Screening for cerebral aneurysm in patients with polycystic liver disease

Background. Polycystic liver disease (PCLD) is an autosomal dominant diseas e Characterized by multiple macrocystic lesions throughout the liver. The a ssociation between PCLD and cerebral aneurysm is well documented, and appro ximately 20% of patients with PCLD have demonstrable cerebral aneurysms at autopsy. The prevalence reported from autopsy series, however, may not refl ect the true prevalence in patients with PCLD. We undertook this study to e valuate the prevalence and diagnosis of cerebral aneurysms in screening cer ebral studies in patients with PCLD. Methods. Patients were identified by searching the hepatobiliary surgical s ervice data base and hospital medical records. Hospital charts were reviewe d to confirm presence of PCLD and to identify screening studies for cerebra l aneurysms. Results. Ten patients with PCLD received screening studies of the cerebral vasculature during a 10 1/2-year period. One patient was found to have an a symptomatic cerebral aneurysm. A 45-year-old woman with no other significan t medical history was referred for evaluation of PCLD, Screening magnetic r esonance angiography (MRA) revealed a 5 mm aneurysm extending anteriorly ne ar the origin of the right ophthalmic artery, without evidence of rupture. Cerebral angiography confirmed these findings, and the aneurysm was clipped . Conclusions. Because cerebral aneurysms can be an important source of morbi dity and mortality in PCLD, we recommend screening by MRA or by computed to mographic angiography (CTA) of the cerebral vasculature in all patients who have PCLD.