天坛小儿神外| 专访宫剑：儿童Chiari畸形的天坛小儿神外诊疗策略(下)-术式与案例视频

昵称63220632 2021-01-06

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天坛小儿神外

第5期

神外前沿讯，很多儿童因各种原因偶然发现小脑扁桃体下疝，到医院咨询时经常听到医生提起Chiari畸形。针对儿童Chiari Ⅰ型的手术，需要解决哪两个主要问题？目前临床上有哪些成熟有效的术式？

北京天坛医院小儿神经外科主任宫剑教授认为，针对儿童Chiari Ⅰ型，北京天坛医院小儿神经外科采用：后颅窝骨减压+小脑扁桃体电凝还纳+硬膜扩张成形术。该术式减压确切，患儿症状缓解明显，脊髓空洞迅速消失，安全可靠，值得推荐。

访谈级案例内容如下：

神外前沿：目前针对Chiari Ⅰ型有哪些手术方式可以选择？

宫剑：针对儿童Chiari Ⅰ型的手术需要解决两个主要问题：1.缓解枕大孔区结构拥挤，充分减压；2.恢复脑脊液循环，解除形成脊髓空洞的“水锤效应”。

Chiari Ⅰ型手术历经百年，国内外均以后路减压为主，辅以小脑扁桃体切除、硬膜成形等，具体术式包括：

1.后颅窝单纯骨减压术（Posterior fossa decompression）：

世界第一例Chiari畸形的后颅窝单纯骨减压术是由荷兰外科医生Van Houweninge Graftdijk在1930年完成的⁴⁴，至今已近百年。他通过去骨瓣减压术（或合并咬除寰椎后弓）扩大后颅窝容积，没有处理硬膜及寰枕筋膜，是单纯的硬膜外操作。该术式简单易行，但后颅窝减压并不充分，术后症状有效缓解率只有30%-73%⁴⁵。目前全球仅有极少的医疗机构仍在使用⁴⁰。

2.后颅窝骨减压加硬膜成形术（Posterior fossa decompression with duroplasty）：

后颅窝骨减压加硬膜成形术是在单纯骨减压术的基础上对硬膜进行剪开并扩张重建，是硬膜下操作，减压效果较单纯骨减压更为充分⁴⁶。2020年英国莱顿医院针对两个术式进行了meta分析，结果显示儿童患者行后颅窝骨减压加硬膜成形术的症状缓解率明显高于单纯骨减压（93.3% vs 74.8%），前者更有利于受阻脑脊液循环的恢复，对伴有脊髓空洞症的患儿尤其有效³¹。据美国弗吉尼亚大学健康科学中心报道，后颅窝骨减压加硬膜成形术术后87.5%患者脊髓空洞可缩小或消失⁴³。

3.硬膜外层切开术（Dura-splitting）：

1993年日本钏路Rosai医院Isu提出无需开放硬膜下腔，行硬膜外层切除术即可达到减压目的47。这其实是介于单纯的后颅窝骨减压术与后颅窝骨减压加硬膜成形术之间的“折中”术式。2018年土耳其穆斯塔法凯末尔大学一项大样本（113例）临床研究发现硬膜外层切除术术后脊髓空洞的缓解率显著低于后颅窝骨减压加硬膜成形术（49.6% vs 54.6%）⁴⁸。这提示硬膜外层切除术并未能做到充分减压，无法有效解除脑脊液循环障碍，尤其不适合用于合并脊髓空洞的患者。

4.小脑扁桃体切除术/电凝术：

小脑扁桃体切除术在1958年首先由美国克利夫兰诊所的神经外科先驱W. James Gardner提出^49,50。此术式将疝入枕骨大孔内的小脑扁桃体直接切除，通过减少颅腔内容物达到减压的目的。该术式切口小，甚至采用神经内镜完成。由于术野狭小，一旦操作不慎合并脑挫伤、脑出血、脑肿胀，直接危及患者生命⁵¹；另外小脑扁桃体属于新小脑（neocerebellum），具有协调同侧肢体随意及精细运动的重要功能，直接手术切除，可能导致显著的运动性共济失调^52,53。

