神经外科“第一”畸形：小脑扁桃体下疝畸形—新闻

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font: 12px 宋体; float: right"> 选择字号：<span onclick="showsize(12)" style="background-color: #587c19; color: #fff; font-size: 12px; cursor: pointer">小</span> <span onclick="showsize(14)" style="background-color: #587c19; color: #fff; font-size: 12px; cursor: pointer">中</span> <span onclick="showsize(16)" style="background-color: #587c19; color: #fff; font-size: 12px; cursor: pointer"> 大</span> </div> </td> </tr> <tr> <td align="center"> <div id="content1" style=" padding:15px; text-align: left; line-height: 24px; word-wrap: break-word;word-break:break-all" class="f14"> <table width="100%" border="0" cellspacing="0" cellpadding="0"> <tr> <td height="20"> </td> </tr> <tr> <td align="center" valign="middle" class="style1" style="font-size: 13px; color: #333333; font-family: 宋体; line-height: 20px"> <b></b></td> </tr> <tr> <td align="center" class="style1" style="font-size: 22px; color: #587c19; font-family: 黑体; line-height: 30px"> 神经外科“第一”畸形：小脑扁桃体下疝畸形</td> </tr> <tr> <td align="center" valign="middle" class="style1" style="font-size: 13px; color: #333333; font-family: 宋体; line-height: 20px"> <b></b></td> </tr> </table> <p > </p><p style=";; margin: 0px 0px 26px; text-align: justify; word-break: break-all;">有一种病，如果不是专科医生，绝大多数人甚至医生都知之甚少。发病时，患者极易被误诊为颈椎病，但其危害性比颈椎病更大，是发病率和危害性综合排名第一的先天性神经畸形，轻则疼痛麻木，出现感觉障碍，重则肌肉萎缩、行走困难，更有甚者会瘫痪、呼吸困难和吞咽困难等，危及生命。这种病就是小脑扁桃体下疝畸形。</p> <div style="text-align: center;" class="ueditorOption ueditorOptionModel" contenteditable="true" id="0"> <img src="https://rmtzx.sciencenet.cn//kxwsprint/66f5245ce4b03b5da6d17567.png" data-wenge-id="124901" wosname="66f5245ce4b03b5da6d17567.png" size="488436" opaid="wenge-rxi7xqy6s3q0/" data-mce-src="https://rmtzx.sciencenet.cn//kxwsprint/66f5245ce4b03b5da6d17567.png" id="img-null"> <span style="display:block;color: #999;font-size:14px;margin:0;text-align:center;line-height:20px;" class="img_caption" placeholder="请输入图说">作者供图</span> </div> <p class="timeLine_wg" style="height:20px;"><span style="display:none"><span contenteditable="false" style="height: 0px; padding: 0px; margin: 0px; display: block; z-index: 9999; color: rgb(255, 255, 255); font-size: 0px; line-height: 0px; position: absolute; border-top: 1px dashed rgb(181, 189, 202); user-select: none; left: 0px; right: 0px; top: 8px;"><span class="timeIdentification" style="width:0px;height:0px;padding:0px;margin:0px;display:block;z-index:9999;color:#fff;position:absolute;font-size: 0px;line-height:0px;border-color:transparent;display:block;border-style:solid;right:0px;border-right-color:#B5BDCA;border-width:8px 8px 8px 0;top:-8px"> </span><span style="width:0px;height:0px;padding:0px;margin:0px;display:block;z-index:9999;color:#fff;position:absolute;font-size: 