Renovascular hypertension in children with neurofibromatosis type 1

Abstract

Arterial hypertension in pediatric patients with neurofibromatosis type 1 (NF 1) is usually due to renal artery stenosis (RAS) mainly involving the proximal part of the vessel. The treatment modalities are highly individualized. In severe and/or bilateral RAS, antihypertensive drugs are either ineffective or have the potential risk for acute renal failure, while percutaneous transluminal angioplasty (PTA) has limited success due to the ostial localization of RAS and the tough fibrotic tissue involved that is refractory to dilatation Renal autotransplantation has potential advantages when medical control and PTA/or bypass techniques failed. Here we report 5 year-old girl with NF 1 and hyponatremic hypertensive syndrome due to severe bilateral disease, occluded proximal part of the right artery and ostial stenosis (80%) of the left one. Only left kidney was identified on 99 in Tc DTP A, but the right one was visualized on the renal ultrasonography and in the late phase of arterial renography due to well developed collateral circulation. Multiple antihyper-tensive drugs (nifedipine, labetolol and minoxidil) in maximal doses and PTA failed to normalize BP while short term therapy with ACEIwith NF1 and hyponatremic hypertensive syndrome due to severe bilateral renovascular disease; occluded proximal part of the right renal artery and ostial stenosis (80%) of the left one. Only left kidney was identified on 99m Tc DTPA, but the right one was visualized on the renal ultrasonography and in the late phase of arterial renography due to well developed collateral circulation. Multiple antyphypertensive drugs (nifedipine, labetolol and minoxidil) in maximal doses and PTA failed to normalize BP while. short term therapy with ACEI, captopril induced transient acute renal failure. Autotransplantation of right kidney saved its function and improved BP control. Our current case Autotransplantation of right kidney saved its function and improved BP control. Our current case is illustrative for a difficult management of renovascular hypertension in children with NF1. This is the first and up to now the only case of autotransplantation performed in Yugoslavia.