Two Causes of Hypertension in a Subject with Generalized Neurofibromatosis

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Abstract
Absrract. A 25-year-old male, with cutaneous neurofibromatosis, admitted with a history of intermittent headache, vomiting and hypertension for several years and attacks of palpitation and sweating for one year. Provocation tests with glucagon and histamine indicated a pheochromocytoma. Angiography of the renal artery revealed a pheochromocytoma in the left adrenal and a stenosis with an aneurysm of the right renal artery. Split renal function studies corresponded to a stenosis of the right renal artery. After removal of the tumour and vascular reconstruction of the right renal artery the patient was discharged in a normotensive state. Split renal function studies, sixteen months later, indicated a stenosis of the left renal artery which a renal angiography could not verify at least not in the main branches. As a result of the vascular reconstruction of the right renal artery, a remarkable increase in the size of the right kidney was noted roentgenologically. A possible explanation of the bewildering stenosis pattern in the split renal function studies postoperatively might be vascular changes in the intrarenal interlobar and/or arcuate arteries in the kidney, predominantly on the left side. The possibility of similar vascular changes in the small coronary vessels as an explanation of the ECG-changes appearing after the vascular reconstruction of the right renal artery stenosis is discussed. The combination between neurofibromatosis, renal artery stenosis and hypertension as well as neurofibromatosis, pheochromocytoma and hypertension have both been reported in the literature. Our case demonstrated hypertension, renal artery stenosis (and aneurysm), neurofibromatosis and pheochromocytoma, which seems to be a rare combination not previously described.