Skip segment Hirschsprung's disease: a systematic review.

Hdl Handle:
http://hdl.handle.net/10147/207464
Title:
Skip segment Hirschsprung's disease: a systematic review.
Authors:
O'Donnell, Anne-Marie; 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):1065-9.
Journal:
Pediatric surgery international
Issue Date:
1-Feb-2012
URI:
http://hdl.handle.net/10147/207464
DOI:
10.1007/s00383-010-2692-4
PubMed ID:
20714729
Abstract:
PURPOSE: Hirschsprung's disease is characterised by the congenital absence of ganglion cells beginning in the distal rectum and extending proximally for varying distances. 'Zonal aganglionosis' is a phenomenon involving a zone of aganglionosis occurring within normally innervated intestine. 'Skip segment' Hirschsprung's disease (SSHD) involves a 'skip area' of normally ganglionated intestine, surrounded proximally and distally by aganglionosis. While Hirschsprung's disease is believed to be the result of incomplete craniocaudal migration of neural crest-derived cells, the occurrence of SSHD has no clear embryological explanation. The aim of this study was to perform a systematic review of SSHD, reported in the literature between 1954 and 2009, in order to determine the clinical characteristics of this rare entity and its significance. METHODS: The first reported case of SSHD was published in 1954. A systematic review of SSHD cases in the literature, from 1954 to 2009, was carried out using the electronic database 'Pubmed'. Detailed information was recorded regarding the age, gender, presenting symptoms and location of the skip segment in each patient. RESULTS: 24 cases of SSHD have been reported in the literature to date. 18/24 (75%) of these cases were males and 6/24 (25%) were females. Of these, 22/24 (92%) were cases of total colonic aganglionosis (TCA), and 2/24 (8%) were rectosigmoid Hirschsprung's disease. Of the 22 TCA cases, 9 (41%) had a skip segment in the transverse colon, 6 (27%) in the ascending colon, 2 (9%) in the caecum and 5 (23%) had multiple skip segments. In both rectosigmoid Hirschsprung's disease cases, the skip segment was in the sigmoid colon. Overall, the length of the skip segment was variable, with the entire transverse colon ganglionated in some cases. CONCLUSION: SSHD occurs predominantly in patients with TCA. The existence of a skip area of normally innervated colon in TCA may influence surgical management, enabling surgeons to preserve and use the ganglionated skip area during pull-through operations.
Language:
eng
MeSH:
Colon/*innervation; Enteric Nervous System/*pathology; Female; Hirschsprung Disease/embryology/*pathology; Humans; Male
ISSN:
1437-9813 (Electronic); 0179-0358 (Linking)

Full metadata record

DC FieldValue Language
dc.contributor.authorO'Donnell, Anne-Marieen_GB
dc.contributor.authorPuri, Premen_GB
dc.date.accessioned2012-02-01T10:25:40Z-
dc.date.available2012-02-01T10:25:40Z-
dc.date.issued2012-02-01T10:25:40Z-
dc.identifier.citationPediatr Surg Int. 2010 Nov;26(11):1065-9.en_GB
dc.identifier.issn1437-9813 (Electronic)en_GB
dc.identifier.issn0179-0358 (Linking)en_GB
dc.identifier.pmid20714729en_GB
dc.identifier.doi10.1007/s00383-010-2692-4en_GB
dc.identifier.urihttp://hdl.handle.net/10147/207464-
dc.description.abstractPURPOSE: Hirschsprung's disease is characterised by the congenital absence of ganglion cells beginning in the distal rectum and extending proximally for varying distances. 'Zonal aganglionosis' is a phenomenon involving a zone of aganglionosis occurring within normally innervated intestine. 'Skip segment' Hirschsprung's disease (SSHD) involves a 'skip area' of normally ganglionated intestine, surrounded proximally and distally by aganglionosis. While Hirschsprung's disease is believed to be the result of incomplete craniocaudal migration of neural crest-derived cells, the occurrence of SSHD has no clear embryological explanation. The aim of this study was to perform a systematic review of SSHD, reported in the literature between 1954 and 2009, in order to determine the clinical characteristics of this rare entity and its significance. METHODS: The first reported case of SSHD was published in 1954. A systematic review of SSHD cases in the literature, from 1954 to 2009, was carried out using the electronic database 'Pubmed'. Detailed information was recorded regarding the age, gender, presenting symptoms and location of the skip segment in each patient. RESULTS: 24 cases of SSHD have been reported in the literature to date. 18/24 (75%) of these cases were males and 6/24 (25%) were females. Of these, 22/24 (92%) were cases of total colonic aganglionosis (TCA), and 2/24 (8%) were rectosigmoid Hirschsprung's disease. Of the 22 TCA cases, 9 (41%) had a skip segment in the transverse colon, 6 (27%) in the ascending colon, 2 (9%) in the caecum and 5 (23%) had multiple skip segments. In both rectosigmoid Hirschsprung's disease cases, the skip segment was in the sigmoid colon. Overall, the length of the skip segment was variable, with the entire transverse colon ganglionated in some cases. CONCLUSION: SSHD occurs predominantly in patients with TCA. The existence of a skip area of normally innervated colon in TCA may influence surgical management, enabling surgeons to preserve and use the ganglionated skip area during pull-through operations.en_GB
dc.language.isoengen_GB
dc.subject.meshColon/*innervationen_GB
dc.subject.meshEnteric Nervous System/*pathologyen_GB
dc.subject.meshFemaleen_GB
dc.subject.meshHirschsprung Disease/embryology/*pathologyen_GB
dc.subject.meshHumansen_GB
dc.subject.meshMaleen_GB
dc.titleSkip segment Hirschsprung's disease: a systematic review.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-

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