Chiari Ⅰ型畸形合并脊髓空洞症的显微外科治疗

目的 研究ChiariⅠ型畸形合并脊髓空洞症的显微手术治疗方法。方法 枕下正中入路，咬开枕骨大孔后缘及寰椎后弓，显微镜下切开硬脑脊膜，软脑膜下切除下疝的小脑扁桃体，重建第四脑室正中孔与两侧小脑延髓外侧池交通、开放脊髓中央管口，修补硬脑脊膜。结果 36例手术均顺利完成，术后恢复平稳、无感染，无死亡。病人肌力。温、痛感觉及肌张力均恢复良好。下疝的小脑扁桃体均消失。随访MRI示，6例空洞症消失，脊髓接近正常；30例脊髓空洞腔明显缩小。结论 采用显微手术切除下疝的小脑扁桃体，开放脊髓中央管口，恢复枕大池结构和功能，是治疗ChiariⅠ型畸形合并脊髓空洞症的有效方法。     

Syringo-Subarachnoid-Peritoneal Shunt Using T-Tube for Treatment of Post-Traumatic Syringomyelia

Various surgical procedures for the treatment of post-traumatic syringomyelia have been introduced recently, but most surgical strategies have been unreliable. We introduce the concept and technique of a new shunting procedure, syringo-subarachnoid-peritoneal shunt. A 54-year-old patient presented to our hospital with a progressive impairment of motion and position sense on the right side. Sixteen years before this admission, he had been treated by decompressive laminectomy for a burst fracture of L1. On his recent admission, magnetic resonance (MR) imaging studies of the whole spine revealed the presence of a huge syrinx extending from the medulla to the L1 vertebral level. We performed a syringo-subarachnoid-peritoneal shunt, including insertion of a T-tube into the syrinx, subarachnoid space and peritoneal cavity. Clinical manifestations and radiological findings improved after the operation. The syringo-subarachnoid-peritoneal shunt has several advantages. First, fluid can communicate freely between the syrinx, the subarachnoid space, and the peritoneal cavity. Secondly, we can prevent shunt catheter from migrating because dural anchoring of the T-tube is easy. Finally, we can perform shunt revision easily, because only one arm of the T-tube is inserted into the intraspinal syringx cavity. We think that this procedure is the most beneficial method among the various shunting procedures.     

Chiari 1.5 Malformation : An Advanced Form of Chiari I Malformation

The Chiari 1.5 malformation is defined as a tonsillar herniation within a Chiari I malformation with additional caudal descent of the brainstem through the foramen magnum. We describe a patient with Chiari I malformation who evolved to Chiari 1.5 malformation during longitudinal follow-up. A 15-year-old girl presented with neck pain during exercise for two years. She had been diagnosed with Chiari I malformation with mild hydrocephalus after minor cervical trauma at the age of six years. At that time, she was asymptomatic. After she complained of aggravated neck pain, neuroimaging (nine years after first imaging) revealed caudal descent of the brainstem and syringomyelia in addition to progression of tonsillar herniation. Posterior fossa decompressive surgery resulted in complete resolution of neck pain. Based on neuroimaging and operative findings, she was diagnosed as Chiari 1.5 malformation. Neuroimaging performed seven months after surgery showed an increased anterior-posterior diameter of the medulla oblongata and markedly decreased syringomyelia. This case demonstrates progressive developmental process of the Chiari 1.5 malformation as an advanced form of the Chiari I malformation.     

枕颈减压、脊髓空洞穿刺抽液治疗Chiari畸形合并脊髓空洞症

目的：探讨一种治疗Chiari畸形合并脊髓空洞症的新手术方法。方法：61例经MRI诊断为Chiari畸形合并脊髓空洞症患,均采用枕颈减压加脊髓空洞空刺抽液术治疗。结果：全部病例术后临床症状,体征均有不同程度改善;随访48例（术后1－5年）,明显转好45例,好转3例,其中8例术后2－4年经MRI复查,脊髓空洞消失2例,空洞明显缩小5例,轻微缩小1例,手术无并发症,无死亡,结论：枕颈减压加脊髓空洞空刺抽液术治疗Chiari畸形合并脊髓空洞症是一种有效的方法。     

