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

J Pediatr Surg. 2008 Aug;43(8):e25-8. doi: 10.1016/j.jpedsurg.2008.04.016.


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.

Publication types

  • Case Reports

MeSH terms

  • Abnormalities, Multiple / diagnosis*
  • Child, Preschool
  • Follow-Up Studies
  • Heart Defects, Congenital / diagnosis*
  • Humans
  • Lichen Planus / diagnosis*
  • Male
  • Monitoring, Physiologic
  • Musculoskeletal Abnormalities / diagnosis*
  • Portal Vein / abnormalities*
  • Risk Assessment
  • Ultrasonography
  • Vena Cava, Inferior / abnormalities*
  • Vena Cava, Inferior / diagnostic imaging