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目的 探讨下丘脑错构瘤导致性早熟的手术治疗。方法 回顾分析 1999年 3月至 2 0 0 0年 7月收治的 6例下丘脑错构瘤导致的单纯性早熟 ,男女各 3例 ,平均发病年龄 7 3个月 ,就诊年龄13个月至 5岁 ,平均为 2岁 6个月。均表现为生长发育快 ,骨龄明显大于实际年龄 ,性激素达到青春期水平 ;女孩表现为乳房发育 ,来月经 ;男孩表现为阴茎和睾丸增大 ,易勃起 ,面部痤疮等。MRI显示垂体柄后上方灰结节部位皮质样信号肿物 ,注药无强化 ,大小为 4～ 12mm。 6例病人均采用右翼点入路显微手术切除错构瘤。结果 6例下丘脑错构瘤均获全切除 ,术后性早熟停止 :男孩勃起消失 ,痤疮消失 ;女孩乳房发育停止 ,月经停止 ,复查性激素均下降至儿童期水平 ;术后无任何并发症。结论 下丘脑错构瘤导致的单纯性早熟首选为显微手术切除错构瘤 ,全切后可治愈
Objective To investigate the surgical treatment of hypothalamic hamartoma leading to precocious puberty. Methods Retrospective analysis of 6 cases of hypothalamic hamartoma in March 1999 to July 2000 caused by simple precocious puberty, 3 cases of male and female, the average age of onset of 73 months, the age of 13 months to 5 The average age is 2 years and 6 months. All showed rapid growth and development, bone age was significantly greater than the actual age, sex hormones reached puberty; girls showed breast development, to menstruation; boys showed increased penis and testicles, erectile easily, facial acne and so on. MRI showed pituitary stalk gray cortex above the site of the cortical signal mass, no drug injection, the size of 4 ~ 12mm. All 6 patients underwent microsurgical resection of hamartoma with right-wing point approach. Results 6 cases of hypothalamic hamartoma were resected, precocious puberty stopped: the boy disappeared, acne disappeared; girls stopped breast development, menstruation stopped, sex hormone levels were reduced to childhood; no postoperative complications. Conclusions Hypothalamus hamartoma is the first choice of simple precocious pouch for microsurgical resection of hamartoma,