모야모야 병, 관상동맥 협착 및 확장형 심근병증을 동반한 말단비대증

A 19-year-old girl with typical features of acromegaly, duration of which was thought to be above 10 years, presented with dyspnea. After serial studies, dilated cardiomyopathy and critical stenosis of left anterior descending coronary artery were demonstrated. Brain MRI revealed the following changes: the pituitary gland was enlarged and its upper margin was upwardly convex; severe cerebral atrophy and multiple signs of vascular abnormalities were present. Cerebral angiographic studies demonstrated the presence of complete occlusion of left carotid artery and severe stenosis of right carotid artery with the formation of collateral networks of vessels. The patient was thus diagnosed as having moyamoya disease that was thought to be congenital according to her past histories. Her cardiac function was slightly improved after percutaneous transluminal coronary angioplasty. Operation for the pituitary lesion and moyamoya disease was not performed due to poor general condition, but serum growth hormone concentration was adequately suppressed after octreotide therapy. We consider that this is a very rare case of acromegaly associated with moyamoya disease, coronary arterial disease and dilated cardiomyopathy.(Korean J Med 58:227-233, 2000)