SESSION TITLE: Cardiovascular Disease Global Case Reports
SESSION TYPE: Global Case Report Poster
PRESENTED ON: Tuesday, October 27, 2015 at 01:30 PM - 02:30 PM
INTRODUCTION: 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. In the literature, the outcomes of percutaneous transluminal angioplasty is known to be poor in other neuro-ectodermic diseases such as neurofibromatosis,
CASE PRESENTATION: 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.
DISCUSSION: Bilateral renal artery stenosis is an unusual vascular malformation associated with epidermal nevus syndrome. A percutaneous transluminal angioplasty was not attempted because of known poor results in other neuro-ectodermic diseases such as neurofibromatosis, and also because of bilateral involvement with a risk of thrombosis and further renal damage compared with acceptable control of blood pressure by medical therapy. The overall therapeutic attitude was made with the motto “primum non nocere” as long as the situation is stable for several years. The optimal treatment strategy for renal artery stenosis remains unclear. There are no randomized clinical trials comparing the effects of angioplasty stenting versus medical therapy alone in such patients
CONCLUSIONS: we need randomized clinical trials comparing the effects of angioplasty stenting versus medical therapy alone in patients with renal artery stenosis.
Reference #1: Solomon LM, Fretzin DF, Dewald RL. The epidermal nevus syndrome. Arch Dermatol. 1968;97:273-285. doi: 10.1001/archderm.97.3.273
Reference #2: Grebe TA, Rimsza ME, Richter SF, Hansen RC, Hoyme HE. Further delineation of the epidermal nevus syndrome: two cases with new findings and literature review. Am J Med Genet. 1993;47:24-30. doi: 10.1002/ajmg.1320470106
Reference #3: Booth TN, Rollins NK. MR imaging of the spine in epidermal nevus syndrome. AJNR Am J Neuroradiol. 2002;23:1607-1610
DISCLOSURE: The following authors have nothing to disclose: Fahad Alsohime
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