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目的探讨儿童下丘脑错构瘤的临床特点及MRI表现,提高对本病的认识水平。方法回顾性分析6例经手术病理证实的下丘脑错构瘤的临床特点及MRI表现,所有病例均行MRI平扫及增强扫描。结果 6例病例临床均表现为性早熟,其中1例同时表现为性早熟及痴笑样癫痫并伴有智力低下,5例在2岁前出现症状,1例在3岁后发病。6例MRI均表现为鞍上下丘脑区单发结节,病灶同脑灰质比较T1WI、T2WI均为等信号,增强后病灶均未强化。结论儿童下丘脑错构瘤临床及MRI表现典型,依据其典型表现即可作出正确诊断。
Objective To investigate the clinical features and MRI findings of hypothalamic hamartoma in children and to raise awareness of the disease. Methods The clinical features and MRI findings of 6 cases of hypothalamic hamartoma confirmed by surgery and pathology were retrospectively analyzed. All patients underwent MRI plain scan and enhanced scan. Results All the 6 cases showed precocious puberty. Among them, 1 case showed precocious puberty and giggle - like epilepsy accompanied by mental retardation, 5 cases presented symptoms before 2 years old and 1 case developed after 3 years old. The MRI findings of all 6 cases were solitary nodules in the supraorbital and hypothalamic regions. Compared with the gray matter, T1WI and T2WI were equal signals, and none of the lesions were enhanced after the enhancement. Conclusions The clinical manifestations and MRI features of hypothalamic hamartoma in children are typical, and the correct diagnosis can be made based on their typical manifestations.