[High blood pressure due to aortic coarctation and renal artery stenosis in a teenager with type 1 neurofibromatosis].
Hypertension affect about 1% of patients with neurofibromatosis type 1 (NF1). Major causes are concomitant pheocromocytoma in adults and renovascular hypertension in children. In most cases, NF1 is associated with renal artery stenosis, smooth cell proliferation and advential fibrosis. We describe a 16 year old girl with hypertension complicating NF1 secondary to severe coarctation of abdominal aorta and tight stenosis of right renal artery, a very uncommon case. She was first diagnosed when she was 3-years-old and managed with antihypertensive drugs (atenolol, hidralazine and nifedipine); she experienced progressive uncontrollable hypertension but no symptoms, thus she was admitted to repeat studies. Laboratory evaluation (including creatinine, serum electrolytes, urinalysis, urine catecholamines and creatinine clearance) was normal Percutaneous transfemoral magnetic resonance angiography disclosed severe coarctation of abdominal aorta, functional occlusion of superior mesenteric artery and tight stenosis of right renal artery with poststenotic dilatation. Patient underwent surgery with aorto-aortic by-pass and right kidney artery reimplantation. Periodical controls confirmed no hypertension, even four years after surgery and normal flow patterns in Doppler ultrasonography. Patients with NF1 must be screened for pheochromoctyoma and renovascular hypertension. If hypertension appears, careful management is mandatory, as periodical follow-up even after surgery, since the long-term recurrence rate of renovascular lesions is not well established.
Pardo
R
R
Sección de Nefrología Pediátrica, Departamento de Pediatría, Hospital Universitario Central de Asturias.
Somalo
L
L
Málaga
S
S
Santos
F
F
D000293:Adolescent; D001017:Aortic Coarctation / Q000150:complications*; D005260:Female; D006801:Humans; D006973:Hypertension / Q000209:etiology*; D009456:Neurofibromatosis 1 / Q000150:complications*; D012078:Renal Artery Obstruction / Q000150:complications*