0px;line-height:0px;border-color:transparent;display:block;border-style:solid;left:0px;border-left-color:#B5BDCA;border-width:8px 0 8px 8px;top:-8px"> </span><span class="hide_time_wg" title="在这插入段落" contenteditable="false" style="width:0px;height:0px;padding:0px;margin:0px;display:block;z-index:9999;color:#fff;position:absolute;font-size: 22px;line-height:17px;height:17px;text-align: center;width:17px;right:17px;background:#B5BDCA;cursor:pointer;top:-8px; border-radius: 2px;" onclick="timeLine(this)">?</span></span></span></p> <p style=";; margin: 0px 0px 26px; text-align: justify; word-break: break-all;">所谓小脑扁桃体，并不是指口腔里的“扁桃体”，而是因小脑的下部外形像扁桃体而得名。小脑扁桃体正常情况下位于颅腔内，但如果它向下进入颈椎管内超过枕骨大孔5mm，就叫做小脑扁桃体下疝。这是一种异于常人的疾病状态，因此被称为小脑扁桃体下疝畸形，又称Chiari畸形。</p> <p style=";; margin: 0px 0px 26px; text-align: justify; word-break: break-all;">Chiari畸形通常在青春期或成年期才被诊断出来，但它其实是一种先天性疾病，大约每1000个新生儿中就会出现1个Chiari畸形患儿。</p> <p style=";; margin: 0px 0px 26px; text-align: justify; word-break: break-all;"><strong>Chiari畸形导致神经压迫和脊髓空洞</strong></p> <p style=";; margin: 0px 0px 26px; text-align: justify; word-break: break-all;">在神经外科，急性脑疝是有致死性的，需要争分夺秒地紧急治疗，而Chiari畸形由于起病缓慢，进展较慢，因此会给神经一个耐受过程，等突破代偿临界后就会出现症状，而且往往是逐渐恶化，但有些患者在短时间内会发生急性加重的情况。</p> <div style="text-align: center;" class="ueditorOption ueditorOptionModel" contenteditable="true" id="1"> <img src="https://rmtzx.sciencenet.cn//kxwsprint/66f524c2e4b03b5da6d17569.png" data-wenge-id="124902" wosname="66f524c2e4b03b5da6d17569.png" size="82170" opaid="wenge-345nq8obbbn0/" data-mce-src="https://rmtzx.sciencenet.cn//kxwsprint/66f524c2e4b03b5da6d17569.png" id="img-null"> <span style="display:block;color: #999;font-size:14px;margin:0;text-align:center;line-height:20px;" class="img_caption" placeholder="请输入图说">作者供图</span> </div> <p class="timeLine_wg" style="height:20px;"><span style="display:none"><span contenteditable="false" style="height: 0px; padding: 0px; margin: 0px; display: block; z-index: 9999; color: rgb(255, 255, 255); font-size: 0px; line-height: 0px; position: absolute; border-top: 1px dashed rgb(181, 189, 202); user-select: none; left: 0px; right: 0px; top: 8px;"><span class="timeIdentification" style="width:0px;height:0px;padding:0px;margin:0px;display:block;z-index:9999;color:#fff;position:absolute;font-size: 0px;line-height:0px;border-color:transparent;display:block;border-style:solid;right:0px;border-right-color:#B5BDCA;border-width:8px 8px 8px 0;top:-8px"> </span><span style="width:0px;height:0px;padding:0px;margin:0px;display:block;z-index:9999;color:#fff;position:absolute;font-size: 0px;line-height:0px;border-color:transparent;display:block;border-style:solid;left:0px;border-left-color:#B5BDCA;border-width:8px 0 8px 8px;top:-8px"> </span><span class="hide_time_wg" title="在这插入段落" contenteditable="false" style="width:0px;height:0px;padding:0px;margin:0px;display:block;z-index:9999;color:#fff;position:absolute;font-size: 22px;line-height:17px;height:17px;text-align: center;width:17px;right:17px;background:#B5BDCA;cursor:pointer;top:-8px; border-radius: 2px;" onclick="timeLine(this)">?