天坛小儿神外宫剑访谈（下篇） 儿童颅咽管瘤经前纵裂入路手术难度分型及热点问题探讨

接上篇 天坛小儿神外宫剑访谈（上篇）-儿童颅咽管瘤经前纵裂入路手术难度分型及热点问题探讨

7、前纵裂入路最大的障碍是前交通动脉，有学者提出主动切断以增加术腔显露，请问您的观点？

前交通动脉直接影响前纵裂入路术野的暴露，无论经第I间隙还是第IV间隙切除肿瘤，前交通动脉的宽度直接限制了额叶牵拉和肿瘤的侧方暴露。理论上，前交通动脉平均长度可达12.7mm，有足够的活动度与延展性[50]，但临床中不少病例由于前交通动脉短粗，严重影响了暴露范围。因此，有些学者提出主动切断前交通动脉换取更加宽阔的手术空间[51]。我们主张应保护好前交通动脉，通过锐性松解蛛网膜，达到最佳暴露效果[50]。损伤前交通动脉孤立了双侧大脑前动脉，增加了大脑前动脉梗塞的风险（图8）[52]。另外，前交通动脉发出重要的前穿质（下丘脑支、视交叉支、胼胝体下动脉），损伤后可能导致穹隆、胼胝体膝部梗塞[53]，表现为顺行性失忆、执行功能障碍、性格改变等“前交通动脉综合征”[54]。

图8：儿童颅咽管瘤手术一例，术中前交通动脉损伤，术后双侧额叶大面积梗塞。虽属罕见，一旦出现，预后极差。

需要特别指出，任何经前纵裂手术都要做好前交通动脉破裂出血的应急预案，特别是婴幼儿，瞬间急性失血危及生命。我们每年经前纵裂入路切除鞍区肿瘤近200例（颅咽管瘤、视路胶质瘤、鞍区生殖细胞类肿瘤等），术中前交通动脉破裂出血概率1-2%，多因肿瘤囊壁与动脉粘连紧密甚至侵蚀，一经游离即管壁破裂，出血凶猛。此时，术者要沉着冷静，与时间赛跑，吸引器准确指向破裂口，防止血液倒灌至深方造成急性脑膨出；助手可以夹住破口，降低流速，此时，术者无论电凝烧灼或动脉瘤夹夹闭（图9），一定要确切止血，切记不要海绵压迫[55,56]或采取肌肉粘附破裂口[57]；一旦迟发出血，患儿瞬间昏迷，严重者危及生命，我们曾有血的教训，在此提醒同行切记。

图9： 本例游离肿瘤顶壁与前交通动脉粘连处时，突然破裂出血、瞬时凶猛，两枚蛇牌720动脉瘤夹夹闭，确切止血。

8、颅咽管瘤复发率高，一旦复发就要再次手术吗？请谈谈您的观点。

颅咽管瘤是良性肿瘤，恶性生长。其侵袭性生长方式，类似于恶性肿瘤，极易复发[19]。一旦颅咽管瘤术后复发，是再次手术还是采取其它治疗方式？由于复发颅咽管瘤已失去瘤体与下丘脑正常界面，手术风险大大提高[59，此时，医生的经验起到决定性作用。他必须准确预判，再次手术和其它治疗，哪种方式对患儿更安全，更有效。若经验不足，一念之差就决定了患儿的生死，一定要慎重抉择。目前除手术外，针对复发颅咽管瘤，应用最广的是伽玛刀治疗，特别适用于术后残留或复发的实体小肿瘤；对于体积较大的囊性肿瘤，可抽吸缩小后再行伽玛刀治疗，肿瘤控制率可达88.5%[58]；针对瘤体囊性变采取囊内放射性治疗（32P，90Yu，186Rh和198Au）、博来霉素治疗、IFN-α治疗，10年有效控制率达80%以上[59]。因此，针对复发颅咽管瘤要制定个体化治疗方案，降低致残致死率（图10）。

图10：8岁男性患儿，主诉：间断头痛2年余，多饮多尿2周。头颅CT示鞍区类圆形钙化影；MRI示鞍内、鞍上囊实性混杂信号，大小约45×28×19mm,不均匀强化，颅咽管瘤诊断明确（上排）。2015年8月行前纵裂入路肿瘤切除术，肿瘤切除满意，术后下丘脑反应轻、恢复好，定期随访。术后2年，发现鞍上囊性变，考虑肿瘤复发，鉴于复发肿瘤体积小、位置深在、囊性为主，再次手术风险大，改行立体定向囊腔穿刺囊液抽吸+伽玛刀治疗。随访至今，肿瘤控制满意，未见生长。目前，术后六年，患儿身高增长近50cm, 正常学习生活,笔者甚感欣慰。

9、颅咽管瘤术后，由于患儿身材矮小，家长急于要求注射生长激素，您的建议？

颅咽管瘤患儿术后生长激素缺乏高达75%[60]，表现为身材矮小、性发育迟缓，家长迫切希望通过注射生长激素促进孩子身高增长。但是，需要特别关注，生长激素具有促进细胞有丝分裂及抗凋亡作用，不但对正常组织有增殖效应，对肿瘤的生长、转移也有促进作用[61]。因此，生长激素可以明确促使颅咽管瘤复发，年龄越小，复发率越高[62]。颅咽管瘤术后患儿，一定要谨慎，不应人为促进肿瘤复发。何时注射，应选择对肿瘤复发影响最小的时机。我们与北京儿童医院内分泌科多年合作，一致建议颅咽管瘤术后经过5年随访，没有肿瘤复发迹象后再使用生长激素，可将复发率降至最低。

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