大田原综合征的外科治疗(附6例报道)

Surgical management of Ohtahara syndrome

目的探讨手术治疗大田原综合征(OS)的适应证、方法和预后。方法回顾性分析我院采用外科手术治疗6例OS的经验。1例半巨脑患者行改良的大脑半球切除术,其余5例行一侧半球的切除性手术联合胼胝体前部切开术。结果本组随访6个月至3.5年,Engel分级I级2例,Ⅱ级2例,Ⅲ、Ⅳ级各1例。Gesell发育商数(DQ)较术前明显提高。本组2例患者出现暂时性的并发症,无手术死亡。结论皮质发育障碍是OS的重要病因。对部分有明确结构性异常的药物难治性OS,早期手术可以有效地控制患者的癫痫发作,并改善患者的精神运动发育迟滞。

Objective To investigate the indications,surgical procedures and outcomes of Ohtahara syndrome （OS）.Methods Surgical experiences of 6 patients with OS were reviewed. One patient with hemimegalencephaly underwent modified functional hemispherectomy,while the other 5 patients underwent hemispherectomy and anterior callosotomy.Results All patients were followed-up from 6 months to 3.5 years post-operation. The results were as following： Engel class I in 2 patients,Engel class Ⅱ in 2 patients,Engel class Ⅲ in 1 patient and class Ⅳ in 1 patient. Gesell development quotient score assessment showed significant improvement in all patients. Temporary complications were observed in 2 patients and there was no dead case.Conclusion Cortical dysplasia plays an important role in the pathogenesis of OS. Early surgical intervention for intractable Ohtahara syndrome with structural abnormality is beneficial to the control of seizure and the prevention of irreversible intelligence and psychomotor retardation.