儿童颅咽管瘤手术治疗和长期随访  

Surgical treatment and long-term follow-up results of pediatric craniopharyngiomas

作　　者：张玉琪[1] 王忠诚[1] 马振宇[1] 罗世祺[1] 甲戈[1] 邢俭[1] 谢坚[1] 

机构地区：[1]北京天坛医院神经外科,100050

出　　处：《中华神经外科杂志》2005年第9期516-520,共5页Chinese Journal of Neurosurgery

基　　金：北京市科技新星项目;获北京市科技进步三等奖

摘　　要：目的探求在保护下丘脑功能的条件下,安全地全切或近全切儿童颅咽管瘤。并长期随访术后病儿的生存情况。方法手术切除儿童颅咽管瘤202例。采用经胼胝体-透明隔间隙-穹隆间入路、经额部纵裂入路等八种入路切除鞍区和第三脑室内的颅咽管瘤。术前和术后采取积极措施预防和治疗下丘脑功能紊乱。并对其中的105例进行了12个月到6年的长期随访。结果全切+近全切184例(91.1%),死亡2例(0.9%)。术后主要并发症为:尿崩症发生率80.7%,长期尿崩症率11.4%;血电解质紊乱74.8%;癫痫9.4%。术后第1年和第4年是死亡高峰。手术后5年生存率68.2%。结论选择合适的手术入路在直视下尽可能全切除颅咽管瘤,并保护第三脑室前下外侧壁(下丘脑)。术后积极防治血钠紊乱和癫痫是确保手术安全的重要措施。术后放疗可明显降低颅咽管瘤的复发率。Objective To explore the method of total and near total removal of craniopharyngioma in children with ideal hypothalamic protection. The long - term life quality of these children were also followed up. Methods From April 1997 to December 2002, 202 cases of pediatric craniopharyngioma undertook surgery. Eight different approaches, including transcallosal - interseptal - interfornieeal approach in 89 cases, transfrontal interhemispherie approach in 68 cases, pterional approach in 20 cases, others in 25 cases, were used to remove eraniopharyngiomas involving the sellar region and the third ventricle. Active preventive and therapeutic measurements of hypothalamie dysfunctions were adopted pre - and postoperatively. One hundred and five cases were followed up with a term of 12 months to 6 years. Results 184 tumors were totally or subtotally removed （91.1%）. Two cases died（0.9% ）. Major postoperative complications included： diabetes insipidus in 81.7% of cases, permanent diabetes insipidus in 11.4% of, blood electrolytes disorder in 74.8% , seizures in 9.4%. The first and fourth years after operation were the peak of death. Five years survival rate was 68.2%. Conclusion Appropriate approaches with direct tumor exposure should be selected to remove craniopharyngioma as much as possible. The anteroinferolateral wall of the third ventricle （hypothalamus） should be protected carefully. Active measurements to prevent and treat blood sodium disorder and seizures could help to reassure the safety of surgery. Postoperative radiotherapy could decrease the reeeurence rate of craniopharyngioma.

关 键 词：颅咽管瘤 手术 生存 儿童 长期随访 手术治疗 下丘脑功能紊乱 纵裂入路 血电解质紊乱 手术切除 

分 类 号：R739.41[医药卫生—肿瘤] R775[医药卫生—临床医学]