
The chapters referring to the different surgical techniques have been updated too, but the concept of the previous editions, to compare the detailed description of one of the pioneer surgeons with the experience of a second author with the same technique, was given up. In the third edition of the book both parts of each chapter dealing with a specific surgical technique have been brought together to create new contributions for each of the different surgical approaches. The chapter "Laparoscopically Assisted Anorectal Pull-through" has been updated and a new chapter "Transanal Pull-through for Hirschsprung's Disease" has been introduced. Finally, the previous chapters dealing with early and late complications have also been brought together and the contribution of Teitel-baum and Coran on long-term results and quality of life has been updated.
The new edition is again a multiauthored book, and we have to thank all the internationally well-known authors and coauthors for their excellent and sophisticated contributions. It is their interest, help and effort that has again made possible the drawing together in one volume of the collective wisdom of many of the leading experts in Hirschsprung's disease and related disorders. Their contributions to this volume again provide a step forward in the elucidation of the genetic basis, and the correct diagnosis and treatment of this interesting disease and its allied disorders.
Besides the authors and coauthors, we would like to thank Mrs. Elisabeth Herschel of the Children's Hospital of Cologne, and the Children's Medical and Research Foundation, Our Lady's Children's Hospital, Dublin, for their support. Finally, we wish to thank the editorial staff of Springer, Heidelberg, Germany, particularly Ms. Gabriele Schroeder, for their interest and encouragement to publish a third edition of this book on a most important subject in paediatric surgery.
Alexander M. Holschneider Prem Puri
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