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Hirschsprung's disease and allied disorders. 2nd edition.
  1. MARK D STRINGER, Consultant Paediatric Surgeon
  1. Leeds Teaching Hospitals NHS Trust
  2. Leeds, UK

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    Hirschsprung's disease and allied disorders. 2nd edition. Edited by Holschneider AM, Puri P. (Pp 503, hardback, $125.00). Australia: Harwood Academic Publishers, 2000. ISBN 90 5702 263 X.

    In 1887, Harald Hirschsprung published the first detailed description of congenital megacolon, but it was not until 1948 that the true nature of the disease was defined. The discoveries at that time included the finding of aganglionosis in the rectum, the demonstration that peristaltic activity in the colon was propagated only as far as the narrowed aganglionic segment of bowel, and the relief of obstructive symptoms by rectosigmoidectomy. The key histological feature in Hirschsprung's disease is the absence of ganglion cells, which not only affects cholinergic neurons but also non-adrenergic, non-cholinergic neurons, which use nitric oxide as their chemical messenger. Aganglionosis is restricted to the rectum and sigmoid colon in 75% of patients, extends more proximally in 15–20%, and affects the entire colon and a variable length of ileum in 8%. Rarely, ganglion cells are absent from most of the gastrointestinal tract.

    This multiauthor text on Hirschsprung's disease contains contributions from an array of world experts. The editors, Alexander Holschneider and Prem Puri, are recognised authorities and they have compiled an invaluable reference source for paediatric surgeons, paediatricians, scientists, and others. The book progresses from discussions of the physiology and pathology of gut innervation, through clinical aspects of Hirschsprung's disease, to surgical techniques and their results and complications, and finally to long term outcomes. The style of the book is similar to the first edition published in 1982. Operative line diagrams and sketches are generally of good quality but all histology and operative photographs are black and white. There is inevitably some repetition of information, particularly in relation to results and complications, but this allows the reader to consider data from different perspectives. Clinical features of Hirschsprung's disease are only briefly discussed and there is little comment on the different methods of rectal biopsy and their complications. However, such deficiencies are minor and more than compensated by outstanding chapters on functional anatomy of the enteric nervous system (Michael Gershon), colonic motor function (James Christensen), and the pathophysiology of Hirschsprung's disease (Alois Scharli).

    Recent developments are discussed, including the obscure relationship between Hirschsprung's disease and intestinal neuronal dysplasia and innovations in surgical practice such as primary pull-through and laparoscopic techniques. Long term complications, principally constipation, enterocolitis, and faecal incontinence, are also reviewed. The latter has a highly variable incidence between different surgical units and is influenced by many factors including the timing and type of pull-through surgery, the extent of aganglionosis, methods of assessment and the age at which they are applied, and whether patients are evaluated with or without dietary modifications, oral medication, enemas and suppositories. Continence may improve during adolescence but techniques such as the antegrade continence enema offer hope for those with intractable soiling.

    This book also reviews recent breakthroughs in the molecular genetics of Hirschsprung's disease. At least three susceptibility genes have been identified: the RET proto-oncogene (chromosome 10), the endothelin-B receptor gene (chromosome 13) and the endothelin-3 gene (chromosome 20). It is estimated that RET mutations account for 50% of familial and 15–20% of sporadic cases of Hirschsprung's disease.

    For anyone interested in Hirschsprung's disease, this book provides the most comprehensive and authoritative source available.

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