小脑扁桃体电凝术（cauterization of the tonsils/tonsillopexy）是小脑扁桃体切除术的替代术式，通过低功率电凝使扁桃体挛缩上移，避免了脑实质的切除。意大利ARNAS Civico医院针对成人患者的研究提示该术式的症状缓解率高达72.0%⁵⁴；范德堡大学医学中心针对儿童患者的研究发现后颅窝骨减压术加扁桃体电凝术可使脊髓空洞有效缓解⁵⁵；美国约翰斯霍普金斯大学医学院神经外科对171例儿童Chiari Ⅰ型手术的回顾性分析显示，在后颅窝骨减压加硬膜成形术的基础上行扁桃体电凝术，术后脊髓空洞的缓解率可以提高6.11倍⁵⁶。有理论认为，下疝的小脑扁桃体如活塞般（piston-like）堵塞椎管内的脑脊液循环通道，导致脑脊液随搏动被泵入脊髓腔内，导致脊髓空洞症⁵⁷。近期的临床研究显示，小脑扁桃体电凝术是针对脊髓空洞的有效术式，有力地证明了这一理论的正确。

5.寰枢椎融合术（atlantoaxial fixation）：

2015年，印度Seth G.S.医学院Goel教授提出Chiari畸形、脊髓空洞均是寰枢关节不稳定的继发表现，因此所有Chiari畸形均建议行寰枢椎融合术，其报道术后症状缓解率96.9%⁸；脊髓空洞体积均可缩小⁵⁸。目前这一学说没有充分的理论依据，引起学术界广泛争议²⁴，不推荐为常规手术方案。

神外前沿：针对儿童Chiari Ⅰ型，天坛小儿神外采用哪种术式？效果如何？

宫剑：通过各种术式的比对与总结，针对儿童Chiari Ⅰ型，北京天坛医院小儿神经外科采用：后颅窝骨减压+小脑扁桃体电凝还纳+硬膜扩张成形术。该术式减压确切，患儿症状缓解明显，脊髓空洞迅速消失，安全可靠，值得推荐。另外，美国约翰斯霍普金斯大学医学院、美国俄克拉荷马州健康科学中心均认可这一术式，并推荐为Chiari Ⅰ型的首选术式，较单纯的骨减压或硬膜成形术更加有效^36,56。

该术式具体手术要点，我们总结为：

1.骨瓣大小应根据不同年龄儿童设计（通常约3×4cm），需要显露双侧小脑扁桃体；枕大孔后环增厚骨质需用咬骨钳咬除；妥善保留枕外隆突的肌肉附着点，以便肌肉对位缝合，避免皮下积液。

2.咬除寰椎后弓时，严禁咬骨钳伸入后弓与硬脊膜之间操作，避免直接压迫延颈髓造成严重后果。

3.儿童寰枕窦发达，硬膜减压时注意边切开边缝扎，避免大量失血。

4.低功率双极电凝小脑扁桃体，务必保持软脑膜完整，软膜外操作可有效避免脑挫伤；电凝可使扁桃体回缩上移，同时其与延颈髓间黏连的纤维小梁需逐一锐性切断；待扁桃体回缩至枕骨大孔水平以上，脑脊液喷涌而出，即达到了满意的减压效果。

5.术腔反复冲洗清亮，不放任何止血材料，避免异物造成术后发热；自体筋膜减张缝合硬膜，达到严密水封效果。

6.术后不使用抗生素，颈托保护下，鼓励尽早下床活动。

需要指出，若Chiari Ⅰ型患儿合并颅底凹陷、颈椎侧弯、寰枕/寰枢椎关节不稳、脑干腹侧受压等^32,59 ，统称为复杂型Chiari畸形，术式复杂，需个体化设计，不在本文讨论范围之内。

最后强调，儿童Chiari Ⅰ型临床常见，90%不需要手术治疗，家长不必过于担心，定期复查即可，成年后部分患者存在自限性而停止发展²⁷。患儿只有出现相关症状或合并脊髓空洞才考虑手术治疗。一旦确定手术，应该及早治疗，通过寰枕部充分减压，加上儿童强大的神经系统自修复功能，效果满意。

