Document Detail

A very rare case of polysplenia syndrome with congenital diffuse pulmonary arteriovenous fistulas.
MedLine Citation:
PMID:  16562797     Owner:  NLM     Status:  MEDLINE    
A five-year-old girl patient was admitted with cyanosis and dyspnea, which started from birth. She had small telangiectatic lesions on her face and cerebral arteriovenous malformation, but no family history of hereditary hemorrhagic telangiectasia. Contrast echocardiography and pulmonary angiography demonstrated diffuse pulmonary arteriovenous fistulas. The patient was diagnosed as polysplenia syndrome characterized with left atrial isomerism, interrupted inferior vena cava, azygous continuation to the superior vena cava, and hepatic veins draining to the right atrium. In contrast to the other polysplenia syndrome cases, in this patient, pulmonary arteriovenous fistulas were not associated with cavopulmonary anastomoses or liver disease.
Dolunay Gürses; Zülal Ulger; Ertürk Levent; A Ruhi Ozyürek
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  The Turkish journal of pediatrics     Volume:  48     ISSN:  0041-4301     ISO Abbreviation:  Turk. J. Pediatr.     Publication Date:    2006 Jan-Mar
Date Detail:
Created Date:  2006-03-27     Completed Date:  2006-04-25     Revised Date:  2007-11-15    
Medline Journal Info:
Nlm Unique ID:  0417505     Medline TA:  Turk J Pediatr     Country:  Turkey    
Other Details:
Languages:  eng     Pagination:  96-9     Citation Subset:  IM    
Department of Pediatric Cardiology, Ege University Faculty of Medicine, izmir, Turkey.
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MeSH Terms
Abnormalities, Multiple*
Arteriovenous Malformations / complications*
Child, Preschool
Heart Defects, Congenital*
Pulmonary Artery / abnormalities*
Pulmonary Veins / abnormalities*
Spleen / abnormalities*

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