Atypical aortic coarctations in type I neurofibromatosis

: From 1971 through 1996, 16 patients were operated on for atypical aortic coarctation. Three of them (two women, 18 and 27 years old, one boy, 13 years old) had typical signs of neurofibromatosis type I (NF1). All had renovascular hypertension due to suprarenal and/or interrenal aortic stenosis. Two had additional bilateral renal artery stenoses, one with a poststenotic aneurysm. In all patients a thoraco-abdominal aorto-aortic bypass was implanted. The renal artery stenoses were bridged by two aorto-renal, a prothesio-renal, and a spleno-renal bypass. Only one kidney had to be explanted 13 years after revascularization for a recurring hilar aneurysm after ex-situ reconstruction was found to be impossible. At their last follow-up examination (at 16, 14, and 9 years) all patients were normotensive without medication and had normal serum-creatinine measurements. Angiography or spiral-CT done in all patients showed normal function of all bypasses. In literature we found another 27 patients with neurofibromatosis operated on for atypical aortic coarctation. In these young and otherwise healthy patients, even complex reconstructions can be performed with a low rate of complications and excellent long-term results. Surgical therapy of all relevant stenoses (at best in a single procedure) is necessary for complete relief of renovascular hypertension. Consequent follow-up is advised for the detection of possible late complications, especially after exclusion of aneurysms.