Parry Romberg syndrome presenting with a giant intracranial aneurysm: A case report

Abstract

A giant intracranial aneurysm was diagnosed in a 10-year-old girl when she developed a right abducens nerve palsy. The aneurysm was treated successfully. Six years later, however, she presented with a progressive en coup de sabre deformity, leading to the diagnosis Parry Romberg Syndrome (PRS), a rare diagnosis characterized by hemifacial atrophy of skin, subcutaneous tissue, skeletal muscle and bones and often associated with various non-specific intracerebral abnormalities. In this patient retrospective analysis of computed tomography and magnetic resonance imaging imaging indeed revealed intracerebral calcifications and aspecific white matter lesions. Remarkably, in this patient the giant intracranial aneurysm turned out to be the presenting symptom of PRS years before the characteristic facial deformities became apparent.