Mass Effect from Hepatomegaly in Polycystic Kidney Disease

A 66-year-old man presented with progressive lower limb edema and an elevated jugular venous pressure. He had undergone renal transplantation 14 years earlier for adult polycystic kidney disease. Serum creatinine and liver function tests were normal. Echocardiogram (Fig. 1a, online video) showed normal left ventricular function (ejection fraction 68 %) but compression of the right atrium (RA) adjacent to the right ventricle (RV) by hepatic cysts (HC). Computed tomography (CT) scan confirmed compression of the right atrium (Fig. 1b) and inferior vena cava (IVC) by an enlarged liver with numerous cysts (Fig. 1c). Hepatic transplantation was considered, but the patient’s clinical condition deteriorated rapidly and he died before this could be pursued.

Figure 1.

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Hepatic involvement in polycystic kidney disease is common and can cause significant mass effect, compressing structures such as the great veins (leading to impaired venous drainage) and abdominal viscera (causing anorexia and weight loss, referred to as “lethal exhaustion” in severe cases).^1,2 These symptoms may be severe enough to warrant percutaneous or surgical cyst drainage. Rarely, even in the absence of biochemical liver failure or portal hypertension, hepatic transplantation may be considered if the symptoms are sufficiently extreme to justify the associated risks of this surgery.^1,3