</span></span></span></p> <p style=";; margin: 0px 0px 26px; text-align: justify; word-break: break-all;">Chiari畸形的危害主要包括两点——神经压迫和脊髓空洞。以Y字路打比方，正常颈椎管内只能有脊髓神经（红箭头），若小脑扁桃体（蓝箭头）进入颈椎管内就会造成拥挤，类似公路上的堵车甚至撞车，这会导致患者因脊髓神经压迫和脑脊液循环不畅，出现压迫症状和脊髓空洞。</p> <p style=";; margin: 0px 0px 26px; text-align: justify; word-break: break-all;">那何为脊髓空洞呢？通俗地讲，脊髓空洞是指脊髓内出现了“积水”。正常情况下，脊髓中央有一根细长的管腔，即中央管，内部呈密闭状态。但若里面有积水（其实是脑积液）并造成脊髓扩张，就称作脊髓空洞。</p> <div style="text-align: center;" class="ueditorOption ueditorOptionModel" contenteditable="true" id="2"> <img src="https://rmtzx.sciencenet.cn//kxwsprint/66f524fbe4b03b5da6d1756b.jpg" data-wenge-id="124903" wosname="66f524fbe4b03b5da6d1756b.jpg" size="37266" opaid="wenge-zcnqvheymq80/" data-mce-src="https://rmtzx.sciencenet.cn//kxwsprint/66f524fbe4b03b5da6d1756b.jpg" id="img-null"> <span style="display:block;color: #999;font-size:14px;margin:0;text-align:center;line-height:20px;" class="img_caption" placeholder="请输入图说">作者供图</span> </div> <p class="timeLine_wg" style="height:20px;"><span style="display:none"><span contenteditable="false" style="height: 0px; padding: 0px; margin: 0px; display: block; z-index: 9999; color: rgb(255, 255, 255); font-size: 0px; line-height: 0px; position: absolute; border-top: 1px dashed rgb(181, 189, 202); user-select: none; left: 0px; right: 0px; top: 8px;"><span class="timeIdentification" style="width:0px;height:0px;padding:0px;margin:0px;display:block;z-index:9999;color:#fff;position:absolute;font-size: 0px;line-height:0px;border-color:transparent;display:block;border-style:solid;right:0px;border-right-color:#B5BDCA;border-width:8px 8px 8px 0;top:-8px"> </span><span style="width:0px;height:0px;padding:0px;margin:0px;display:block;z-index:9999;color:#fff;position:absolute;font-size: 0px;line-height:0px;border-color:transparent;display:block;border-style:solid;left:0px;border-left-color:#B5BDCA;border-width:8px 0 8px 8px;top:-8px"> </span><span class="hide_time_wg" title="在这插入段落" contenteditable="false" style="width:0px;height:0px;padding:0px;margin:0px;display:block;z-index:9999;color:#fff;position:absolute;font-size: 22px;line-height:17px;height:17px;text-align: center;width:17px;right:17px;background:#B5BDCA;cursor:pointer;top:-8px; border-radius: 2px;" onclick="timeLine(this)">?</span></span></span></p> <p style=";; margin: 0px 0px 26px; text-align: justify; word-break: break-all;">脊髓空洞的危害相比神经受压迫更加严重和隐匿，其症状和体征包括肢体麻木、无力、肌肉萎缩（尤其是大小鱼际肌）和夏科氏关节。夏科氏关节又称神经性关节病，表现为无痛性关节肿胀、骨折、关节脱位或半脱位、关节稳定性下降、关节活动超常以及关节畸形，多累及肩部，其次是肘和腕关节。</p> <p style=";; margin: 0px 0px 26px; text-align: justify; word-break: break-all;">脊髓空洞会导致脊髓高张力，造成神经脱髓鞘改变、神经纤维断裂，长时间的脊髓高张力又会引起脊髓神经元细胞凋亡，使脊髓萎缩，造成不可逆性神经损伤。这就是许多病人手术后脊髓空洞依然难以治愈的主要原因。