脊髓栓系综合征

脊髓栓系综合征 【概述】 脊髓栓系综合征(tethered cord syndrome，TCS)系脊髓圆锥以下终丝或马尾固定于椎管，于脊柱生长期中，牵拉脊髓圆锥不能向头侧移动而产生脊髓或圆锥牵拉损害症状之综合征。而脊髓纵裂(diastematomyelia)则系脊髓被椎管内骨性纵隔分为两半并产生脊髓压迫症状之疾病，部分病人脊髓纵裂与脊髓栓系综合征同时存在，故二者分两节描述。 【病理与病理生理】 1(终丝粗大并固定于骶椎者 病理改变是终丝拉紧脊髓成低位，圆锥位于腰水平或2以下，部分马尾纤维可能粘连于终丝之上。 2(先天性疾病 主要是脊髓脊膜或马尾脊膜膨出，手术后局部粘连将马尾固定于椎管内手术区，因脊膜膨出大多在幼儿时行手术治疗，在发育成长中，马尾固定脊髓不能正常向上移位，而致脊髓低位，脂肪瘤在腰骶部分布非常广泛，向上可至圆锥，向下至骶尾部，向外至皮下，硬膜外、硬膜下与马尾神经广泛粘连，马尾分散在脂肪瘤之中，分离甚为困难。 3(椎管内肿瘤 主要是皮样囊瘤和畸胎瘤，多发生在腰骶部，亦有在枕颈部，在腰骶部者，于长大过程中，逐渐压迫马尾，产生症状，马尾神经可深深粘连于肿瘤壁或包膜上。 【病因与发病机制】 1(终丝(filum terminale)肥大增粗固定于椎管壁 栓住圆锥(conus medullaris)不能向头端移动。 2(先天性疾病 如脊髓脊膜膨出(myelomeningocele)或脂肪脊髓脊膜膨出(lipomyelomeningocele)，病变可以固定终丝或马尾，而于病儿生长过程中产生脊髓栓系综合征，或是于幼儿时期做脊髓脊膜膨出手术，术后局部粘连而固定圆锥产生脊髓栓系综合征。Kanev与Bierbrauer报道一组177例脂肪脊髓脊膜膨出，观察到脊髓神经功能与年龄的关系，在初检时没有神经症状占37,，于年岁增长中发生神经症状，于脂肪脊髓脊膜膨出修补手术与栓系综合征松解后神经症状缓解。 3(先天畸形或椎管内肿瘤 如脊椎闭合不全(dysraphism)的先天性皮肤瘘道，可有纤维索条或瘘道连通至椎管内，产生脊髓栓系综合征，Wang等报道5例，先天性皮肤瘘道(congenital dermal sinus，CDS)与椎管内相连，椎管内病变为皮样或表皮样囊肿(dermoid or epidermoid cyst)亦可为畸胎瘤(teratoma)，多发生在腰骶部，亦在枕颈部，极少在胸椎，脊髓皮肤瘘道常是此病的提示。 4(成人隐性脊椎闭合不全(dysraphism) Klekamp等报道23例脊椎闭合不全畸形，于成人发生神经症状，其中4例脊膜膨出(meningocele)与16例错构瘤(hamartomas)，年龄23~67岁，平均39岁，发生脊髓症状。其中19例症状加重，行手术治疗，此中11例application; infection and antibacterial drug of clinical application; transfusion refers to levy; nutrition support of adapted card and clinical application; common life support technology (as cycle breathing support, and nutrition support,) and emergency technology of application; common guardianship instrument using. Look: shock, cardiac respiratory arrest and acute organ failure, systemic inflammatory response syndrome and severe infection and multiple organ dysfunction syndrome (MODS), severe disturbance of body fluid environment such as critical theory and progress of the illness. 2. basic requirements (1) species and cases of study requirements: severe pneumonia in disease-disease myocardial infarction ... 3. high requirements (1) learning disease species: disease species disease species image radiation: rheumatic heart congenital heart intestinal Crohn's (Crohn) disease intestinal tuberculosis bile duct cancer chronic pancreatic inflammatory urinary system stone urinary system tumor adrenal disease thyroid disease cranial within infection nervous system tumor nuclear medical: Digest road bleeding explicit like brain blood flow perfusion explicit like testicular blood pool explicit like salivary glands explicit like (2) clinical knowledge, and skills requirements: understand various image check 错构瘤有脊髓栓系综合征。 脊髓栓系综合征与圆锥位置及活动度的关系:Warder与Oakes在12年间观察73例脊髓栓系综合征病人，其中圆锥在腰 ~腰 椎间盘以下者(低位)60例，在腰 ~腰 椎间1212盘以上者13例(正常位置)。其隐性脊椎闭合不全的皮肤瘘道，脊椎畸形及其他畸形的发生率在二组相同。故圆锥正常位置亦可发生脊髓栓系综合征。 