Autosomal Dominant Polycystic Kidney Disease (ADPKD)

Imaging studies

A clear understanding of etiology, pathology, extra renal manifestations and complications is essential to plan a strategy for diagnostic imaging procedure and in the expectation of imaging findings.

• Does not produce symptoms or manifest pathological findings until adult life

• Findings with imaging procedures are large kidneys with multiple fluid filled spherical cysts of varying sizes.

• Ultrasound is the preferred diagnostic imaging procedure

• Diagnostic information can also be obtained from flat plate, IVP, CT and MRI

Pathology:

• Grossly enlarged kidneys with multiple cysts studding the surface of kidneys
• Cysts are spherical of varying sizes and contain straw colored fluid
• Cysts are distributed throughout cortex and medulla

Ultrasound characteristics

• Progressive appearance and increase in size with age

• Large Kidneys with multiple, bilateral, fluid filled cysts of varying sizes

• At least 3-5 cysts

CT Characteristics

• More sensitive than ultrasound in detection of small cysts

Extra renal manifestations

• May have associated cysts in liver, pancreas and spleen
• Aneurysms in the region of circle of Willis may be present
• Chronic Diverticular disease
• Mitral valve prolapse

 Complications

• Hemorrhage
  • When cysts appear dense consider infection, hemorrhage or neoplasm
    • Case with dense cyst due to neoplasm : Contrast CT  CT   CT   Delayed Scan
• Nephrolithiasis occurs in 15-20%
• Chronic renal failure

ADPKD             Renal cancer          MSK