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Volume: 15 Issue: 1 February 2017 - Supplement - 1

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Auxiliary Partial Orthotopic Living Liver Transplant for Wilson Disease

Wilson disease is a genetic disease involving copper metabolism disturbances that result in copper accumulations, especially in the liver and brain. Wilson disease can be treated with pharmacologic agents, such as chelators that induce urinary excretion of copper or zinc salts that inhibit copper absorption in the digestive tract. Liver transplant is the only treatment option for Wilson disease when liver failure has occurred. In some patients, that is, in those with Child-Pugh A score, neurologic disease can be seen without hepatic failure. Our recommendation is for these patients to have auxiliary partial orthotopic liver transplant. Here, we present a 36-year-old male patient with neurologic disease associated with Wilson disease who had successful related living-donor auxiliary partial orthotopic liver transplant using a left lobe. The patient, as a result of neurologic symptoms that included tremor walking and speaking problems and low serum ceruloplasmin level of 7 mg/dL, was diagnosed with Wilson disease, and a liver biopsy was performed. Chronic necroinflammatory disease activity was 4/18, and the patient received chelation treatment. His hepatic functions were normal. The donor was the patient’s 57-year-old father whose liver function tests were also normal. The graft-to-recipient weight ratio was 1% using a left lobe graft. After transplant, serum ceruloplasmin levels on day 15 and month 1 were 14 and 19 mg/dL. At month 1, liver function tests were normal. Doppler ultrasonography showed normal vascular flow of the native liver and the graft. The patient’s neurologic symptoms were progressively reduced. Progressive neurologic deterioration with no hepatic insufficiency is considered a suitable indication for auxiliary partial orthotopic liver transplant; this procedure is suggested before the neurologic and liver failure symptoms of Wilson disease occur.


Key words : Child-Pugh score, Neurologic disease, Progressive neurological deterioration

Introduction

Wilson disease (WD) is a genetic disease involving copper metabolism disturbances that result in copper accumulations, especially in the liver and brain. Wilson disease can be treated with pharmacologic agents, such as chelators that induce urinary ex­cretion of copper or zinc salts that inhibit copper absorption in the digestive tract.1 Liver transplant is the only treatment option for WD patients when liver failure has occurred.2 In some patients, that is, in those with Child-Pugh A score, neurologic disease can be seen without hepatic failure. Our recom­mendation is for these patients to have an auxiliary partial orthotopic liver transplant procedure. Here, we report a case of successful related living-donor auxiliary partial orthotopic liver transplant that used a left lobe in a patient with neurologic disease associated with WD.

Case Report

The patient was a 36-year-old man with neurologic symptoms of WD such as tremor walking and speaking problems. Wilson disease was diagnosed, with the patient having a low serum ceruloplasmin level of 7 mg/dL. Liver biopsy was performed. Chronic necroinflammatory disease activity was 4/18. The patient was referred to the department of neurology for chelation treatment. His hepatic functions were normal.

The donor was the patient’s 57-year-old father whose liver function tests were normal. The graft-to-recipient weight ratio was 1% using a left lobe graft. A recipient left hepatectomy and a donor left hepatectomy were performed. The donor’s left lobe was then implanted in the left side of the recipient. The left-middle hepatic vein of the graft was anastomosed in end-to-end fashion to the recipient’s left-middle hepatic vein. The left portal vein of the graft and the left portal vein of the recipient was also anastomosed in end-to-end fashion. The hepatic artery was anastomosed to the common hepatic artery in an end-to-side fashion. The bile duct of the graft was anastomosed to the common bile duct of the recipient by polytetrafluoroethylene graft (1.5 cm in length, 6 mm diameter). End-to-side anastomosis was done between the common bile duct and the polytetrafluoroethylene graft, and end-to-end anastomosis was done between the polytetrafluoro­ethylene graft and the graft bile duct (Figure 1 and Figure 2). The patient’s immunosup­pressive protocol included tacrolimus, steroids, and mycophenolate mofetil for maintenance therapy.

