Isolated hypoganglionosis: systematic review of a rare intestinal innervation defect.

Hdl Handle:
http://hdl.handle.net/10147/207457
Title:
Isolated hypoganglionosis: systematic review of a rare intestinal innervation defect.
Authors:
Dingemann, Jens; Puri, Prem
Affiliation:
National Children's Research Centre, Our Lady's Children's Hospital, Crumlin,, Dublin-12, Ireland.
Citation:
Pediatr Surg Int. 2010 Nov;26(11):1111-5.
Journal:
Pediatric surgery international
Issue Date:
1-Feb-2012
URI:
http://hdl.handle.net/10147/207457
DOI:
10.1007/s00383-010-2693-3
PubMed ID:
20721562
Abstract:
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:
eng
MeSH:
Biopsy, Needle; Child, Preschool; Diagnosis, Differential; Female; Hirschsprung Disease/*diagnosis; Humans; Intestinal Diseases/diagnosis; Intestines/*innervation; Male; Myenteric Plexus/pathology
ISSN:
1437-9813 (Electronic); 0179-0358 (Linking)

Full metadata record

DC FieldValue Language
dc.contributor.authorDingemann, Jensen_GB
dc.contributor.authorPuri, Premen_GB
dc.date.accessioned2012-02-01T10:25:30Z-
dc.date.available2012-02-01T10:25:30Z-
dc.date.issued2012-02-01T10:25:30Z-
dc.identifier.citationPediatr Surg Int. 2010 Nov;26(11):1111-5.en_GB
dc.identifier.issn1437-9813 (Electronic)en_GB
dc.identifier.issn0179-0358 (Linking)en_GB
dc.identifier.pmid20721562en_GB
dc.identifier.doi10.1007/s00383-010-2693-3en_GB
dc.identifier.urihttp://hdl.handle.net/10147/207457-
dc.description.abstractPURPOSE: 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.en_GB
dc.language.isoengen_GB
dc.subject.meshBiopsy, Needleen_GB
dc.subject.meshChild, Preschoolen_GB
dc.subject.meshDiagnosis, Differentialen_GB
dc.subject.meshFemaleen_GB
dc.subject.meshHirschsprung Disease/*diagnosisen_GB
dc.subject.meshHumansen_GB
dc.subject.meshIntestinal Diseases/diagnosisen_GB
dc.subject.meshIntestines/*innervationen_GB
dc.subject.meshMaleen_GB
dc.subject.meshMyenteric Plexus/pathologyen_GB
dc.titleIsolated hypoganglionosis: systematic review of a rare intestinal innervation defect.en_GB
dc.contributor.departmentNational Children's Research Centre, Our Lady's Children's Hospital, Crumlin,, Dublin-12, Ireland.en_GB
dc.identifier.journalPediatric surgery internationalen_GB
dc.description.provinceLeinster-

Related articles on PubMed

All Items in Lenus, The Irish Health Repository are protected by copyright, with all rights reserved, unless otherwise indicated.