Document Detail

Cecal volvulus in the Cornelia de Lange syndrome.
MedLine Citation:
PMID:  7807358     Owner:  NLM     Status:  MEDLINE    
Cornelia de Lange syndrome is a congenital malformation characterized by severe growth failure, mental retardation, and multiple physical anomalies. A variety of gastrointestinal anomalies have been described, including malrotation, colonic duplication, and nonfixation of the colon. Two patients with Cornelia de Lange syndrome presented to our institution with acute distal bowel obstruction. In both cases, emergency laparotomy showed cecal volvulus with necrosis of the terminal ileum, cecum, and ascending colon, secondary to nonfixation of the colon. Resection and an end-ileostomy were performed and later successfully reversed in both patients. Intestinal obstruction is a known cause of death in these children, and nonfixation of the colon has been identified during autopsy. Parents of children with Cornelia de Lange syndrome should be counseled as to the possibility of bowel obstruction resulting from cecal volvulus. This awareness may lead to earlier identification and treatment of this potentially lethal gastrointestinal tract anomaly.
K Husain; P Fitzgerald; G Lau
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Journal of pediatric surgery     Volume:  29     ISSN:  0022-3468     ISO Abbreviation:  J. Pediatr. Surg.     Publication Date:  1994 Sep 
Date Detail:
Created Date:  1995-02-02     Completed Date:  1995-02-02     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  0052631     Medline TA:  J Pediatr Surg     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  1245-7     Citation Subset:  IM    
Division of Pediatric Surgery, Children's Hospital at Chedoke-McMaster, Hamilton, Ontario, Canada.
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MeSH Terms
Cecal Diseases / radiography,  surgery*
Child, Preschool
Colon / abnormalities
De Lange Syndrome / radiography,  surgery*
Intestinal Obstruction / radiography,  surgery*

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