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Auxiliary partial orthotopic living donor liver transplantation for a child with congenital absence of the portal vein

โœ Scribed by Yuji Soejima; Tomoaki Taguchi; Keiko Ogita; Akinobu Taketomi; Tomoharu Yoshizumi; Hideaki Uchiyama; Takuro Ohno; Mitsuo Shimada; Yoshihiko Maehara

Publisher
John Wiley and Sons
Year
2006
Tongue
English
Weight
153 KB
Volume
12
Category
Article
ISSN
1527-6465

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โœฆ Synopsis

Congenital absence of the portal vein (CAPV) is a rare malformation of the mesenteric vasculature in which visceral venous blood bypasses the liver, completely draining into the systemic circulation through a congenital porto-systemic shunt. Liver transplantation has rarely been indicated for patients with this disease. We present a child with CAPV who was managed successfully by living donor auxiliary partial orthotopic liver transplantation (APOLT), while preserving the right lobe of the native liver. In conclusion, APOLT for patients with CAPV is a feasible and ideal procedure because portal vein (PV) diversion is not necessary.

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## Congenital absence of the portal vein (CAPV) is a very rare venous malformation in which mesenteric venous blood drains directly into the systemic circulation. There is no portal perfusion of the liver and no portal hypertension. This abnormality is usually coincidentally discovered in children,