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

Abstract

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.

Diagnostic and medical strategy for renovascular hypertension: report from a monocentric pediatric cohort

“…The best long-term results are obtained when the lesion is short (less than 10 mm), in the middle of the renal artery, isolated, and due to FMD, with an initial residual stenosis of less than 10 % [4,7,8,19,31]. However, in other cases, the success of this technique is less than 30 % [4,10,19].…”

“…We propose that tomography is performed for severe cases, which are defined by a young age, NF1 disease, multiple lesions, or mid-aortic syndrome detectable by Doppler. NF1 was considered as a severity criterion because of the importance of identifying lesions, which are often present as multiples and located at the ostia before to perform a PTA reputed to be difficult [4,10,19]. Likewise, younger ages were considered as a criterion of severity because of the difficulty in performing an accurate Doppler at this age and due to the young age of mid-aortic syndrome in our cohort.…”

Retinal Abnormalities Characteristic of Inherited Renal Disease

“…Hypertension occurs because of proximal renal artery stenosis, coarctation of the aorta, or phaemochromocytoma. 84 Retinal abnormalities include ischemia, 85 hamartoma including Combined Hamartoma of the Retina and Retinal Pigment Epithelium (CHRRPE, both NF1 and NF2 86 ), optic glioma (NF1), and optic atrophy secondary to pressure on the optic nerve.…”

Revascularization in a 17-Year-Old Girl with Neurofibromatosis and Severe Hypertension Caused by Renal Artery Stenosis

“…2 Renal artery stenosis typically causes the hypertension; other causes are aortic coarctation and pheochromocytoma. 6 Although vascular abnormalities such as stenoses, aneurysms, and arteriovenous malformations are frequently found in patients with NF1…”