Serum ceruloplasmin levels on day 15, month 1, and month 3 posttransplant were 14 mg/dL, 19 mg/dL, and 27 mg/dL. Liver function tests at month 1 were normal. Doppler ultrasonography results showed normal vascular flow of the native liver and the graft. Abdominal computed tomo­graphy scans performed at month 1 were normal for native and graft livers (Figure 3). The patient’s neurologic symptoms were progressively relieved.

At month 2, postoperative bilirubin levels were elevated, but ultrasonographic findings were normal. We performed endoscopic retrograde cholangiopan­creatography. Sphincter Oddi stenosis was revealed. We performed endoscopic sphincterotomy and stent placement (Figure 4). After the endoscopic retrograde cholangiopancreatography procedure, bilirubin levels returned to normal ranges.

Discussion

Since 1988, auxiliary partial liver transplant has been an alternative treatment for orthotopic liver trans­plant.3 Auxiliary liver transplant is a technique in which the donor liver graft is placed alongside the native liver without native liver resection (heterotopic) or after resection of part of the native liver, replacing it with a segmental graft (orthotopic). In 2008, we reported the long-term survival of our first patient with heterotopic segmental auxiliary liver transplant and who is still alive with good graft function.4 Usually, auxiliary partial orthotopic liver transplant is a preferred treatment option for liver disorders in which the liver structure is preserved.1

Wilson disease may cause severe cirrhosis; however, hepatic carcinoma is rarely seen in these patients.1 Wilson disease associated with severe liver involvement is effectively cured by orthotopic liver transplant. Progressive neurologic deterioration with no hepatic insufficiency is considered a suitable indication for auxiliary partial orthotopic liver transplant.

Here, we report a successful auxiliary partial orthotopic liver transplant in a patient without cirrhosis and with neurologic disorder due to WD. Our patient was followed for 7 months. Normal ceruloplasmin level (20 mg/dL), normal liver function tests, and normal neurologic symptoms were observed. To our knowledge, we are the first to report on the efficacy of auxiliary partial orthotopic liver transplant in WD. This case showed that progressive neurologic deterioration with no hepatic insufficiency is considered a suitable indication for auxiliary partial orthotopic liver transplant. Therefore, we suggest that this procedure may also be considered for WD patients with combined hepatic and neuro­logic involvement. However, orthotopic liver trans­plant for patients with severe neurologic impairment is still open to debate.


References:

  1. Cheng Q, He SQ, Gao D, et al. Early application of auxiliary partial orthotopic liver transplantation in murine model of Wilson disease. Transplantation. 2015;99(11):2317-2324.
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  2. Litwin T, Dzieżyc K, Karliński M, Chabik G, Czepiel W, Członkowska A. Early neurological worsening in patients with Wilson’s disease. J Neurol Sci. 2015;355(1-2):162-167.
    CrossRef - PubMed
  3. Terpstra OT, Schalm SW, Weimar W, et al. Auxiliary partial liver transplantation for end-stage chronic liver disease. N Engl J Med. 1988; 319(23):1507-1511.
    CrossRef - PubMed
  4. Haberal M, Sevmis S, Karakayali H, Ozcay F, Moray G, Arslan G. Long-term survival in a patient after heterotopic segmental auxiliary liver transplantation. Pediatr Transplant. 2008 Nov;12(7):816-20.
    CrossRef - PubMed


Volume : 15
Issue : 1
Pages : 182 - 184
DOI : 10.6002/ect.mesot2016.P64


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From the Departments of 1General Surgery and Transplantation, 2Gastroenterology, 3Anesthesiology, and 4Pathology, Baskent Universality Faculty of Medicine, Ankara, Turkey
Acknowledgements: The authors declare that they have no sources of funding for this study, and they have no conflicts of interest to declare.
Corresponding author: Prof. Mehmet Haberal, MD, FACS (Hon), FICS (Hon), FASA (Hon), Department of General Surgery, Baskent University, Ankara, Turkey
Phone: +90 312 212 7393
Fax: +90 312 215 0835
E-mail: rectorate@baskent.edu.tr