下丘脑错构瘤所致单纯性早熟的显微外科治疗

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２０００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

Microsurgical treatment of hypothalamic hamartoma causing precocious puberty

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.