Pathophysiology, epidemiology, classification and treatment options for polycystic liver diseases

Table 5 Summary of Schnelldorfer’s classification that aims at differentiating patients who could benefit from resection or transplantation

	Type A	Type B	Type C	Type D
Symptoms	Absent or mild	Moderate or severe	Severe (or moderate)	Severe (or moderate)
Cyst characteristics	Any	Limited No. large cysts	Any	Any
Areas of relative normal liver parenchyma	Any	≥ 2 sectors	≥ 1 sector	< 1 sector
Presence of portal vein or hepatic vein occlusion in the preserved hepatic sectors	Any	Absent	Absent	Present
Recommended therapy	Observation or medical therapy	Cyst fenestration	Partial hepatectomy with possible fenestration of remnant cysts	Liver Transplantation