Document Detail

Congenital extrahepatic portosystemic shunt associated with heterotaxy and polysplenia.
MedLine Citation:
PMID:  20069288     Owner:  NLM     Status:  MEDLINE    
BACKGROUND: Heterotaxy with polysplenia is associated with many cardiovascular anomalies including the occasional occurrence of congenital extrahepatic portosystemic shunts (CEPS). Missing this anomaly can lead to inappropriate and ineffective therapy. OBJECTIVE: To emphasize the importance and associated anatomy of CEPS in conjunction with heterotaxy with polysplenia. MATERIALS AND METHODS: Review of three young children who presented with cyanosis and pulmonary hypertension without a cardiac etiology. They were known (1) or discovered (2) to have heterotaxy with polysplenia. RESULTS: There was absence of the intrahepatic inferior vena cava (IVC) with azygos or hemiazygos continuation in all three cases. In spite of normal liver function, they were discovered to have large portosystemic shunts, splenorenal in location, along with diffuse peripheral pulmonary arterial dilatation suggestive of CEPS (Abernethy malformation) with hepatopulmonary or, more accurately, portopulmonary syndrome. All CEPS were ipsilateral to the spleens. Patency of the portal veins in these cases allowed for percutaneous shunt closure with resolution of cyanosis. CONCLUSION: CEPS is associated with heterotaxy with polysplenia and can be symptomatic because of pulmonary arteriovenous (AV) shunting. Portal and hepatic vein patency are critical for determining feasibility of CEPS closure.
Beverley Newman; Jeffrey A Feinstein; Ronald A Cohen; Brian Feingold; Jacqueline Kreutzer; Hitendra Patel; Fandics P Chan
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Publication Detail:
Type:  Case Reports; Journal Article     Date:  2010-01-13
Journal Detail:
Title:  Pediatric radiology     Volume:  40     ISSN:  1432-1998     ISO Abbreviation:  Pediatr Radiol     Publication Date:  2010 Jul 
Date Detail:
Created Date:  2010-06-10     Completed Date:  2010-10-12     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0365332     Medline TA:  Pediatr Radiol     Country:  Germany    
Other Details:
Languages:  eng     Pagination:  1222-30     Citation Subset:  IM    
Department of Radiology, Stanford University School of Medicine, Lucile Packard Children's Hospital, 725 Welch Road, Stanford, CA 94305, USA.
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MeSH Terms
Abnormalities, Multiple / diagnosis*
Bile Ducts, Extrahepatic / radiography*,  ultrasonography*
Child, Preschool
Diagnostic Imaging / methods*
Spleen / abnormalities*,  radiography*,  ultrasonography
Vena Cava, Inferior / abnormalities*,  radiography*,  ultrasonography

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