Congenital atresia of portal vein with portocaval shunt associated with cardiac defects, skeletal deformities, and skin lesions in a boy

Manphool Singhal, Anupam Lal, Babu R. Thapa, Mahesh Prakash, Krishna P. Shanbhogue, Niranjan Khandelwal

Research output: Contribution to journalArticle

10 Scopus citations

Abstract

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.

Original languageEnglish (US)
Pages (from-to)e25-e28
JournalJournal of Pediatric Surgery
Volume43
Issue number8
DOIs
StatePublished - Aug 1 2008

Keywords

  • Café au lait macules
  • Cardiac anomalies
  • Clinodactyly
  • Congenital absence of portal vein
  • Lichen planus
  • Portocaval shunt

ASJC Scopus subject areas

  • Surgery
  • Pediatrics, Perinatology, and Child Health

Fingerprint Dive into the research topics of 'Congenital atresia of portal vein with portocaval shunt associated with cardiac defects, skeletal deformities, and skin lesions in a boy'. Together they form a unique fingerprint.

  • Cite this