Bilateral pheochromocytoma in a boy

We report on a 8 year old boy in whom ophthalmological signs (scotoma, fund us hypertonicus stage IV) and an elevated blood pressure lead to the diagno sis of an isolated pheochromocytoma of the left adrenal. 6 months before, a cerebral convolsion was interpreted as a benign convulsion caused by infec tion. The boy was cured by adrenalectomy. However, 3 years after the initia l diagnosis, he showed again typical clinical signs of a pheochromocytoma w hich was finally confirmed by laboratory tests and computertomography. The right adrenal was removed. Family history was negative. By molecular analys is, an association with von Hippel-Lindau disease was found. Discussion: Our case emphasizes the necessity to exclude a phacomatosis in all cases of pheochromocytoma.