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17 March 2018 : Case report  Japan

Living Donor Liver Transplantation for Wilson’s Disease Associated with Fulminant Hepatic Failure: A Case Report

Management of emergency care, Rare disease

Yu Huang12ABCDEF, Mitsuhisa Takatsuki1ABCDEF, Akihiko Soyama1ABCDEF, Masaaki Hidaka1ABCDEF, Shinichiro Ono1BDE, Tomohiko Adachi1BDE, Takanobu Hara1BDE, Satomi Okada1BDE, Takashi Hamada1BDE, Susumu Eguchi1ADEFG

DOI: 10.12659/AJCR.907494

Am J Case Rep 2018; 19:304-308

Abstract

BACKGROUND: Liver transplantation is indicated for patients with Wilson’s disease (WD) who present either with acute liver failure or with end-stage liver disease and severe hepatic insufficiency as the first sign of disease. However, almost all reported cases have been treated with death donor liver transplantation. Here we report the case of a patient with WD associated with fulminant hepatic failure (WD-FHF) who underwent living donor liver transplantation (LDLT).

CASE REPORT: A 17-year-old female was diagnosed with WD-FHF based on high uric copper (10 603 μg/day, normal <100 μg/day), low serum ceruloplasmin (15 mg/dL, normal >20 mg/dL) and Kayser-Fleischer (K-F) corneal ring, and acute liver failure (ALF), acute renal failure (ARF) and grade 2 hepatic encephalopathy (HE). The model for end-stage liver disease (MELD) score was 35. Due to her critical condition, the patient underwent LDLT utilizing a right liver graft from her 44-year-old mother. The right hepatic vein (RHV) and inferior right hepatic vein (iRHV) were reconstructed. She developed severe liver dysfunction due to a crooked hepatic vein caused by compression from the large graft. To straighten the bend, a reoperation was performed. During the operation, we tried to relieve the compressed hepatic vein by adjusting the graft location, but the benefits were limited. We therefore performed stenting in both the RHV and iRHV on postoperative day 9. The patient gradually improved, exhibiting good liver and renal functions, and was finally discharged on postoperative day 114.

CONCLUSIONS: When WD-FHF deteriorates too rapidly for conservative management, LDLT is an effective therapeutic strategy.

Keywords: Hepatolenticular Degeneration, Liver Failure, Acute, Liver Transplantation

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