Ruptured intracranial aneurysm in an adolescent with Alport's syndrome - Anew expression of type IV collagenopathy: Case report

BACKGROUND Aneurysmal subarachnoid hemorrhage is uncommon in the pediatric and adolescent age groups. Collagenopathies, particularly of collagen type III and IV, have been recognized in the pathogenesis of intracranial (i.c) aneurysms in recent studies of cerebrovascular immunoreactivity and the mol ecular architecture of the walls of cerebral blood vessels. Alport's syndro me is a genetic disease characterized by imperfect synthesis of type IV col lagen leading to sensorineural hearing loss and hematuria. The hematuria re sults from an abnormal synthesis of the type TV collagen of the glomerular basement membrane. CASE DESCRIPTION After a motor vehicle accident, this 14-year-old male driv er presented with diffuse subarachnoid hemorrhage confirmed by computerized tomography. Subsequent cerebral angiography revealed a left carotid artery bifurcation aneurysm. A retrospective review of the patient's history disc losed renal biopsy-proven hereditary Alport's syndrome. The patient underwe nt left pterional craniotomy and clipping of the aneurysm, which had clearl y ruptured. CONCLUSIONS The authors present the first clinical observation of a rupture d cerebral aneurysm in an adolescent male with Alport's syndrome. In this c ase the type IV collagenopathy of Alport's syndrome may have contributed to the genesis of this adolescent's aneurysm. Intracranial aneurysms may be m ore common in the population of collagenopathies than previously suspected and non-invasive screening may be appropriate. (C) 2000 by Elsevier Science Inc.