A rare cause of ischemic stroke: fibromuscular dysplasia

Abstract

Childhood ischemic stroke is uncommon and may be associated with many causes and require extensive evaluation. Fibromuscular dysplasia is a rare cause of unknown etiology of childhood stroke which is mostly related with renovascular hypertension and in adults about 85% of cases renal artery has been involved, whereas the intracerebral circulation is the main area affected in children and the documented cause of stroke. We report a 4-year-old girl who presented with facial paralysis and diagnosed as intracranial fibromuscular dysplasia without renal artery involvement.

Fibromuscular Dysplasia: A Rare Case of Renal Artery Stenosis Presenting with Stroke

“…The clinical presentation may vary from an asymptomatic condition to multiorgan disease including renovascular HT, AIS, cranial hemorrhage due to arterial dissection and abdominal pain or claudication. The disease can mimic vasculitis such as polyarteritis nodosa, giant cell arteritis or Takayasu arteritis (1,5). Our patient manifested with the symptoms of arterial insufficiency of brain and arterial occlusion of kidney.…”

“…Pediatric stroke is a rare condition with an incidence of 0.6 to 7.9 per 100.000 children per year (1,(7)(8)(9). Furthermore, pediatric stroke is rare but a significant cause of short-and long-term morbidity and mortality in children.…”

“…According to the clinical condition of the patient, anticoagulation and antiplatelet therapy as well as percutaneous angioplasty are the treatment of choice. Percutaneous angioplasty, anticoagulation and antiplatelet therapies are especially recommended for patients with transient ischemic attack or stroke (1). The goal of treatment is the control of BP and preventing the complications associated with HT in FMD patients.…”

“…This entity is rarely seen in children (1). The estimated incidence of childhood arterial ischemic stroke associated with renal arteriopathy and FMD is approximately 1:3000000 per year (2).…”

“…The estimated incidence of childhood arterial ischemic stroke associated with renal arteriopathy and FMD is approximately 1:3000000 per year (2). This condition is typically associated with stenotic lesions in the renal, cerebral and cardiac arteries causing a characteristic "string of beads" appearance on angiography (1,(3)(4)(5). The most commonly described form of FMD is medial fibroplasia/dysplasia typically affecting young adult females with renal and cerebral vessel involvement, refractory hypertension (HT) and stroke.…”