Document Detail

Clinical features of Hirschsprung's disease associated with Down syndrome: a 30-year retrospective nationwide survey in Japan.
MedLine Citation:
PMID:  20006024     Owner:  NLM     Status:  MEDLINE    
PURPOSE: To identify the clinical features in diagnosis and treatment of Hirschsprung's disease (HD) associated with Down syndrome (DS), the authors retrospectively analyzed data for patients with DS from the past 3 nationwide surveys in Japan. This survey was already approved by the Japanese Society of Pediatric Surgeons. METHODS: Patient data were collected in 3 phases-phase I (1978-1982), n = 47; phase II (1988-1992), n = 79; and phase III (1998-2002), n = 90. In total, data on 216 patients (5.6%) of 3852 were collected and analyzed. RESULTS: The incidence of DS in patients with HD was 2.9%, 7.1%, and 8.2% in phases I, II, and III, respectively, with a corresponding male/female ratio of 5:1, 2.4:1, and 5:1. The ratio of the extent of aganglionosis was nearly consistent across all phases. In phases I, II, and III, the incidence of total colonic aganglionosis was 2.1%, 0%, and 2.2%; and that of cardiovascular anomalies, 36.1%, 45.6%, and 55.6%; and that of preoperative enterocolitis, 31.0%, 26.6%, and 24.4%. The 2 most common surgical procedures were the Soave procedure, including transanal endorectal pull-through, and Duhamel procedure including Z-shaped anastomosis. The mortality rate decreased over time, from 26.1% in phase I to 11.4% in phase II and 7.8% in phase III. Almost all mortality cases were associated with cardiovascular anomalies: 54.5%, 62.5%, and 85.7% in phases I, II, and III, respectively. CONCLUSIONS: The incidence of HD with DS has increased over time. The number of male patients and cardiac anomalies has also increased in the last 10 years. Total colonic aganglionosis was rare. A marked decrease in the overall mortality rate was observed.
Satoshi Ieiri; Mayumi Higashi; Risa Teshiba; Isamu Saeki; Genshiro Esumi; Junko Akiyoshi; Takanori Nakatsuji; Tomoaki Taguchi
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Publication Detail:
Type:  Comparative Study; Journal Article    
Journal Detail:
Title:  Journal of pediatric surgery     Volume:  44     ISSN:  1531-5037     ISO Abbreviation:  J. Pediatr. Surg.     Publication Date:  2009 Dec 
Date Detail:
Created Date:  2009-12-16     Completed Date:  2010-02-12     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0052631     Medline TA:  J Pediatr Surg     Country:  United States    
Other Details:
Languages:  eng     Pagination:  2347-51     Citation Subset:  IM    
Department of Pediatric Surgery, Reproductive and Developmental Medicine, Kyushu University, Fukuoka 812-8582, Japan.
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MeSH Terms
Abnormalities, Multiple / diagnosis,  epidemiology
Digestive System Surgical Procedures / methods
Down Syndrome / diagnosis*,  epidemiology*
Enterocolitis / epidemiology
Health Surveys
Heart Defects, Congenital / diagnosis,  epidemiology,  surgery
Hirschsprung Disease / diagnosis*,  epidemiology*,  surgery
Japan / epidemiology
Longitudinal Studies
Retrospective Studies
Sex Factors
Treatment Outcome

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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