Document Detail

Sirenomelia with esophageal atresia.
MedLine Citation:
PMID:  11436149     Owner:  NLM     Status:  MEDLINE    
Sirenomelia, is a rare fatal condition characterized by fusion of the lower extremities. It has been suggested that sirenomelia is a severe form of caudal regression syndrome complex for which the pathogenesis is controversial. Our case is a sirenomelia associated with esophageal atresia and tracheoesophageal fistula. In the literature, it has been pointed out that the VATER association may represent a less severe form of sirenomelia but no sirenomelia case associated with esophageal atresia and tracheoesophageal fistula has been found. Finally, we detected a single large artery which diverts the blood of the embryo's caudal part to the placenta with the detailed autopsy especially with focusing on the abdominal vasculature. This vascular steal is thought to be the main pathogenic mechanism of the condition.
S Sözübir; F Güven; T Ozkamaci; F Yildiz; M Tugay; A Say; O Z Pektas
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Advances in clinical pathology : the official journal of Adriatic Society of Pathology     Volume:  4     ISSN:  1125-5552     ISO Abbreviation:  Adv Clin Path     Publication Date:  2000 Oct 
Date Detail:
Created Date:  2001-07-03     Completed Date:  2001-08-02     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  9709997     Medline TA:  Adv Clin Path     Country:  Italy    
Other Details:
Languages:  eng     Pagination:  165-8     Citation Subset:  IM    
Department of pediatric surgery, Facutlty of Medicine, University of Kocaeli, Izmit, Istanbul.
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MeSH Terms
Abdomen / blood supply
Blood Vessels / abnormalities,  pathology
Ectromelia / complications*,  pathology
Esophageal Atresia / complications*
Fatal Outcome

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