Hypothalamic-pituitary dysfunction associated with moyamoya disease in children

Moyamoya disease is a rare cerebral vascular disease that results in narrow ing of the vessels of the circle of Willis and the formation of a network o f collateral vessels at the base of the brain for compensatory perfusion. M oyamoya disease has the highest incidence during the first decade of life, and children present most frequently with transient ischemic attacks. We pr esent two cases of Moyamoya disease in children with associated hypothalami c-pituitary dysfunction. Both children presented to the endocrinologist for decreased growth velocity. One child had hypothyroidism and both had growt h hormone deficiency. A review of the literature reveals a few isolated cas e reports of hypothalamic-pituitary dysfunction occurring with Moyamoya dis ease and with other states of cerebral vascular insufficiency. We suggest t hat children with compromise of cerebral vascular perfusion be monitored cl osely for clinical signs and symptoms of hypothalamic-pituitary dysfunction .