A 55-year-old man with autosomal dominant polycystic kidney disease (eGFR 34 mL/min) presents with sudden severe headache and photophobia. CT head shows subarachnoid haemorrhage. Which extrarenal manifestation is MOST strongly associated with this condition?

• A Hepatic cysts
• B Intracranial berry aneurysm ✓
• C Mitral valve prolapse
• D Colonic diverticulae

Explanation

ADPKD is associated with intracranial berry aneurysms in approximately 8% of patients, which can rupture causing subarachnoid haemorrhage. This is the most clinically significant extrarenal association because of its life-threatening potential. Hepatic cysts are the most common extrarenal manifestation overall but are usually benign. Mitral valve prolapse and colonic diverticulae are also seen but carry less acute mortality risk.

Reference: Brenner and Rector's The Kidney, 11th ed.

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