Autosomal dominant hepato-renal polycystic disease (ADHRPD)

Case contributed by Dennis Odhiambo Agolah

Presentation

Clinically palpable abdominal mass in a patient with end stage renal disease (ESRD).

Patient Data

Age: 50 Years
Gender: Female

Both kidneys and the entire liver organ are diffusely enlarged with multiple variable sized surface parenchymal anechoic to hypoechoic cystic lesions that posteriorly enhances and are thin to thick-walled with some of the hepatic cysts on the right lobe exhibiting low to mid level internal echoes likely attributable to internal hemorrhagic insult. There is hepatomegaly (right lobe spans 21.5 cm in the mid-clavicular line) with bilateral nephromegaly (right kidney measures ~741 cc; left kidney measures ~1184 cc in volume).

The kidneys shows loss of cortical-medullary differentiation. Bilateral intra-renal arterial Doppler survey shows absent end diastolic flow (AEDF) with increased resistive indices.

Case Discussion

Entire hepatic Couinaud liver segments and the kidneys parenchyma are riddled with numerous smaller and larger cystic lesions resulting into diffuse hepato-nephromegaly consistent with autosomal dominant hepato-renal polycystic disease. They are avascular on color and power flow Doppler mapping.

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