儿童下丘脑错构瘤的诊断与治疗

The diagnosis and treatment of hypothalamic hamartoma in children

目的 探讨儿童下丘脑错构瘤的诊断和治疗。方法 对 18例儿童下丘脑错构瘤的临床特点进行分析 ;应用CT、MRI进行诊断。手术除 1例采用经终板入路外 ,有 10例采用经翼点入路切除错构瘤 ;其他病例采用γ刀或药物治疗。结果 其临床表现特点为痴笑样癫痫和性早熟 ,少数病例有其他类型癫痫、智力低下或合并先天畸形。CT和MRI显示脚间池或垂体柄后上方有等信号改变 ,注药后无强化。 18例中 ,手术治疗 11例 ,有效率为 91% (10 / 11) ,对单纯性早熟者可治愈 ;γ刀治疗 36Gy大剂量有效 1例 ,无效 2例 ;药物治疗对性早熟有效 ,但对痴笑样癫痫及其他癫痫发作无效。结论 下丘脑错构瘤首选手术治疗。

Objective To investigate the diagnosis and treatment of hypothalamic hamartoma in children. Methods Eighteen cases of hypothalamic hamartoma in children, includeding 9 boys and 9 girls, were examined with CT and MRI. Eleven cases underwent operation. Post operation follow up was conducted for 0.5～6 years. Results The main clinical features of hypothalamic hamartoma were precocious puberty and gelastic seizures, some combine with other kinds of seizures, mental retardation or congenital abnormalities. The effective rate of surgery was 91%; patients with simple precocious puberty were cured. Conclusion Microsurgery is the first choice of treatment for hypothalamic hamartoma.