Management of renal vascular disease in neurofibromatosis type 1 and the role of percutaneous transluminal angioplasty.

BACKGROUND The effectiveness of percutaneous transluminal angioplasty (PTA) in renal artery stenosis (RAS) associated with neurofibromatosis type 1 (NF1) has not been established. A review of the literature revealed 14 studies, mainly case reports, of which a total of 16 patients had been treated with PTA, all prior to any surgery being undertaken. METHODS A retrospective case review of 10 children with renal vascular disease secondary to NF was conducted to evaluate the outcome of different treatment modalities, including PTA. Four patients had unilateral disease, while six had bilateral disease, which was associated with middle aortic syndrome in three. Treatment was a combination of antihypertensive drug therapy, PTA and surgery. PTA was performed 15 times on six patients, nine procedures prior to surgery. RESULTS The success rate for PTA on primary stenoses was 33% with improvement in blood pressure (BP) control in another 33%. Angioplasties performed on stenoses post-surgery had a success rate of 67%. There were no major complications and PTA had no adverse effect on subsequent surgery. The pre-surgery results obtained at a tertiary referral centre are consistent with those reported in the literature. CONCLUSION PTA is a safe and moderately effective treatment modality for RAS secondary to NF1. Although there is only limited success in primary stenoses as there is no adverse effect on subsequent surgery we feel it should be considered as first line management when clinically indicated.