病例展示

典型病例一：

7岁男性患儿，主诉“后枕、颈、肩部阵发性疼痛2年，进行性加重”。疼痛发作时，头颈部针刺样刺痛，每次持续约10分钟后自行缓解。查体示：患儿神清语利、自主体位，肌力肌张力正常，无感觉分离障碍，无双手鱼际肌萎缩。我院3D-CT除外扁平颅底及颅底凹陷（图1）；MRI提示：小脑扁桃体下疝并C2脊髓空洞形成（图2）。

图1.术前CT未见明显扁平颅底及颅底凹陷。

图2.术前MR显示典型Chiari畸形 I型表现。

鉴于患儿有特征性头颈肩部疼痛，影像学呈典型Chiari畸形 Ⅰ型表现，小脑扁桃体下疝超过5mm，合并脊髓空洞，具备手术指征。依据天坛小儿神外诊疗规范，于2020年10月08日在全麻下行“后正中入路枕下骨减压+扁桃体电凝还纳+硬膜扩张成形术”。

图3.枕下减压，骨窗大小4X3cm，寰枕筋膜及寰枕部硬膜明显增厚，双侧小脑扁桃体受挤压变形，疝入椎管。

图4.低功率双极电灼双侧扁桃体，使之挛缩上抬，还纳入颅。寰枕交界处充分减压，脑脊液流出通畅（左图）；寰枕交界充分减压后，自体筋膜减张严密缝合（右图）。

术后患者一般情况好，头颈疼痛缓解明显。术后当晚颅脑CT显示枕下减压充分（图5）；术后1周复查头颈MR提示脊髓空洞较前明显缩小，寰枕交界处不再拥挤。术后1月来院复查，头颈疼痛消失，头颈MR提示小脑扁桃体还纳满意，颈髓空洞消失（图6、7）。

图5.术后当晚复查头颅CT显示枕下减压满意。

图6.术后1周（图中）及1月（图右）复查头颈MR，提示小脑扁桃体还纳入颅，脊髓空洞消失。

图7. 术后1周（图中）及1月（图右）复查头颈MR，提示小脑扁桃体还纳满意，枕大孔区结构拥挤得到有效缓解。

典型病例二

8岁男性患儿，主诉“体检时偶然发现小脑扁桃体下疝，随访四年，扁桃体进行性下疝并脊髓空洞形成”。初诊时患者扁桃体下疝不超过1mm，经过4年随访，下疝已达15mm，并伴有C2-4脊髓空洞。患者无明显临床症状及体征。MRI提示：小脑扁桃体下疝并C2-4脊髓空洞形成（图1）。

图1.术前MRI显示小脑扁桃体下疝伴颈髓空洞形成

患儿于2019-7-29在全麻下行“后正中入路枕下骨减压+扁桃体电凝还纳+硬膜扩张成形术”。术后患儿恢复好，无新发症状、体征。术后6个月门诊复查，见小脑扁桃体还纳满意，脊髓空洞显著缓解（图2、3）。

图2. 术后6月（图右）复查头颈MR，提示小脑扁桃体还纳入颅，脊髓空洞明显缩小。

图3. 术后6月（图右）复查头颈MR，提示小脑扁桃体还纳满意，枕大孔区结构拥挤得到有效缓解。

手术视频

1、充分枕下减压（扫描下图中二维码收看）

2、小脑扁桃体电凝还纳（扫描下图中二维码收看）

3、硬膜扩张成型（扫描下图中二维码收看）

作者简介

宫剑，教授，主任医师，博士研究生导师，北京天坛医院小儿神经外科病区副主任（主持工作），全面负责病区医疗，科研，教学工作。主要研究方向：1.儿童颅内肿瘤；2.儿童先天性疾患。目前主持科技部十三五、国家自然科学基金等多项课题，国内外专业杂志发表医学论著30余篇。目前担任中国医师协会神经外科医师分会小儿专家委员会副主任委员，中国医药教育协会小儿神经外科分会副主任委员。

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