Intra-arterial vasodilators infusion for management of reversible cerebral vasoconstriction syndrome in a 12-year-old girl: A case report

Abstract

Reversible cerebral vasoconstriction syndrome (RCVS) is a vascular disease characterized by diffuse transient vasoconstriction and vasodilatation of the cerebral arteries. It is commonly associated with recurrent severe acute headaches with or without focal neurological deficits due to hemorrhages, infarcts, and even posterior reversible encephalopathy syndrome. The optimal management of acute neurologic deficits caused by RCVS is still uncertain. Calcium channel blockers (CCBs) such as nimodipine or verapamil have been reported to be effective in adult series. Intra-arterial injection of nimodipine, verapamil, and milrinone has recently been demonstrated to be safe and effective for treating severe segmental vasoconstriction in adults. CCBs are the most used treatment in the available pediatric literature. Intra-arterial vasodilators have been reported in some rare pediatric reports with more severe diseases, but their utility is still under investigation. We report a case of a 12-year-old girl who underwent a severe course of RCVS complicated by multiple cerebral infarcts, treated by several sessions of intra-arterial vasodilators infusion.

Keywords: case report; children; headache; pediatric intensive care unit; reversible cerebral vasoconstriction syndrome; vessel wall MRI.

Figure 1

Day 4 MRI with TOF sequence (A) and 3D SPACE T1 imaging of the vessel wall before (B) and after gadolinium injection (C). The reconstruction of (B) and (C) images follows the dotted line on the TOF sequence (A: dotted line). For easy identification, the internal carotid artery is surrounded by a dotted circle on (B) and (C). On the TOF sequence, the right internal carotid artery is occluded and the right anterior circulation is supplied downstream by the right PComm and AComm. On vessel wall imaging, there is a spontaneous marginalized and asymmetric hyperintense signal (B, arrow) that enhances after injection of gadolinium, without enhancement of the vessel wall itself (C), highly indicative of a wall hematoma due to focal intradural dissection of the right internal carotid artery. MRI, magnetic resonance imaging; PComm, posterior communicating artery; AComm, anterior communicating artery.

Figure 2

Subtracted DSA on day 12 before any vasodilator infusion (A) and after the first vasodilator infusion (B). The cerebral arteries showed diffuse and severe stenosis (A, arrows), which were improved after the first intra-arterial vasodilator infusion. In particular, blood flow reappeared in a pericallosal branch (B, arrow). On the last follow-up DSA, after the last vasodilator infusion 10 days after the first DSA, the stenosis had almost disappeared with a near-normal appearance of the cerebral arteries (C). DSA, digital angiography.