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

Abstract

Patient: Female, 17Final Diagnosis: Fulminant Wilson’s diseaseSymptoms: General jaundice • malaise • abdominal painMedication: —Clinical Procedure: ICUSpecialty: TransplantologyObjective:Rare diseaseBackground: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.

Living donor liver transplantation for Wilson disease: A collective review

“…A Case of LDLT for WD-Induced ALF in Japan [20] Authors report the case of a patient with WD associated with fulminant hepatic failure (WD-FHF) who underwent LDLT. 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 corneal ring, and ALF, acute renal failure and grade 2 hepatic encephalopathy.…”

Living Related Liver Transplantation for Metabolic Liver Diseases in Children

“…Published experience, predominantly from Asian populations, has shown that transplants using heterozygous donors are safe with complete functional correction of the defect (7)(8)(9)(10)(11)(12)(13)(14)(15). Heterozygotes for WD gene have abnormal ceruloplasmin levels in almost 25% of subjects, most of whom have normal urinary copper excretion (11).…”

Pathophysiology of Acute Liver Failure

“…WD is an autosomal‐recessive disease characterized by copper buildup in the liver and other end organs . Patients can present with neurologic symptoms, chronic liver disease, or ALF . Fulminant WD represents about 3% of ALF cases and usually occurs in young female patients within the first 2 decades of life .…”