Neurofibromatosis of the Renal Artery

multiple neurofibromatosis of von Recklinghausen is characterized by areas of cutaneous pigmentation and multiple tumors of the nervous system. Neurofibromas are most common along the peripheral nerves, being subcutaneous in site, but have also been described in the gastrointestinal and genitourinary tracts (1, 2). Meningiomas and gliomas are associated with neurofibromatosis, as are vascular anomalies such as cavernous hemangiomas. It has been suggested that neurofibromatosis is related to neurocutaneous disorders, such as the Sturge-Weber syndrome and von Hippel-Lindau's disease. It is well established that pheochromocytomas occur relatively frequently in patients with neurofibromatosis (3). That neurofibromatosis may be the cause of renal artery abnormalities and hypertension is not well known. The subject was recently reviewed by Halpern and Currarino (4) who found 7 previously reported cases and added 3 of their own. Of the 10 patients, 1 was not operated upon, 2 remained hypertensive after surgery, and 4 did not have long-term followup. Only 3 patients were known to be normotensive nine or more months after operation. In these the evidence is strong that the hypertension was caused by the lesions which were repaired. The case to be described was followed closely for one year after definitive surgery. It supports the concept that neurofibromatosis of a renal artery is a cause of systemic diastolic hypertension. Report of a Case A white girl was first seen at the age of four because of a tumor in the left popliteal region. Physical examination revealed many cafe-au-lait spots over the abdomen and thighs and a blood pressure of 130/80 (Fig. 1). The tumor of the leg was excised, and microscopic examination revealed a typical neurofibroma. At nine years of age a neurofibroma of the scalp was removed. The blood pressure at that time was 150/110 and a phentolamine test, an intravenous urogram, and urinary catecholamines were normal. Hypotensive medication was begun and continued until the first kidney operation. When the patient was fourteen an abdominal aortography was unsatisfactory because of technical problems. Six months later, a large neurofibroma was removed from the right flank. In September 1964, when the girl was sixteen years old, an intravenous urogram showed minimal dilatation of the right renal collecting system and a kink of the right ureter at the ureteropelvic junction (Fig. 2). A radioactive renogram obtained with the patient relatively dehydrated showed an abnormal curve on the right. Split renal function studies demonstrated reduction in urine flow, sodium excretion, creatinine, and para-aminohippurate (PAH) clearances on the right. PAH concentration was higher on the right side than on the left. The study was done according to the method of Stamey (5), but because the third period of urine collection was not entirely satisfactory the test could not be considered positive.