显微手术切除复发性儿童颅咽管瘤的疗效

Outcomes of microsurgical resection of recurrent craniopharyngiomas in children

目的探讨显微手术切除复发性儿童颅咽管瘤的疗效。方法纳入2008年4月至2014年12月首都医科大学三博脑科医院神经外科采用显微手术切除的复发性儿童颅咽管瘤73例，回顾性分析其临床疗效。结果73例中，肿瘤全切除率为87．7％（64例），次全切除率为12．3％（9例）；围手术期病死率为2．7％（2例），迟发性脑脊液鼻漏发生率为2．7％（2例）。随访52例，随访时间为6—93个月，中位时间45个月。随访期肿瘤的复发率为36．5％（19／52），其中全切除者肿瘤的复发率为30．2％（13／43），次全切除者为6／9，完全切除者与次全切除者比较，肿瘤复发率的差异有统计学意义（P=0．039）；随访的52例中，26例（50．O％）有明显的生长发育迟缓，24例（46．2％）需日服抗尿崩药物，5例（9．6％）肥胖。随访期病死率为13．4％（7／52）。45例存活患儿中，Kamofsky功能状态评分为100分者4例（8．9％），90分者35例（77．8％），80分者3例（6．7％），60分者2例（4．4％），40分者1例（2．2％）。结论采用显微手术切除儿童复发性颅咽管瘤可以取得较满意的疗效，术后下丘脑一垂体功能障碍的恢复仍然是个难题。

Objective To investigate the clinical outcomes in children with recurrent craniopharyngimas after microsurgical resection of the tumors. Methods The clinical outcomes of 73 cases of recurrent childhood craniopharygngiomas from April 2008 to December 2014 were retrospectively analyzed. All patients underwent microsurgical resection of the tumors at Neurosurgery Department of Sanbo Brain Hospital, Capital Medical University. Results Total removal of the tumor was achieved in 64 （87.7%） of the 73 patients and the subtotal resection rate was 12.3% （9 cases）. The perioperative mortality was 2.7% （2 cases）. A total of 52 patients were followed up from 6 to 93months, with the median of 45 months. The tumor recurrence rate was 36.5% （19/52） during the follow-up period. For those patients whose tumors were removed totally, the tumor recurrence rate was 30.2% （13/43）, and it was 66. 7% （6/9） for those whose tumors were removed subtotally. There was statistical difference between the two groups （ P=0. 039）. The fatality rate was 13.4% （7/52） during the follow-up. We used the Karnofsky Performance Scale to assess the life quality of the 45 children. The results showed that 4 children scored 100 points, 35 scored 90, 3 scored 80, 2 scored 60, and 1 scored 40. There were 26 （50. 0% ,26/52） children demonstrating significant growth retardation, 24 （46. 2%, 24/52） needed oral desmopressin to treat diabetes insipidus, and 5 （9. 6%, 5/52） showed significant obesity. Conclusions Microsurgical resection of recurrent craniopharyngiomas in children may result in good outcomes. The treatment of postoperative hypothalamic-pituitary deficiency is still a challenge.