Intracranial fibromuscular dysplasia and stroke in children

Background: The prevalence of cerebral aneurysms is increased in fibromuscular dysplasia (FMD). The presence of FMD may serve as discouragement to elective endovascular aneurysm treatment. Outcomes of endovascular intervention for aneurysms through vessels affected by FMD have not been reported. Methods: A prospectively maintained database of patients undergoing intracranial embolization was reviewed for patients with FMD who underwent endovascular aneurysm treatment. Results: A total of 1,025 patients were screened and 31 (3.0%) had cerebrovascular FMD. These patients underwent a total of 43 embolization procedures; 27 of these procedures were performed through an affected vessel. All but 1 patient were female and the average age was 62 years. “String-of-pearls”-type FMD was the most common subtype (90%). The internal carotid arteries were more commonly affected (65%) than the vertebral arteries (48%). All patients underwent treatment of cerebral aneurysms, most of which (87%) were incidentally discovered; 6 patients (19%) also had incidental vessel dissection. The average aneurysm size was 7.1 mm. The morphology was saccular in 93% of the cases, and 86% were in the anterior circulation. The most commonly performed treatment was flow diversion (67%), in the majority of cases by pipeline embolization. Other procedures performed were coiling (19%), stent-coiling (12%), and intrasaccular flow disruption (2%). All but 1 procedure (98%) were successful. There were no major complications; 1 patient experienced a transient ischemic attack. Follow-up angiography was performed in 88% of the cases, without evidence for disease progression after treatment. The average time to last angiographic follow-up was 17 months (±13). Conclusions: Elective embolization of intracranial aneurysms can be performed safely through vessels affected by FMD.

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“…They were predominantly middle-aged, female, and white [18,19]. Most patients had more than one vessel affected.…”

Section: Discussionmentioning

confidence: 99%

Safety Assessment of Endovascular Treatment of Cerebral Aneurysms in Patients with Fibromuscular Dysplasia

et al. 2017

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“…Ectasia may affect only one part of the circumference and short smooth strictures, tubular stenoses and oc clusions may occur -appearances which may simulate atheroma. The disease is generally multifocal and typically involves the upper cervical segments o f the internal carotid and vertebral arteries where atheroma is unusual; the external carotid and its branches and rarely the intracranial vessels may be affected [13]. Infraradiological disease may be dem onstrated at autopsy and in angiographically normal vessels [16].…”

Section: Discussionmentioning

confidence: 99%

Cerebral Angiography in Vasculitis Affecting the Nervous System

Kendall¹

1984

Eur Neurol

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Of 199 patients with clinico-laboratory evidence of vasculitis, 42 were submitted to cerebral angiography; 35 angiograms were abnormal, but only 21 were characteristic of the particular disease process. A pattern consistent with vasculitis diagnosed in 19 angiograms, was due to neoplasm in 4 and neurofibromatosis, DXT and amphetamines each in 1 case. Moya-Moya type collaterals shown in 20 angiograms were due to the idiopathic disease in 10 but due to non-inflammatory pathologies in 7. Fibromuscular dysplasia shown in 28 angiograms was an incidental finding in 20 of these. Of 56 cerebral angiograms for ischaemic stroke in patients under 15 years old, 17 were normal and only 8 vascular lesions were likely to have been of inflammatory origin. In routine clinical practice cerebral angiography, though essential in the management of mycotic aneurysm and in the diagnosis of Moya-Moya disease isolated vasculitis and fibromuscular dysplasia, plays only a limited role in the diagnosis of other types of vasculitis.

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“…In cervicocephalic FMD, the vessel most commonly affected is the extracranial internal carotid artery, and the average patient age at diagnosis is 50 years. On the other hand, a rare variant of intracranial FMD, which is detected mainly in children and young adults, causes cerebral ischemia and bleeding [3]. Intracranial aneurysms occur in 25–50% of patients with cervicocephalic FMD, suggesting that FMD is associated with a predisposition to aneurysm formation [4].…”

Section: Introductionmentioning

confidence: 99%

Multiple Intracranial Aneurysms with Intraventricular Hemorrhage in a Child with Unilateral Fibromuscular Dysplasia of the Renal Artery

Park

Kang²,

Lim³

et al. 2008

Pediatr Neurosurg

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A rare case involving multiple intracranial aneurysms with intraventricular hemorrhage (IVH) in a child, accompanied by unilateral fibromuscular dysplasia (FMD) of the renal artery, is reported. A 12-year-old girl presented with secondary hypertension and fourth-ventricle IVH. Cerebral angiography showed multiple aneurysms in the anterior communicating artery, right posterior cerebral artery, and right superior cerebellar artery. Abdominal angiography revealed marked stenosis and dilatation of the left renal artery. Coil embolization of the intracranial aneurysms was successfully performed. Then, a left nephrectomy and autotransplantation were performed. At the time of discharge, she was in an almost normal neurological condition. The association between renal artery FMD and aneurysmal rupture is extremely rare. This case is the first report of multiple intracranial aneurysms with IVH treated successfully with coils in a child concomitant with FMD of the renal artery.

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Intracranial fibromuscular dysplasia and stroke in children

Cited by 32 publications

References 19 publications

Safety Assessment of Endovascular Treatment of Cerebral Aneurysms in Patients with Fibromuscular Dysplasia

Safety Assessment of Endovascular Treatment of Cerebral Aneurysms in Patients with Fibromuscular Dysplasia

Cerebral Angiography in Vasculitis Affecting the Nervous System

Multiple Intracranial Aneurysms with Intraventricular Hemorrhage in a Child with Unilateral Fibromuscular Dysplasia of the Renal Artery

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