[HTML][HTML] Bilateral renal artery stenosis and epidermal nevus syndrome in a child

F Alsohim, P Abou-Jaoude, J Ninet, JP Pracros… - Pediatric …, 2011 - Springer
F Alsohim, P Abou-Jaoude, J Ninet, JP Pracros, A Phan, P Cochat
Pediatric Nephrology, 2011Springer
Epidermal nevus syndrome is a rare congenital sporadic neuro-ectodermic disorder,
characterized by the presence of epidermal nevi in association with various developmental
abnormalities of the skin, eyes, nervous, skeletal, cardiovascular and urogenital systems.
We describe a 5-year-old boy with conjunctival lipodermoid, cervical and facial sebaceous
nevi who presented at 3 years of age with hypertension due to bilateral renal artery stenosis
together with multiple vascular anomalies (aorta, celiac trunk, superior mesenteric artery) as …
Abstract
Epidermal nevus syndrome is a rare congenital sporadic neuro-ectodermic disorder, characterized by the presence of epidermal nevi in association with various developmental abnormalities of the skin, eyes, nervous, skeletal, cardiovascular and urogenital systems. We describe a 5-year-old boy with conjunctival lipodermoid, cervical and facial sebaceous nevi who presented at 3 years of age with hypertension due to bilateral renal artery stenosis together with multiple vascular anomalies (aorta, celiac trunk, superior mesenteric artery) as shown by magnetic resonance angiography. Systemic arterial hypertension was difficult to control despite combined anti-hypertensive drugs and the surgical repair of the aortic coarctation.
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