Abstract:

Neurofibromatosis type 1 (NF1) is an autosomal dominant disorder is characterized by various clinical manifestations as a result of dysplasia of neuroectodermal and mesodermal tissues. Cafe-au-lait spots, axillary and inguinal freckling, cutaneous neurofibromas with a variable clinical expression, iris Lisch nodules, and multiple tumors of central and peripheral nervous system, as well as other systems may be involved. NF1 patients develop hypertension due to renovascular diseases, mid-aortic syndrome, or pheochromocytoma. In pediatric NF1 patients elevated blood pressure is usually due to renal artery stenosis, generally involving the origin or the proximal tract of the vessel, and is associated in 25% of patients with coarctation of the abdominal aorta. Several patients with NF1 and hypertension have been reported in the literature, showing the extreme variability in anatomical lesions. Although NF1 is a rare disease, ıt is an important cause of renovasculer hypertension in childhood.Abdominal bruit is an alerting physical finding in diagnosis of renal artery stenosis. We present two cases with NF1 developed hypertension.Our cases emphasises the need for blood pressure measurement in children with NF1 screening for stenosis in the major branch vessels of the aorta in children with NF1 and renal artery stenosis is required in hypertensive NF1 patients.

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