Hepatic Cysts

A 43-year old female with a history of autosomal dominant polycystic kidney disease (ADPKD) presented to the emergency room with a gradual onset of right upper quadrant abdominal pain for the past 7 days. She described the pain as dull and associated with intermittent episodes of nausea. Physical examination was remarkable for profound hepatomegaly and marked nodularity of the liver edge. Laboratory evaluation was significant for a creatinine of 4.1 mg/dL. Liver function tests were within normal range.

Subsequent computed tomography (CT) of the abdomen revealed innumerable cystic lesions of varying sizes in the liver and kidney.

Discussion. Hepatic cysts are the most common extrarenal manifestation of ADPKD and have a highly variable prevalence. The prevalence of hepatic cysts increases with age and are thought to be directly proportional to worsening renal cystic disease and renal function.^1-2

Despite the severity of hepatic cystic disease, liver function is often persevered—with only mild increases in transaminase levels typically noted.² In cases of massive hepatic cystic lesions, increases in transaminases, alkaline phosphatase, and portal-systemic shunting may be present.²

Hepatic cysts often remain clinically silent. However, patients may present with complaints of abdominal pain. In a small number of patients, abdominal pain may be disabling—likely secondary to massive hepatomegaly caused by a large number of cysts.¹ Rare complications of hepatic cystic disease such as cyst infection, obstructive jaundice, and portal hypertension have been also reported.¹

Partial hepatectomy, surgical fenestration, alcohol sclerosis, and percutaneous drainage of several dominant cysts are modalities often used to alleviate the symptoms of hepatic cystic disease if present.^2,3 

Our patient refused any intervention at this time, despite the explanation of potential complications. ■

References:

1.  Perrone R. Extrarenal manifestations of ADPKD. Kindey Int. 1997;51:2022-2036.

2.  Thomsen H, Thaysen J. Frequency of hepatic cysts in adult polycystic kidney disease. Acta Med Scand. 1988;224:381-384.

3.  Telenti A, Torres V, Gross JJ, et al. Hepatic cyst infection in autosomal dominant polycystic kidney disease. Mayo Clinic Proc. 1990;65:933-942.