Isolated hypoganglionosis: systematic review of a rare intestinal innervation defect.
Affiliation
National Children's Research Centre, Our Lady's Children's Hospital, Crumlin,, Dublin-12, Ireland.Issue Date
2012-02-01T10:25:30ZMeSH
Biopsy, NeedleChild, Preschool
Diagnosis, Differential
Female
Hirschsprung Disease/*diagnosis
Humans
Intestinal Diseases/diagnosis
Intestines/*innervation
Male
Myenteric Plexus/pathology
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Pediatr Surg Int. 2010 Nov;26(11):1111-5.Journal
Pediatric surgery internationalDOI
10.1007/s00383-010-2693-3PubMed ID
20721562Abstract
PURPOSE: Isolated hypoganglionosis (IH) is rare and resembles Hirschsprung's disease. The diagnosis by suction biopsy is difficult. A full-thickness biopsy is essential. Histological features of IH include sparse, small myenteric ganglia, low acetylcholinesterase activity in the lamina propria, and hypertrophy of muscularis mucosae and circular muscle. This review investigates the epidemiology, symptoms, diagnosis, and outcome of patients with IH. METHODS: A systematic review, using the keywords "isolated hypoganglionosis" and "intestinal hypoganglionosis" was performed. Publications were reviewed for epidemiology, histological methods, operative procedures, follow-up, and patient's outcome. RESULTS: Eleven publications from 1978 to 2009 reported 92 patients with IH (69 males and 23 females, age 4.85 years), presenting with constipation or enterocolitis. Diagnosis of IH was made by full-thickness biopsy and AChE staining in 91% of patients with additional staining in 82%; 54 patients had bowel resection and pull-through procedures; 11 patients had ileostomy or colostomy, and 2 had sphincter myectomy. Complications reported were enterocolitis, constipation, and residual disease. Seven patients (8%) died due to enterocolitis or short-bowel complications. CONCLUSION: This review confirms that diagnosis of IH is only possible by histochemical examination of a full-thickness biopsy. Despite advanced age at diagnosis of IH, epidemiological and clinical data are similar to aganglionosis.Language
engISSN
1437-9813 (Electronic)0179-0358 (Linking)
ae974a485f413a2113503eed53cd6c53
10.1007/s00383-010-2693-3
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