Bilateral renal artery stenosis and epidermal nevus syndrome in a child
- نوع فایل : کتاب
- زبان : انگلیسی
- مؤلف : Fahad Alsohim & Pauline Abou-Jaoude & Jean Ninet & Jean-Pierre Pracros & Alice Phan & Pierre Cochat
- چاپ و سال / کشور: 2011
Description
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.
Pediatr Nephrol DOI 10.1007/s00467-011-1949-z Received: 14 December 2010 / Revised: 25 May 2011 / Accepted: 31 May 2011
