Ruptured intracranial aneurysm and recessive polycystic kidney disease : a rare association

Abstract EN

Autosomal recessive polycystic kidney disease (ARPKD) is the most common childhood-onset ciliopathy.

Intracranial aneurysms (ICA) are a serious complication of autosomal dominant polycystic kidney disease (ADPKD).

However, there are only three reports about ICA in an adult patient with ARPKD.

We describe a rare case of a 29-year-old man with ARPKD presenting with subarachnoid hemorrhage secondary to a ruptured intracranial aneurysm.

The diagnosis of ARPKD was at the age of eight years based on typical ultrasonography findings with polycystic kidneys and liver disease.

Magnetic resonance cholangiography showed a nonobstructive dilatation of intrahepatic bile ducts.

Liver biopsy showed hepatic fibrosis.

None of the family members was affected.

At the age of 15 years, he had progressed to end-stage kidney disease, and hemodialysis was started.

The patient had always a severe arterial hypertension.

At the age of 29 years, he complained of headaches with an uncontrolled hypertension and disturbance of consciousness, computed tomography angiography showed subarachnoid hemorrhage and multiple cerebral aneurysms.

Early neurologic screening of intracranial aneurysm should be recommended in ARPKD like in ADPKD patients.