Congenital absence of the portal vein in liver transplantation for biliary atresia.

Abstract

BACKGROUND:

Extrahepatic biliary atresia is the most common indication for liver transplantation in children. Coexistent congenital anomalies are common (25%), but the combination of polysplenia syndrome (10%) and absence of portal vein is rare.

AIMS:

We report a case of successful reduced size liver transplantation on a 13-month-old girl with extrahepatic biliary atresia, polysplenia syndrome and congenital absence of the portal vein.

METHODS:

Technical and post-operative problems related to absence of the portal vein and polysplenia are described and the literature reviewed.

RESULTS:

The donor portal vein was successfully anastomosed to the confluence of the superior mesenteric vein and splenic vein. The child is well with good graft function at a follow-up of 20 months.

CONCLUSION:

Absence of the portal vein and polysplenia syndrome is not a contraindication for liver transplantation although an increased post-operative morbidity may be expected.

• Authors: P Andreani, P Srinivasan, C S Ball, N D Heaton, M Rela
• Journal: International journal of surgical investigation (Int J Surg Investig) Vol. 2 Issue 1 Pg. 81-4 ( 2000) ISSN: 1028-5229 [Print] Switzerland
• PMID: 12774342 (Publication Type: Case Reports, Journal Article, Review)

Topics

• Biliary Atresia (complications, surgery)
• Female
• Humans
• Infant
• Liver Transplantation
• Portal Vein (abnormalities)
• Spleen (abnormalities)