四类病因，在儿童和成人还有些不同，徐林等(1999)报道72例TCS，68例为15岁以下儿童，其病因是曾修补脊膜膨出32例，有脂肪瘤者38例，脊髓纵裂8例，脊髓空洞4例，极少有单纯终丝肥大固定，以先天性疾病为主，Huttmann等(2001)报道54例成人TCS，其患者脂肪瘤32例，终丝粗而紧28例，脊髓纵裂和术后粘连12例。 【诊断要点】 诊断要点概述 依据检查，做出脊髓栓系综合征的诊断，并无困难，再者有脊髓栓系综合征病史，曾行手术治疗者，亦有相当数量患者症状再发，Herman等报道做过脊髓脊膜膨出或脂肪脊髓脊膜膨出的患者中，约有2/5可再出现脊髓栓系综合征，他们从1981~1988年做341例脊髓栓系松解患者中，有153例又出现脊髓栓系综合征。 临床表现 1(发病年龄 儿童TCS发病多在幼儿时期以4岁左右发病者最多，徐林组的72例中2岁以下4例，2~6岁34例，7~10岁20例，11~15岁8例，16~25岁6例，男44例，女28例。成人发病早晚不等，有的在儿童时已有症状，成人后加重，亦有成人后发病者，Huttmann等54例成人TCS，17例儿童时无症状，8年后才出现症状，39例儿童时有症状，至成人加重。 Van-Leeuwen等(2001)报道57例成人TCS，年龄19~75岁，平均30~37岁出现症状，并作出诊断。 2(症状 主要为下肢感觉和运动症状、足部畸形或溃疡、泌尿系症状、肛门括约肌症状等。 徐林等报道72例儿童TCS的症状为:下肢感觉障碍44例，肌萎缩68例，一侧或双侧下肢腰运动障碍56例，小腿或足部畸形48例，足部溃疡12例，尿失禁、肛门失禁60例等，肾积水16例。 成人症状则主要为下肢感觉障碍，运动力弱或缺失，但足部畸形少见，括约肌症状多见，肛门区感觉减弱或丧失。 检查: 1(神经学检查 下肢感觉障碍区肌力减弱、走路不稳、腱反射改变，会阴区肛门感觉及肛门括约肌功能丧失，尿失禁，即可以诊断下神经单位损害，亦可出现上神经单位受损症状。 application; infection and antibacterial drug of clinical application; transfusion refers to levy; nutrition support of adapted card and clinical application; common life support technology (as cycle breathing support, and nutrition support,) and emergency technology of application; common guardianship instrument using. Look: shock, cardiac respiratory arrest and acute organ failure, systemic inflammatory response syndrome and severe infection and multiple organ dysfunction syndrome (MODS), severe disturbance of body fluid environment such as critical theory and progress of the illness. 2. basic requirements (1) species and cases of study requirements: severe pneumonia in disease-disease myocardial infarction ... 3. high requirements (1) learning disease species: disease species disease species image radiation: rheumatic heart congenital heart intestinal Crohn's (Crohn) disease intestinal tuberculosis bile duct cancer chronic pancreatic inflammatory urinary system stone urinary system tumor adrenal disease thyroid disease cranial within infection nervous system tumor nuclear medical: Digest road bleeding explicit like brain blood flow perfusion explicit like testicular blood pool explicit like salivary glands explicit like (2) clinical knowledge, and skills requirements: understand various image check 2(畸形 如背部皮肤瘘道、肛门畸形、下肢畸形(如马蹄内翻足)、脊柱裂、脊髓脊膜膨出、脊柱侧弯等存在，常提示有脊髓栓系综合征存在。 3(X线检查 胸腰骶椎平片，有无脊椎闭合不全、畸形。 4(MRI检查 可显示脊髓栓系综合征之表现，如粗大之终丝、低位圆锥、脊髓脊膜膨出脂肪瘤等，脊髓及马尾紧张紧贴椎管后壁有皮样囊瘤、脊髓积水(hydromyelia)及脊髓纵裂者约占30,(图1)。 图1 脊髓栓系 患者女，18岁，排尿控制困难，夜尿，MRI腰骶段有小脊膜马尾膨出， 脊髓栓系，牵拉圆锥下移达腰 平面，与脂肪增生混在 4 5(下肢肛门括约肌电图、尿动力学检查 有助于脊髓圆锥损害的诊断。 【治疗概述】 手术治疗 许多作者指出，出现脊髓栓系综合征症状者应尽早手术治疗，对伴有脊髓脊膜膨出者，不必等发生脊髓栓系综合征症状，而应早期治疗。 切除增粗并固定的终丝，解除对脊髓圆锥的栓系。对有先天畸形或疾病的病例，如脊髓脊膜膨出、皮样囊瘤等，应同时切除或修复。在近正常的终丝与马尾神经不易区别，肿瘤及脊髓脊膜膨出常与马尾神经粘连一起，为避免术中分离或切除肿瘤时损伤马尾，可用于术中诱发电位监护。