儿童复发性颅咽管瘤的综合治疗与长期随访

Combined treatment and follow-up of recurrent craniopharyngiomas in children

目的分析儿童复发性颅咽管瘤的临床特点,探讨其治疗方法,并评估病儿长期随访的生存质量。方法回顾性分析36例儿童复发性颅咽管瘤病人的临床资料。根据肿瘤的影像学特点,选择合适的手术入路,采用额部纵裂入路17例,胼胝体-穹窿间入路10例,翼点入路5例,额底入路4例;并对病人进行长期随访。结果 肿瘤全切除14例,近全切除20例,大部分切除2例。术后视力改善21例,保持3例,恶化3例。术后出现多饮、多尿16例,电解质紊乱13例,中枢性高热1例,单侧肢体活动差1例,死亡1例。30例随访4个月~7年,肿瘤复发5例。GOS评分：5分24例,4分1例,3分1例,1分4例。结论儿童复发性颅咽管瘤再复发率高,且与周围组织黏连紧密,但手术治疗或手术联合放疗仍是有效的治疗方法。

Objective To investigate the microsurgical treatment and analysis of the postoperative complications for craniopharyngiomas.Methods Clinical data of 103 patients with craniopharyngioma undergoing microsurgical resection were analyzed retrospectively.The operation was performed via pterional approach in 76 cases,subfrontal approach in 16,transcallosal approach in 10 and transsphenoidal approach in 1.Results Total tumor removal was achieved in 65 cases,subtotal removal in 26 and partial removal in 12.Postoperatively diabetes insipidus occurred in 69 cases,epilepsy in 7,central high fever in 5,oculomotor nerve injury in 4,permanent hypopituitarism in 3.Two patients died within 1 month after operation.Sixty-seven patients were followed up for mean of 3.5 years,ranged from 2 months to 11 years.Normal work and life enjoyment were found in 52 patients,adiposity in 12,anterior pituitary hypofunction in 3.The tumors recurred in 11 cases and 6 patients died.Conclusions The choice of microsurgical approach for craniopharyngioma resection is based on the location,size and characteristic of the tumor,while pterional approach is appropriate for most patients.Skillful microsurgery,meticulous manipulation,and active management for postoperative complications are very important in reducing the fatality and disability rate and improving life quality.