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Living Donor Liver Transplantation for Unresectable Liver Adenomatosis Associated with Congenital Absence of Portal Vein: A Case Report and Literature Review

Unusual setting of medical care, Rare disease, Educational Purpose (only if useful for a systematic review or synthesis), Rare co-existance of disease or pathology

Vladislav Brasoveanu, Mihnea Ioan Ionescu, Razvan Grigorie, Mariana Mihaila, Nicolae Bacalbasa, Radu Dumitru, Vlad Herlea, Andreea Iorgescu, Dana Tomescu, Irinel Popescu

“Dan Setlacec” Center of General Surgery and Liver Transplantation, Fundeni Clinical Institute, Bucharest, Romania

Am J Case Rep 2015; 16:637-644

DOI: 10.12659/AJCR.895235

Available online:

Published: 2015-09-19

BACKGROUND: Abernethy malformation (AM), or congenital absence of portal vein (CAPV), is a very rare disease which tends to be associated with the development of benign or malignant tumors, usually in children or young adults.
CASE REPORT: We report the case of a 21-year-old woman diagnosed with type Ib AM (portal vein draining directly into the inferior vena cava) and unresectable liver adenomatosis. The patient presented mild liver dysfunction and was largely asymptomatic. Living donor liver transplantation was performed using a left hemiliver graft from her mother. Postoperatively, the patient attained optimal liver function and at 9-month follow-up has returned to normal life.
CONCLUSIONS: We consider that living donor liver transplantation is the best therapeutic solution for AM associated with unresectable liver adenomatosis, especially because compared to receiving a whole liver graft, the waiting time on the liver transplantation list is much shorter.

Keywords: Female, Adenoma - surgery, Follow-Up Studies, Humans, Liver Neoplasms - surgery, Liver Transplantation, Living Donors, Portal Vein - abnormalities, Time Factors, Vascular Malformations - surgery, young adult