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下丘脑错构瘤所致单纯性早熟的显微外科治疗 被引量:8

Microsurgical treatment of hypothalamic hamartoma causing precocious puberty
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摘要 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 。 Objective To study the surgical treatment of hypothalamic hamartoma causing precocious puberty. Methods Six children, (three girls and three boys) were treated for precocious puberty secondary to hypothalamic hamartoma by resection of the hamartoma. The mean age was 30 months (ranged from 13 months to 5 years) and the mean ages at onset of pubertal development was 7 3 months. All patients were presented with a fast development and bone age, pubertal sex hormone level. Boys were presented with excessive growth of external genitalia, erecting frequently and acne in face; girls with development of breast and menses. Magnetic resonance imaging demonstrated an isointense mass below the tuber cinereum, ranged from 4 to 12mm in diameter. The hamartomas were totally removed via a right frontotemporal approach. Results The symptoms and signs of precocious puberty completely regressed postoperatively in all patients without any complication, and sex hormone level fells to prepubertal. Conclusions Microsurgery is the first choice of treatment for precocious puberty simple due to hypothalamic hamartoma and precocious puberty can be cured if hypothalamic hamartoma were totally removed.
出处 《中华神经外科杂志》 CSCD 北大核心 2000年第6期341-344,共4页 Chinese Journal of Neurosurgery
关键词 下丘脑错构瘤 性早熟 显微外科手术 儿童 Hypothalamic Hamartoma Precocious puberty Microsurgery
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