• World Neurosurg · Sep 2020

    Review Case Reports

    Scleroderma's possible dual role in the pathophysiology of intracranial aneurysms:Case report and literature review.

    • Roland Jabre, Anass Benomar, and Michel W Bojanowski.
    • Division of Neurosurgery, Centre Hospitalier de l'Université de Montréal (CHUM), Montréal, Québec, Canada.
    • World Neurosurg. 2020 Sep 1; 141: 267-271.

    BackgroundScleroderma, or systemic sclerosis, is an autoimmune disorder affecting connective tissues, including blood vessels. Although the exact mechanism is not understood, it results in the production of an abnormal amount of collagen. Cases have been reported in which patients with scleroderma also had intracranial aneurysms. We wish to gain insight into any potential association between the 2 diseases.Case DescriptionWe reviewed the literature of scleroderma cases with cerebral aneurysms and added our own case, focusing on patient and aneurysm characteristics. Including the present case, this paper pertains to 11 cases with 26 aneurysms. Fifty-five percent had multiple aneurysms, of which two thirds had >2. When data were available, 35% of aneurysms were ≥1 cm, 12% of which were giant. Four aneurysms were fusiform (15%). Patients presented with subarachnoid hemorrhage in 45% of cases.ConclusionsScleroderma may lead to the formation of an aneurysm, and the abnormal growth of collagen may protect it from early rupture, allowing it to reach a large size.Copyright © 2020 Elsevier Inc. All rights reserved.

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