CREST-associated ruptured right MCA aneurysm: A case report and review of the literature

Intracranial aneurysms associated with systemic sclerosis and Calcinosis, Raynaud’s phenomenon, Esophageal dysmotility, Sclerodactyly, and Telangiectasia (CREST) syndrome are rare, and their pathophysiological relationship remains uncertain. We report the case of a 24-year-old male with systemic sclerosis and clinical features suggestive of CREST syndrome who presented with subarachnoid hemorrhage caused by a ruptured right middle cerebral artery bifurcation aneurysm. The aneurysm was treated with microsurgical clipping, followed by stent-assisted coiling for a residual aneurysm neck identified on follow-up angiography. The patient had a favorable neurological outcome with mild persistent cognitive deficits. A review of the literature identified only a small number of reported intracranial aneurysm cases associated with systemic sclerosis or CREST syndrome. This case differs from previously reported CREST-associated cases in patient age, sex, and aneurysm morphology, and highlights the possible contribution of connective tissue disease, smoking, and prior amphetamine exposure to aneurysm formation and rupture.