患者,男,16岁。发育迟缓,身材矮小,肥胖,面部色素沉着多年,双眼视力进行性减退3年。体重45 ks,身高133cm。面部及身干广泛奶油咖啡色斑点。无胡须、阴毛,外生殖器幼儿型。表情呆板,反应迟缓,智能差,右视力光感,左视力米远数指;左颞侧偏盲,双视乳头原发萎缩。眼球活动明显受限,颅平片示蝶鞍扩大,骨质破坏。CT诊断:颅咽管瘤。 Patient, male, 16 years old. Stunted, short stature, obesity, facial pigmentation for many years, binocular vision progressive decline of 3 years. Weight 45 ks, height 133cm. Wide range of creamy and brown spots on the face and body. No beard, pubic hair, external genitalia children. Facial expression is dull, slow reaction, poor intelligence, right vision light perception, left vision meters far; left hemianopia, bilateral primary atrophy of the nipple. Obvious limitations of eye activity, flat skull showed enlarged sella, bone destruction. CT diagnosis: craniopharyngioma.