Document Detail

Gastrointestinal malformations associated with prune belly syndrome: three cases and a review of the literature.
MedLine Citation:
PMID:  3537997     Owner:  NLM     Status:  MEDLINE    
Prune belly syndrome (PBS), a triad consisting of abdominal musculature hypoplasia, urinary tract malformations, and cryptorchidism, is frequently associated with other congenital malformations. Although it is acknowledged that gastrointestinal (GI) malrotation and mesenteric anomalies are frequent in PBS, other GI anomalies are generally considered to be exceedingly rare. Here we describe 3 autopsy cases with severe malformations of both midgut and hindgut derivatives and review the world literature to evaluate the spectrum of GI malformations associated with this syndrome. The relatively high frequency of distal stenoses and atresias suggests that the anomalous mesenteric attachments may predispose to prenatal volvulus and subsequent anatomic bowel obstruction. Postnatal volvulus is also occasionally observed. Infants with PBS also appear to be at a higher risk for persistence of the common fetal cloaca.
J R Wright; R F Barth; J C Neff; E T Poe; M E Sucheston; L E Stempel
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Publication Detail:
Type:  Case Reports; Journal Article; Research Support, Non-U.S. Gov't; Review    
Journal Detail:
Title:  Pediatric pathology / affiliated with the International Paediatric Pathology Association     Volume:  5     ISSN:  0277-0938     ISO Abbreviation:  Pediatr Pathol     Publication Date:  1986  
Date Detail:
Created Date:  1986-12-31     Completed Date:  1986-12-31     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  8303527     Medline TA:  Pediatr Pathol     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  421-48     Citation Subset:  IM    
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MeSH Terms
Digestive System Abnormalities*
Infant, Newborn
Prune Belly Syndrome / pathology*

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