病变区的神经根应从椎间孔处向近侧松解确认与终丝分开，在脊膜application; infection and antibacterial drug of clinical application; transfusion refers to levy; nutrition support of adapted card and clinical application; common life support technology (as cycle breathing support, and nutrition support,) and emergency technology of application; common guardianship instrument using. Look: shock, cardiac respiratory arrest and acute organ failure, systemic inflammatory response syndrome and severe infection and multiple organ dysfunction syndrome (MODS), severe disturbance of body fluid environment such as critical theory and progress of the illness. 2. basic requirements (1) species and cases of study requirements: severe pneumonia in disease-disease myocardial infarction ... 3. high requirements (1) learning disease species: disease species disease species image radiation: rheumatic heart congenital heart intestinal Crohn's (Crohn) disease intestinal tuberculosis bile duct cancer chronic pancreatic inflammatory urinary system stone urinary system tumor adrenal disease thyroid disease cranial within infection nervous system tumor nuclear medical: Digest road bleeding explicit like brain blood flow perfusion explicit like testicular blood pool explicit like salivary glands explicit like (2) clinical knowledge, and skills requirements: understand various image check 膨出者应保留蛛网膜，以免粘连，硬膜有缺损者，以椎旁筋膜修补缝合。 Schneider等报道用激光多普勒流量计(laser-doppler flow metry)在术中进行监测脊髓的松解否，当脊髓被栓系牵拉时。脊髓的血流量减少，当栓系解除后，脊髓不受牵拉，其微循环血流量恢复，脊髓已不受牵拉，作者做了10例儿童，在栓系松解之前，平均血流量为12.6ml(2min?100g组织)，而栓系松解之后，平均血流量恢复至29.4ml/(min?100g组织)，此亦说明栓系综合征时脊髓的病理生理改变。 术中松解马尾神经可用CO激光，为防止术后脊髓圆锥与椎管壁粘连，再发生脊髓栓系2 综合征，Inolle等用Gore-Tex外科膜覆盖脊髓并与侧方硬膜缝合固定，做12例，随诊23个月至7年，没有症状复发，并且又经MRI检查未发现手术区脊髓粘连，认为此方法对治疗及预防脊髓栓系综合征是有效的。 【预后】 早期治疗者，恢复较好。还因原始疾病治疗的难易而不同，例如脊髓脊膜膨出，如脊膜膨出并不严重，马尾粘连较轻，可以较彻底分离而不增加损伤，则手术效果较好，如脊膜膨出与马尾粘连严重，分离困难，则效果较差。在儿童甚至幼儿时期已功能丧失之马尾损害，则手术后很难恢复，而近来加重之神经症，如疼痛、麻木、夜遗尿，椎间盘突出症状等，则常可以恢复，即便某些神经症状不能恢复，但手术松解后，停止进行，亦应是治疗的适应证。Herman等再手术的153例脊髓栓系综合征中，100例为修复脊膜膨出之后，而53例为修复脂肪脊髓脊膜膨出之后，前者平均年龄6岁，后者为8岁，其症状为肌力减弱、走路障碍、脊柱侧弯、尿失禁与骨科其他畸形，手术做栓系松解，所见到的病理改变有皮样囊瘤、脊髓积水(hydromgelia)、终丝紧张及脊髓纵裂。随诊平均4年，脊髓脊膜膨出患者中93,症状停止进行或改善，7,症状仍进行，而所有脂肪脊髓脊膜膨出患者症状稳定或有改善。 Reigel等报道伴有脊椎裂的脊髓栓系综合征患者，手术松解后的脊柱曲线改变，包括脊柱侧弯、驼背及前突(lordosis)，做过栓系松解手术的262例中，经20年随访，216例做2 369次脊柱X线片检查，另外，74例无脊髓栓系病史者，亦做系列X线片检查作为对照结果:腰、骶部病变的脊髓栓系综合征，于松解术后，其脊柱侧弯可停止或减慢进行，而胸椎病变者，术后脊柱侧弯并不停止进展。腰~腰病变者，栓系松解后，其腰椎前突发生13 改变，但腰及骶部病变者，栓系松解后，并不改变其正常腰前突的进展。栓系松解术后4、5 可减少驼背的发生或进展。 脊髓的活动性与脊髓栓系综合征的后果有关，Johnson与Levy对32例脊椎闭合不全的儿童行MRI检查观察脊髓的纵向活动，17例无症状儿童，其脊髓有正常或稍减少的活动，未行手术，而15例有脊髓栓系综合征症状和体征的儿童，其脊髓活动明显减少者，其症状无改善，而脊髓活动轻度或中度减少者，症状趋于改善，所有先前曾行脊髓脊膜膨出手术，脊髓活动明显减少，年龄,10岁者，其症状加重。 避免腰椎不稳和神经症状加重的主要措施是有限切除，切断固定的终丝是手术主要目的，对皮样囊瘤、畸胎瘤，应争取全切除，但如马尾粘连囊壁深层，剥离困难时，应保留一部分囊壁或将囊开口于硬膜外，对脊膜膨出术后粘连和脂肪瘤应有限分离，减压为主。 