Albright A L, Lee P A
Department of Neurosurgery, Children's Hospital of Pittsburgh, University of Pittsburgh School of Medicine, Pennsylvania.
J Neurosurg. 1993 Jan;78(1):77-82. doi: 10.3171/jns.1993.78.1.0077.
Five children, three girls and two boys, were treated for precocious puberty secondary to hypothalamic hamartoma by resection of the hamartoma. The patients' ages at onset of pubertal development ranged from 6 to 19 months. The hamartomas ranged in size from 6 to 10 mm; four were pedunculated, one was sessile, and all were located below the tuber cinereum. The hamartomas were excised via a right subtemporal approach, with transection at the inferior surface of the hypothalamus; two were adherent posteriorly to the basilar artery and brain stem, and the adhesions were divided. Postoperatively, three children exhibited a transient oculomotor paresis and one other child required eye-muscle surgery. The symptoms and signs of precocious puberty completely regressed postoperatively in all patients. Preoperative hormone assays of testosterone, luteinizing hormone, and follicle-stimulating hormone were within the pubertal range in all five children; postoperative assays fell to prepubertal levels. The children have been followed for 0.5 to 10.5 years (mean 5.0 years) postoperatively, without evidence of recurrence of precocious puberty. One child has begun spontaneous puberty at a normal age. It is concluded that complete resection of hypothalamic hamartomas causing precocious puberty is curative.
五名儿童，三女两男，因下丘脑错构瘤导致性早熟，接受了错构瘤切除术治疗。青春期发育开始时患者的年龄为6至19个月。错构瘤大小为6至10毫米；四个有蒂，一个无蒂，均位于灰结节下方。错构瘤通过右颞下入路切除，在下丘脑下表面横断；两个在后方与基底动脉和脑干粘连，粘连被分离。术后，三名儿童出现短暂动眼神经麻痹，另一名儿童需要进行眼肌手术。所有患者性早熟的症状和体征术后完全消退。术前所有五名儿童的睾酮、黄体生成素和卵泡刺激素激素检测均在青春期范围内；术后检测降至青春期前水平。术后对这些儿童进行了0.5至10.5年（平均5.0年）的随访，没有性早熟复发的迹象。一名儿童已在正常年龄开始自然青春期发育。结论是，完全切除导致性早熟的下丘脑错构瘤可治愈。
