A 79 y.o. woman presents with RUQ pain
What do you notice on CT?
Our patient had polycystic liver disease which is defined as ten or more cysts in the liver. Her pain was probably from bleeding into a cyst.
Polycystic liver disease is an inherited disorder affecting 1 in 100,000 people. The cysts enlarge with age and usually become symptomatic by age 50. Three gene mutations have been linked to polycystic liver disease PRKCSH,LRP5 and SEC63 but more than 50% of individuals with the disease do not have these. As the cysts enlarge they can produce bile duct compression, IVC thrombosis and PE, or portal hypertension. They commonly enlarge under the influence of estrogen and therefore are more common in women.
The differential for liver cysts includes hydatid disease, neoplastic cysts (cystadenoma, cystic sarcoma, metastatic ca from the ovaries, colon, kidneys or pancreas) and bile duct related cysts (Caroli’s disease, bile duct duplication)
World-wide, the most common liver cyst is caused by infection with the larval stage of a tapeworm, Echinococcus granulosus. Dogs are the definitive host and sheep, cattle, goats and pigs are the intermediate hosts. In northern areas wolf and deer can be hosts, especially where reindeer are domesticated. In the European type, the definitive host is the dog but even camels can be intermediate hosts.
hydatid cysts can occur in many organs including uterus and bone.
The differential for liver cysts includes hydatid disease, neoplastic cysts (cystadenoma, cystic sarcoma, metastatic ca from the ovaries, colon, kidneys or pancreas) and bile duct related cysts (Caroli’s disease, bile duct duplication). Our patients pain resolved and she was discharged without treatment. In severe cases of polycystic liver a transplant can be performed.
Gevers TJ, Drenth JP. Diagnosis and management of polycystic liver disease. Nat Rev Gastroenterol Hepatol. 2013. 10(2):101-8
Abu-Wasel B, et al. Pathophysiology, epidemiology, classification and treatment options for polycystic liver disease. World J Gastroenterol. 2013. 19(35):5775-86.
Miliadis L, Giannakopoulos, et al. Spontaneous rupture of a large non-parasitic liver cyst: a case report J Med Case Reports 2010;4:2 doi 10.2286/1752-1947-4-2.