Orthotopic Liver Transplantation in Massive Polycystic Liver

Introduction: Liver failure is uncommon in patients with polycystic liver due to hepatic regeneration. The minority of patients will progress over the years to advanced liver disease or develop complications because of massive hepatomegaly. We present the case of a patient with a massive polycystic liver disease that required an orthotopic liver transplantation. Methods: 61 years old female with familiar and personal history of polycystic liver and kidney disease. Physical examination revealed a massive but not painful hepatomegaly. She presented several episodes of liver cyst infection, requiring antibiotic treatment and percutaneous drainage, however kidney and liver function were not altered. Treatment with subcutaneous Lanreotide was initiated but after a new episode of liver cyst infection, orthotopic liver transplantation was proposed. An abdominal CT scan was performed before surgery showing a sever liver and kidney polycystic diseases with no vascular abnormalities. Results: Hepatectomy was performed, with removal of the inferior vena cava and its total clamping. A giant hepatomegaly with several cysts over its entire surface and ascites (Image 1) was appreciated. Donor liver was implanted with anastomosis of the inferior vena cava, portal vein, biliary duct, and hepatic artery. Reperfusion was correct with a total ischemia of 7 and a half hours. Conclusion: Orthotopic liver transplantation in polycystic liver disease is a highly complex surgical intervention due to liver size, displacement of the vascular structures and organs, and with greater risk of blood loss during surgery.