application; infection and antibacterial drug of clinical application; transfusion refers to levy; nutrition support of adapted card and clinical application; common life support technology (as cycle breathing support, and nutrition support,) and emergency technology of application; common guardianship instrument using. Look: shock, cardiac respiratory arrest and acute organ failure, systemic inflammatory response syndrome and severe infection and multiple organ dysfunction syndrome (MODS), severe disturbance of body fluid environment such as critical theory and progress of the illness. 2. basic requirements (1) species and cases of study requirements: severe pneumonia in disease-disease myocardial infarction ... 3. high requirements (1) learning disease species: disease species disease species image radiation: rheumatic heart congenital heart intestinal Crohn's (Crohn) disease intestinal tuberculosis bile duct cancer chronic pancreatic inflammatory urinary system stone urinary system tumor adrenal disease thyroid disease cranial within infection nervous system tumor nuclear medical: Digest road bleeding explicit like brain blood flow perfusion explicit like testicular blood pool explicit like salivary glands explicit like (2) clinical knowledge, and skills requirements: understand various image check application; infection and antibacterial drug of clinical application; transfusion refers to levy; nutrition support of adapted card and clinical application; common life support technology (as cycle breathing support, and nutrition support,) and emergency technology of application; common guardianship instrument using. Look: shock, cardiac respiratory arrest and acute organ failure, systemic inflammatory response syndrome and severe infection and multiple organ dysfunction syndrome (MODS), severe disturbance of body fluid environment such as critical theory and progress of the illness. 2. basic requirements (1) species and cases of study requirements: severe pneumonia in disease-disease myocardial infarction ... 3. high requirements (1) learning disease species: disease species disease species image radiation: rheumatic heart congenital heart intestinal Crohn's (Crohn) disease intestinal tuberculosis bile duct cancer chronic pancreatic inflammatory urinary system stone urinary system tumor adrenal disease thyroid disease cranial within infection nervous system tumor nuclear medical: Digest road bleeding explicit like brain blood flow perfusion explicit like testicular blood pool explicit like salivary glands explicit like (2) clinical knowledge, and skills requirements: understand various image check