A 7-year-old girl with a previous diagnosis of Parry-Romberg syndrome presented with acute headache (►Figures 1 and 2). Parry-Romberg syndrome is a subtype of localized scleroderma of the head that, although rare, may be associated with giant intracranial aneurysms.1 The etiology of these aneurysms is still poorly understood; however, it is believed that it may be related to endothelial inflammatory injury or vasa vasorum microangiopathy and vascular wall ischemia.1,2 Dissecting pseudoaneurysm of intracranial arteries is rare and may result in acute headache and neurological deficits.1,2 The characterization of mural enhancement in vessel wall magnetic resonance imaging (MRI) in this case corroborated the hypothesis of a vasculitis-related etiology.
Volume-rendered time-of-flightangiography. Thesuperior view depicts the dissecting pseudoaneurysm of the basilar artery (arrow) and a right internal carotid artery aneurysm (arrowhead).
Vessel Wall MRI. Precontrast (A), postcontrast (B), and image fusion (C) depict the dissecting pseudoaneurysm of the basilar artery composed of a large subadventitial hematoma (asterisks) associated with inflammatory changes (short arrows). Note the circumferential wall enhancement of the basilar artery causing stenosis (long arrows) and right internal carotid artery aneurysm (arrowheads).
References
- 1 Bosman T, Van Bei Jnum J, Van Walderveen MAA, Brouwer PA. Giant intracranial aneurysm in a ten-year-old boy with parry romberg syndrome. A case report and literature review. Interv Neuroradiol 2009;15(02):165–173
- 2 Kuechler D, Kaliaperumal C, Hassan A, Fanning N, Wyse G, O’Sullivan M. Aneurysmal subarachnoid haemorrhage in Parry-Rhomberg syndrome. BMJ Case Rep 2011;2011: bcr1020114920
Publication Dates
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Publication in this collection
21 Nov 2022 -
Date of issue
July 2022
History
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Received
20 Dec 2021 -
Accepted
06 Feb 2022