Genitourinary Radiology > Kidneys > Masses > Cystic > Autosomal Dominant Polycystic Kidney Disease (ADPKD)


Autosomal Dominant Polycystic Kidney Disease (ADPKD)

 

ADPKD is an autosomal dominant disease with a prevalence of 0.1%, accounting for 10% of patients on chronic dialysis. Patients have multiple kidney cysts leading to enlarged, palpable kidneys. The disease progresses slowly, eventually resulting in end-stage renal disease and the need for dialysis or transplant.

ADPKD is associated with hepatic cysts (70%), intracranial berry aneurysms (50%), and pancreatic or splenic cysts (5%).

Imaging shows enlarged kidneys with multiple cysts that have variable signal characteristics. On T1-weighted MRI, some cysts have clear, watery fluid (hypointense) while others have blood and protein (hyperintense). Some cysts exhibit layering caused by cellular debris.

CT shows multiple cysts with variable density. Calcifications in the cyst wall are common, as are pressure deformities of the calyces and infundibula.

Contrast enhanced CT in a patient with ADPKD shows bilateral slightly enlarged kidneys which are nearly totally replaced by small renal cystic lesions, many of which contain calcifications in the cyst walls (arrows).

Gray scale ultrasound image of kidney in the sagittal (longitudinal plane) shows very little normal renal parenchyma (arrows) with the enlarged kidney replaced by innumerable simple cysts (C). Hydronephrosis would be a consideration except that these lesions do not connect to the collecting systemas hydronephrosis would (e.g., calyces to infundibuli to renal pelvis).

3D coronoal reconstruction from enhanced CT scan of the abdomen and pelvis in another patient with ADPKD. This patient also has innumerable cysts replacing the renal parenchyma and enlarged the bilateral kidneys. These kidneys were approximately 17-18 cm in length each (normal is 9 to 13 cm).


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