Polycystic liver disease: Classification, diagnosis, treatment process, and clinical management

Table 1 Brief summary of various polycystic liver disease

Disease	Genes mutation	Kidney involvement
ADPKD	PKD1, PKD2, GANAB	Yes
ARPKD	PKHD1	Yes
PCLD	PRKCSH, SEC63, LRP5, GANAB, ALG8, SEC61B, PKHD1	Usually not

ADPKD: Autosomal dominant polycystic kidney disease; ARPKD: Autosomal recessive polycystic kidney disease; PCLD: Isolated polycystic liver disease.

Table 2 Gigot classification

	Number of cysts	Cyst size	Remaining areas of noncystic liver parenchyma
Gigot type I	< 10	Large (> 10 cm)	Large
Gigot type II	Multiple	Small, medium	Large
Gigot type III	Multiple	Small, medium	Few

Table 3 Schnelldorfer classification

	Symptoms	Cyst characteristics	Areas of relative normal liver parenchyma	Isosectoral portal vein or hepatic vein occlusion of preserved sector
Type A	Absent or mild	Any	Any	Any
Type B	Moderate or severe	Limited Number with large cysts	> 2 sectors	Absent
Type C	Severe (or moderate)	Any	> 1 sector	Absent
Type D	Severe (or moderate)	Any	< 1 sector	Present

Table 4 Qian classification

	Number of cysts	Symptomatic hepatomegaly
Grade 0	0	No
Grade 1	1-10	No
Grade 2	11-20	No
Grade 3	> 20	No
Grade 4	> 20	Yes