Question

Adult polycystic kidney disease (PCKD) is associated with which of these?

• A. Fusiform abdominal aortic aneurysm
• B. Berry aneurysm
• C. Saccular aneurysm of ascending aorta
• D. Aneurysm of the arch of aorta

Hint:

ADPKD's weak vessel walls show up most clearly as circle of Willis (berry) aneurysms, not aortic aneurysms.

Answer 1

The Correct Option is B

Autosomal dominant polycystic kidney disease (ADPKD, the adult form of PCKD) is not just a kidney condition, it comes with several extrarenal associations, and one of the best known is a specific type of blood vessel abnormality in the brain.

1. Fusiform abdominal aortic aneurysm: Not the classic association. Aortic aneurysms are linked more to atherosclerosis and connective tissue disorders, not specifically to ADPKD.
2. Berry aneurysm: This is the well known association. ADPKD patients have a higher rate of saccular (berry) aneurysms in the circle of Willis at the base of the brain, because the vessel wall defect that weakens the kidney tubules also weakens cerebral artery walls. Rupture of one of these causes a subarachnoid hemorrhage, which is why ADPKD patients with a family history of such bleeds are screened with MR angiography.
3. Saccular aneurysm of ascending aorta: Not a recognized feature of ADPKD.
4. Aneurysm of the arch of aorta: Also not a recognized feature of ADPKD.

Cysts in ADPKD can also form in the liver, pancreas and spleen, and patients can have mitral valve prolapse and colonic diverticula, but the single best known vascular association tested here is the berry aneurysm.

Let's summarize:

• ADPKD weakens vessel walls generally, and this shows up most clearly as berry aneurysms in the brain.
• Aortic aneurysms (fusiform or saccular, ascending or arch) are not the classic ADPKD association.

So the correct answer is berry aneurysm.