Document Detail

Congenital atresia of portal vein with portocaval shunt associated with cardiac defects, skeletal deformities, and skin lesions in a boy.
MedLine Citation:
PMID:  18675622     Owner:  NLM     Status:  MEDLINE    
Congenital absence of portal vein is a rare anomaly that results from aberrant venous development in early embryonic life. The intestinal and splenic venous drainage bypass the liver and may drain directly into inferior vena cava or the left renal vein or the left hepatic vein. This rare anomaly is commonly associated with other congenital malformations and generally limited to females. We describe a rare case of aberrant portal vein development with congenital portocaval shunt (end-to-side) in a 3.5-year male child associated with cardiac defects (atrial and ventricular septal defects), skeletal deformities (flexion deformity and clinodactyly of digits and toes), and lichen planus with café au lait macules of skin.
Manphool Singhal; Anupam Lal; Babu R Thapa; Mahesh Prakash; Krishna P Shanbhogue; Niranjan Khandelwal
Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Journal of pediatric surgery     Volume:  43     ISSN:  1531-5037     ISO Abbreviation:  J. Pediatr. Surg.     Publication Date:  2008 Aug 
Date Detail:
Created Date:  2008-08-04     Completed Date:  2008-11-26     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0052631     Medline TA:  J Pediatr Surg     Country:  United States    
Other Details:
Languages:  eng     Pagination:  e25-8     Citation Subset:  IM    
Department of Radiodiagnosis, Postgraduate Institute of Medical Education and Research, Chandigarh 160012, India.
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MeSH Terms
Abnormalities, Multiple / diagnosis*
Child, Preschool
Follow-Up Studies
Heart Defects, Congenital / diagnosis*
Lichen Planus / diagnosis*
Monitoring, Physiologic
Musculoskeletal Abnormalities / diagnosis*
Portal Vein / abnormalities*
Risk Assessment
Vena Cava, Inferior / abnormalities*,  ultrasonography

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