儿童鞍膈下颅咽管瘤的临床特点及手术治疗

摘要

目的 分析儿童鞍膈下颅咽管瘤的临床特点及手术治疗策略.方法 回顾性纳入南方医科大学南方医院神经外科2000年12月至2015年1月收治的68例鞍膈下颅咽管瘤患儿,均行手术切除.根据手术方式不同分为经蝶窦组(19例)和经颅组(49例),其中经颅组根据手术入路不同分为侧方入路组(12例)和前纵裂入路组(37例).所有患儿术后行门诊随访.分析鞍膈下颅咽管瘤的临床特点及影响手术方式、手术入路选择和术后复发的因素.结果 鞍膈下颅咽管瘤是儿童颅咽管瘤的常见类型(41.7％,68/163),最常见的临床表现为生长发育迟缓和视力视野改变,分别占86.8％(59/68)和69.1％ (47/68).58.8％ (40/68)的患儿术前即出现全垂体功能减退症.68例患儿的随访时间为18～270个月,平均(71.4±53.4)个月.头颅MRI增强扫描发现19.1％ (13/68)的患儿肿瘤复发,71.4％(20/28)的患儿术后出现新发的垂体激素轴功能障碍.肿瘤体积＞10 cm3、鞍上肿瘤横径/鞍内肿瘤最大横径＞2、垂直于鞍膈平面的肿瘤最大径＞4 cm和鞍上肿瘤囊腔存在复杂的生长方式为选择经颅手术的因素(均P＜0.05).鞍内肿瘤未循膜性边界切除、鞍内肿瘤最大横径＞2.5 cm是影响肿瘤复发的独立危险因素(均P＜0.05).结论 鞍膈下颅咽管瘤患儿无论选择经蝶窦还是经颅手术,增加鞍内肿瘤的显露和切除均可以降低术后复发的风险.经颅手术时选择前纵裂入路更有助于增加肿瘤的鞍内显露.%Objective To analyze the clinical characteristics and surgical treatment strategy of infradiaphragmatic craniopharyngiomas in children.Methods A retrospective analysis was conducted on 68 cases of childhood-onset infradiaphragmatic craniopharyngiomas who underwent surgical treatment at Department of Neurosurgery,Nanfang Hospital of Southern Medical University from December 2000 to January 2015.Among them,19 patients received transsphenoidal surgery,49 cases underwent craniotomy including 12 through lateral approach and 37 through anterior interhemispheric fissure approach.All patients were clinically followed up post operation.Analysis was conducted to explore the factors affecting the surgical operations,approaches and recurrence of infradiaphragmatic craniopharyngiomas.Results Infradiaphragmatic craniopharyngioma was common in childhood-onset craniopharyngiomas and accounted for 41.7％ (68/163).Growth retardation and impaired vision were two most common clinical manifestations which accounted for 86.8％ (59/68) and 69.1％ (47/68),respectively.There were 58.8％ (40/68) of the patients who presented preoperative panhypopituitarism.The follow-up ranged from 18 to 270 months (mean:71.4±53.4 month),and 13 out of 68 (19.1％) patients showed recurrence during the follow-up,20 out of 28 (71.4％) non-panhypopituitarism patients presented newly onset deficiency of pituitary hormones.Multiple factors,including the tumor size bigger than 10 cm3,ratio of suprasellar tumor diameter to intrasellar tumor diameter ＞ 2,sagittal maximal tumor diameter greater than 4 cm,tumor with suprasellar complex tumor cysts,were more likely to result in craniotomy (all P ＜ 0.05).Tumor resection not along the membrane boundary of intrasellar part and the maximal diameter larger than 2.5 cm were two independent risk factors for recurrence (all P ＜ 0.05).Conclusions Infradiaphragmatic craniopharyngioma have negative impacts upon the patients' quality of life.Exposure and removal of the intrasellar part of tumor could reduce the risk of recurrence for either transsphenoidal operation or craniotomy.Anterior interhemispheric fissure approach seems to be helpful for the exposure of intrasellar tumor and superior to the lateral approach.