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.
Figures 1. Volume-rendered time-of-flightangiography. Thesuperior view depicts the dissecting pseudoaneurysm of the basilar artery (arrow) and a right internal carotid artery aneurysm (arrowhead).
Figure 2. 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
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