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Intracranial fibromuscular dysplasia in a 5-year-old child
N C Chiu1, G R DeLong, E R Heinz
1Department of Pediatrics, Duke University Medical Center, Durham, North Carolina 27710, USA.
Insights
This case report details a rare instance of fibromuscular dysplasia in a young child, leading to a stroke. The patient recovered well with supportive care, highlighting an unusual presentation of this vascular condition.
Area of Science:
- Neurology
- Vascular Medicine
- Pediatrics
Background:
- Fibromuscular dysplasia (FMD) is a non-atherosclerotic, non-inflammatory vasculopathy.
- It commonly affects the renal and carotid arteries, but intracranial involvement is less frequent, particularly in children.
Observation:
- A 5-year-old girl presented with an acute stroke in the middle cerebral artery.
- Angiography revealed focal changes in the right middle cerebral artery, dilatation of the right internal carotid artery, and dissection of middle cerebral artery branches.
Findings:
- The patient was diagnosed with intracranial fibromuscular dysplasia.
- Management focused on intracranial pressure; no specific medications were administered post-discharge.
- The patient experienced a remarkable recovery with minimal residual effects.
Implications:
- This case highlights an unusual age of onset and location for intracranial fibromuscular dysplasia.
- It underscores the importance of considering FMD in pediatric stroke evaluations.
- Further research into pediatric intracranial FMD is warranted to understand its unique characteristics and management strategies.
Abstract:
A 5-year-old girl presenting with acute middle cerebral artery stroke was diagnosed as having intracranial fibromuscular dysplasia by angiographic findings of focal changes in the proximal right middle cerebral artery and pathological dilatation of the right internal carotid artery at the base of the skull, as well as dissection of at least one of the middle cerebral artery branches and nonfilling of two or perhaps three remaining middle cerebral artery segmental branches. Her clinical condition improved after management of the increased intracranial pressure. She did not receive any medication after discharge and had virtually no residual sequelae. Our case documents an unusual location and age of onset of a patient with fibromuscular dysplasia.
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