A meta-analysis of clinical outcome in patients with total intestinal aganglionosis.

Hdl Handle:
http://hdl.handle.net/10147/207441
Title:
A meta-analysis of clinical outcome in patients with total intestinal aganglionosis.
Authors:
Ruttenstock, Elke; Puri, Prem
Affiliation:
Children's Research Centre, Our Lady's Children's Hospital, Crumlin, Dublin-12,, Ireland.
Citation:
Pediatr Surg Int. 2009 Oct;25(10):833-9.
Journal:
Pediatric surgery international
Issue Date:
1-Feb-2012
URI:
http://hdl.handle.net/10147/207441
DOI:
10.1007/s00383-009-2439-2
PubMed ID:
19669647
Abstract:
Total intestinal aganglionosis (TIA) is the most extreme and rare form of Hirschsprung disease (HD). Until few years ago TIA was considered to be a uniformly fatal condition. Survival has improved in the recent years with the advent of parenteral nutrition, innovative surgical techniques and small bowel transplantation. The purpose of this meta-analysis was to determine the clinical outcome of TIA following various surgical procedures. A meta-analysis of cases of TIA reported in the literature between 1985 and 2009 was performed. Detailed information was recorded regarding the extent of aganglionosis, surgical procedures performed and clinical outcome. In case of survivors, authors of reports were contacted to obtain the up-to-date clinical status of the patient. There were 68 cases of TIA reported worldwide, 40 (58.8%) males and 28 (41.2%) females. 6 (8.8%) patients had extension of aganglionosis up to the stomach, 19 (27.9%) up to the duodenum and 43 (63.2%) patients had aganglionosis up to 20 cm below the duodeno-jejunal flexure. Family history of HD was documented in 10 (14.7%) patients. RET-gene mutation were identified in 10 (71.4%) of the 14 patients investigated of RET germline mutations. Eleven patients (16.2%) died prior to surgical treatment, 25 patients (36.8%) only had jejunostomy, while 20 (29.4%) had Ziegler's myectomy-myotomy. 12 (17.6%) patients received intestinal transplantation (ITx) or combined liver-intestinal transplantation (LITx). Forty-five (66.2%) patients died at ages ranging from 1 day to 8 years. Twenty-three (33.8%) patients were alive; the longest survivor was 10 years old after LITx. Innovative surgical procedures and parenteral nutrition have improved clinical outcome of patients with TIA in recent years. Intestinal transplantation appears promising in the management of TIA.
Language:
eng
MeSH:
Adolescent; Child; Child, Preschool; Female; Hirschsprung Disease/diagnosis/*surgery; Humans; Infant; Infant, Newborn; Male; Treatment Outcome
ISSN:
1437-9813 (Electronic); 0179-0358 (Linking)

Full metadata record

DC FieldValue Language
dc.contributor.authorRuttenstock, Elkeen_GB
dc.contributor.authorPuri, Premen_GB
dc.date.accessioned2012-02-01T10:25:05Z-
dc.date.available2012-02-01T10:25:05Z-
dc.date.issued2012-02-01T10:25:05Z-
dc.identifier.citationPediatr Surg Int. 2009 Oct;25(10):833-9.en_GB
dc.identifier.issn1437-9813 (Electronic)en_GB
dc.identifier.issn0179-0358 (Linking)en_GB
dc.identifier.pmid19669647en_GB
dc.identifier.doi10.1007/s00383-009-2439-2en_GB
dc.identifier.urihttp://hdl.handle.net/10147/207441-
dc.description.abstractTotal intestinal aganglionosis (TIA) is the most extreme and rare form of Hirschsprung disease (HD). Until few years ago TIA was considered to be a uniformly fatal condition. Survival has improved in the recent years with the advent of parenteral nutrition, innovative surgical techniques and small bowel transplantation. The purpose of this meta-analysis was to determine the clinical outcome of TIA following various surgical procedures. A meta-analysis of cases of TIA reported in the literature between 1985 and 2009 was performed. Detailed information was recorded regarding the extent of aganglionosis, surgical procedures performed and clinical outcome. In case of survivors, authors of reports were contacted to obtain the up-to-date clinical status of the patient. There were 68 cases of TIA reported worldwide, 40 (58.8%) males and 28 (41.2%) females. 6 (8.8%) patients had extension of aganglionosis up to the stomach, 19 (27.9%) up to the duodenum and 43 (63.2%) patients had aganglionosis up to 20 cm below the duodeno-jejunal flexure. Family history of HD was documented in 10 (14.7%) patients. RET-gene mutation were identified in 10 (71.4%) of the 14 patients investigated of RET germline mutations. Eleven patients (16.2%) died prior to surgical treatment, 25 patients (36.8%) only had jejunostomy, while 20 (29.4%) had Ziegler's myectomy-myotomy. 12 (17.6%) patients received intestinal transplantation (ITx) or combined liver-intestinal transplantation (LITx). Forty-five (66.2%) patients died at ages ranging from 1 day to 8 years. Twenty-three (33.8%) patients were alive; the longest survivor was 10 years old after LITx. Innovative surgical procedures and parenteral nutrition have improved clinical outcome of patients with TIA in recent years. Intestinal transplantation appears promising in the management of TIA.en_GB
dc.language.isoengen_GB
dc.subject.meshAdolescenten_GB
dc.subject.meshChilden_GB
dc.subject.meshChild, Preschoolen_GB
dc.subject.meshFemaleen_GB
dc.subject.meshHirschsprung Disease/diagnosis/*surgeryen_GB
dc.subject.meshHumansen_GB
dc.subject.meshInfanten_GB
dc.subject.meshInfant, Newbornen_GB
dc.subject.meshMaleen_GB
dc.subject.meshTreatment Outcomeen_GB
dc.titleA meta-analysis of clinical outcome in patients with total intestinal aganglionosis.en_GB
dc.contributor.departmentChildren'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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