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[Moyamoya disease associated with kidney angiodysplasia in a child]
A Dibi1, Z Maana1, F Jabourik1
1Service de pédiatrie IV, hôpital d'Enfants de Rabat, université Mohamed V, CHU Ibn Sina, boulevard Ibn Rochd, 10100 Rabat, Maroc.
Insights
Moyamoya disease, a rare cause of childhood stroke, can be linked to renal angiodysplasia, potentially causing severe hypertension. This case highlights the need for comprehensive care for affected children.
Area of Science:
- Neurology
- Pediatrics
- Vascular Medicine
Background:
- Moyamoya disease is a progressive occlusive vascular disorder affecting the circle of Willis arteries.
- It leads to the formation of collateral circulation to compensate for arterial blockages.
Observation:
- An 11-year-old girl experienced sudden loss of consciousness and right-sided motor deficits.
- Clinical findings included severe hypertension (220/120mmHg) and a large left hemispheric ischemic lesion on MRI.
- Cerebral arteriography revealed a characteristic "wisp of smoke" appearance indicative of moyamoya disease.
Findings:
- The patient presented with moyamoya disease, a rare cause of stroke in children.
- Renal arteriography identified dysplasia in the upper polar branches of the right kidney.
- The association between moyamoya disease and renal angiodysplasia is unusual and may contribute to malignant hypertension.
Implications:
- This case underscores the rare association of moyamoya disease with renal angiodysplasia and its potential to cause malignant hypertension in pediatric patients.
- Recognized long-term issues include cognitive impairment and social dependence, necessitating integrated medical, surgical, social, and psychological support.
- Early diagnosis and multidisciplinary management are crucial for improving outcomes in children with moyamoya disease.
Introduction:
Moyamoya disease is a progressive, chronic occlusive vascular disease of the circle of Willis arteries leading to the development of collateral circulation to compensate the occlusion.
Case Report:
An 11-year-old girl presented an abrupt loss of consciousness and a right-sided motor deficit. Clinical examination found hypertension with 220/120mmHg arterial blood pressure. Brain magnetic resonance imaging showed a large left hemispheric ischemic lesion. Cerebral arteriography demonstrated an abnormal anastomotic vascular network with fine arteriolar ends in several territories, with a "wisp of smoke" aspect arguing in favor of moyamoya disease. Renal arteriography revealed dysplasia of the upper polar branches of the right kidney.
Conclusion:
Moyamoya disease is a rare cause of stroke in children. Its association with renal angiodysplasia is unusual and may be responsible for malignant hypertension. Cognitive impairment and social dependence have recently been recognized as an important unresolved social issue. Affected children require medical-surgical, social, and psychological care.
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