Introduction:
Polycystic liver disease is observed in 75-90% of patients with autosomal dominant polycystic kidney disease (ADPKD). ADPKD has a high prevalence of 1/1000. Hepatomegaly severely reduces quality of life and liver transplantation seems to be method of choice for many patients. Because of the rarity of this disease and the small number of symptomatic patients with massive hepatomegaly indicated for the transplantation, there is no standard approach for explantation of the liver.
Case presentation:
In our case, 57-year-old woman with massive hepatomegaly was treated with simultaneous split liver and kidney transplantation with bilateral nephrectomy.
Discussion:
For the native liver excision we used unique surgical approach - sharp liver transection under hepatic vascular exclusion. Because we experienced some cases with massive bleeding during the polycystic liver explantation, we decided to change the surgical approach. The technique offers limited blood loss and comfortable operation field exposure.
Conclusion:
The giant polycystic liver could safely be explanted only using sharp transection hepatectomy under hepatic vascular exclusion. There is significant difference between blood loss in patients treated with or without hepatic vascular exclusion.