Feocromocitoma y neurofibromatosis de von Recklinghausen: crisis postparto y trombosis de arteria renal / Pheochromocytoma and von Recklinghausen neurofibromatosis presenting with a crisis during the puerperal period: report of 1 case

ABSTRACT

Pheochromocytoma is observed with higher frequency in patients with von Recklinghausen neurofibromatosis. We report a 36 years old female with von Recklinghausen neurofibromatosis who developed mild hypertension during the fourth month of pregnancy. A cesarean section was performed at 37 weeks of pregnancy. Thereafter, the patient presented severe hypertensive and hypotensive crises, sinus tachycardia and fever. No evidences of an infectious disease were found. Abdominal ultrasound examination showed a right adrenal mass of 7 x 5 cm. High levels of urinary cathecolamines confirmed the diagnosis of pheochromocytoma. After three weeks of prazosin therapy, the patient was operated. During the surgical procedure, an encapsulated pheochromocytoma was found and excised. A right renal atrophy and renal artery thrombosis were also found and a nephrectomy was done. Postoperative evolution was uneventful and the patient remains with normal blood pressure levels six months after the operation