Hypertension in children with neurofibromatosis

Hypertension in neurofibromatosis is mostly a consequence of a stenosis of the renal artery or is due to phaeochromocytoma. Riccardi pointed out primary hypertension in patients with several cervical neurofibromas in the absence of phaeochromocytoma and he noticed that the elevation of BP was often already present in children. Nine (15.8%) of 57 neurofibromatosis patients (age from 1.5 to 23 years) examined, presented BP levels above the 95th percentile on several occasions and three in particular had severe hypertension with compromised target organs. Two of them had a stenosis of the renal artery, in the third an organic origin of hypertension was not demonstrated, but there was an asymptomatic glioma of the hypothalamus. The other six children had a labile or borderline hypertension and two of them had, respectively, a glioma of the thalamus and of the optical chiasm. Elevation of the catecholamine metabolites or other causes of hypertension were not found in any of these patients. These preliminary data show a high incidence of hypertension in neurofibromatosis, primary or due to organic causes and overall they point out a possible correlation between hypertension and cerebral neoplasia.