Presentation
Long standing abdominal pain, normal liver function test.
Patient Data
Both kidneys are enlarged, measuring about 22 cm in length bilaterally, occupied by innumerable, variable-sized cysts with different densities, some of which are hyperattenuating (hemorrhagic).
Multiple innumerable hypoattenuating liver lesions, without obvious biliary connection.
Features suggesting autosomal-dominant polycystic kidney and liver disease.
Case Discussion
Patients with autosomal-dominant polycystic disease present a wide spectrum of hepatic and renal involvement. Some patients have renal cysts only; others have liver cysts only; while others show variable degrees of both kidney and liver involvement.
The morbidity and mortality of autosomal-dominant polycystic kidney disease are largely attributable to the renal disease, although rupture of berry aneurysms of the circle of Willis may lead to patient death.
Even in severe disease, hepatic failure is rare, and most patients are asymptomatic, however, patients with extensive polycystic liver disease may develop jaundice as a result of extrinsic compression of the common hepatic duct by cysts in the porta hepatis.
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