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19 September 2015 : Case report  Romania

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 coexistence of disease or pathology

Vladislav BrasoveanuADEF, Mihnea Ioan IonescuBEF, Razvan GrigorieC, Mariana MihailaAB, Nicolae BacalbasaF, Radu DumitruD, Vlad HerleaBD, Andreea IorgescuC, Dana TomescuAD, Irinel PopescuACDEF

DOI: 10.12659/AJCR.895235

Am J Case Rep 2015; 16:637-644

Abstract

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

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American Journal of Case Reports eISSN: 1941-5923
American Journal of Case Reports eISSN: 1941-5923