</p> <p style=";; margin: 0px 0px 26px; text-align: justify; word-break: break-all;">Chiari畸形最常见的症状是枕下头痛或咳嗽引起的头痛，另外包括肌肉无力、四肢麻木、失去平衡、协调性差和关节僵硬等。在一项定性报告364名Chiari患者症状的研究中，57%出现头疼头晕，52%出现肢体不平衡，57%出现肌肉无力，30%出现痉挛。</p> <p style=";; margin: 0px 0px 26px; text-align: justify; word-break: break-all;"><strong>Chiari畸形目前无法治愈</strong></p> <p style=";; margin: 0px 0px 26px; text-align: justify; word-break: break-all;">目前，Chiari畸形无法治愈，但通过减压手术可以缓解相关症状。减压手术的适应证包括严重头痛、扁桃体下降> 5mm和/或椎管中出现有威胁的脊髓空洞。而根据小脑扁桃体下疝和脑干下疝的长度以及是否存在脊髓空洞，患者的手术方法也有所不同，但无论外科医生选择何种减压入路手术，总体目标都是恢复脑脊液在脑和椎管之间的正常流动。</p> <p style=";; margin: 0px 0px 26px; text-align: justify; word-break: break-all;">通俗地讲，就是一句话——“有下疝无症状可观察，有下疝有症状要手术，有下疝有空洞早手术”。具体而言，无论是在体检中还是其他任何情况下，如果发现自己有Chiari畸形但无症状，此时若无脊髓空洞，可继续观察；若有脊髓空洞，建议限期手术；如果有Chiari畸形且有症状，那么无论有无脊髓空洞都建议择期手术治疗。</p> <p style=";; margin: 0px 0px 26px; text-align: justify; word-break: break-all;">原因很简单。对于有症状的Chiari畸形，无论有无脊髓空洞都意味着患者脊髓神经受到了压迫，需要手术减压。而对于无症状的Chiari畸形，有脊髓空洞意味着患者存在脑积液循环障碍，这也需要手术治疗，因为空洞形成后脊髓处于高张力状态，类似吹起的气球。而无脊髓空洞意味着患者无神经压迫、无脑积液循环受阻，因此只需定期复查和观察。</p> <p style=";; margin: 0px 0px 26px; text-align: justify; word-break: break-all;"><strong>合理的手术方案有助于患者获益</strong></p> <p style=";; margin: 0px 0px 26px; text-align: justify; word-break: break-all;">在预后方面，由于Chiari畸形无法治愈，因此无症状、无脊髓空洞的Chiari畸形患者可能需终生带病生存，但生存质量基本不受任何影响。无症状有脊髓空洞、有症状无脊髓空洞和有症状有脊髓空洞的病人，若不做手术，通常病情会逐渐恶化和加重。大宗数据显示，手术治疗可使至少80%的患者病情有所改善。</p> <p style=";; margin: 0px 0px 26px; text-align: justify; word-break: break-all;">为什么还有20%的患者手术效果差甚至病情加重？这其中的原因众多。既包括病人的原因，比如术前病程长、病情重、未及时手术治疗、术后康复不当等；也包括术者的原因，比如手术理念、手术方法和手术技术欠缺、术后出现并发症等。值得一提的是，此类疾病在医生间的认知理念差异较大，导致手术治疗方法众多，目前并未有统一的术式，这也造成了患者术后疗效的差异化。</p> <p style=";; margin: 0px 0px 26px; text-align: justify; word-break: break-all;">基于这两点，建议广大患者重视Chiari畸形，一旦发现，及时到神经外科就诊。另外，术者也要提升手术理念和手术技术，兼顾患者的自身情况，做到个体化治疗。</p> <p style=";; margin: 0px 0px 26px; text-align: justify; word-break: break-all;">很多患者也想知道，如果第一次手术治疗失败了，还有没有再次手术治疗的机会。这取决于患者前次手术方式是否合适、减压是否完全、并发症控制是否到位等，需要综合评判能否“翻修”。所以，有些患者还是有机会的。我曾经就遇到1例因寰枢椎脱位导致Chiari畸形和脊髓空洞的患者，他第一次手术只做了减压而未做复位固定，导致手术无效，第二次我为他做了复位固定和小脑上托（修补）手术。术后，患者小脑扁桃体下疝和脊髓空洞明显好转。因此，正确的手术方案极其重要。</p> <p style=";; margin: 0px 0px 26px; text-align: justify; word-break: break-all;">总而言之，Chiari畸形是一种先天发育性疾病，既是进展性疾病又是致残性疾病，但也是一种可治性疾病，手术效果较好、手术风险较低。延迟手术，尤其对于合并脊髓空洞的患者而言，不仅手术效果差，而且会造成不可逆性神经功能障碍。因此，早发现、早手术依然是目前的国际主流推荐策略，而合理的手术方案和精湛的手术技术有助于患者从手术中获益。</p> <p class="lastPadding" style=";; margin: 0px 0px 26px; text-align: justify; word-break: break-all;">（作者单位：北京清华长庚医院神经外科）</p><div> </div><div style="text-indent:0em;font-weight:bold;font-size:13px;border:solid 1px #333 ;padding:10px;" class="bq">版权声明：凡本网注明“来源：中国科学报、科学网、科学新闻杂志”的所有作品，网站转载，请在正文上方注明来源和作者，且不得对内容作实质性改动；微信公众号、头条号等新媒体平台，转载请联系授权。邮箱：shouquan@stimes.cn。</div> <div style="border-bottom: solid 1px #bfc89d; 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