空洞分流对ChiariⅠ畸形伴脊髓空洞症近远期疗效的影响

目的比较chiariⅠ畸形伴脊髓宅洞症空洞分流与否对近远期疗效的影响，探讨脊髓空洞分流的必要性和临床意义，为临床选择手术方法提供依据．方法回顾性分析21例ChiariⅠ畸形伴脊髓空洞症手术治疗病例，观察8例经后颅窝减压 颈1、2或3椎板切开 硬脑膜修补(A组)与13例同时行脊髓空洞-蛛网膜下腔分流病人(B组)术后早期、随访半年以上临床症状与体征的变化，并采用Kamofsksyr行为能力评分法对两组病例远期疗效进行评定．同时比较术后空洞变化与临床症状的关系。结果A组术后早期症状改善5例(62．5％)。稳定3例，无症状恶化者，随访4例．按照Kamofsky，评分标准，临床症状改善3例，有效率75％(3／4)-B绀术后早期症状改善6例．稳定5例．恶化1例，有效率46.3％(6／13)；随访8例，临床症状改善7例，有效率87％(7/8)。随访MRI结果显示，B绀空洞缩小程度明显优于A组，多数空洞缩小者，症状好转，少数病例症状无变化结论后颅窝减压加颈1、2或3椎板切开及硬脑膜修补足治疗ChiariⅠ畸形伴脊髓空洞症的有效方法.加行空洞分流能有效缩小空洞体积，多数患者远期疗效优于未分流者，但部分病人存在无效或症状恶化可能。     

Diagnosis of Chiari I malformation and related syringomyelia: radiological and neurophysiological studies

Introduction The diagnosis of Chiari I malformation relies mainly on the anatomical demonstration of the abnormal position of the cerebellar tonsils below the foramen magnum, and of the associated bony anomalies of the posterior cranial fossa and craniocervical junction, as well as of the eventually-associated spinal cord cavitations. Consequently, the neuroradiological work-up plays a fundamental role in the definition of the malformation and in the follow-up of operated patients.Review The authors review the pertinent literature on the neuroradiology of the Chiari I malformation, with special regard to MRI, with the aim of providing the reader with an updated instrument for its diagnosis.     

Clinical features of Chiari I malformations

Background With the advent of MRI, the diagnosis of Chiari I malformation is being made in younger patients, often with minimal or no neurologic symptoms.Clinical features The symptoms and signs of a Chiari I malformation may be related to the compression of neural and/or dural structures at the cranio-cervical junction or may be related to the associated syringomyelia. Scoliosis is a particularly important and common finding in those children with syringomyelia.     

小脑扁桃体下疝并脊髓空洞症的诊治进展

小脑扁桃体下疝畸形也称Chiari畸形(Chiarimalformation,CM),是一种以小脑扁桃体向下疝入枕骨大孔为特征的先天性畸形,脊髓空洞症(syringomyelia,SM)为其常见的合并症。本文就CM并SM的诊治进展作一综述。一、CM脊髓空洞的发病机理1.CM的发病机理:Chiari畸形的发病学说很多,但均不能满意地解释一些问题。现较流行的学说为CM胚胎中胚层枕骨部体节发育不良,导致枕骨发育滞后,而小脑脑干发育正常出现后颅窝过度拥挤现象,从而疝出到椎管内[1]。研究表明病人后颅窝容积明显小于正常人是该病的特征性表现[2,3]。Badie等[4]测量病人和正常人…     

两种不同术式治疗Chiari畸形合并脊髓空洞症的疗效分析

目的 通过回顾性比较后颅窝成形术及枕大池成形术对Chiari畸形合并脊髓空洞症的治疗,明确两种不同术式治疗Chiari畸形合并脊髓空洞的疗效.方法 对收治的85例Chiari畸形合并空洞患者,其中39例患者行后颅窝成形术(后颅窝减压+硬脑膜成形);46例患者行枕大池成形术(后颅窝减压+硬脑膜成形+小脑扁桃体切除+蛛网膜粘连分解).结果 两组远期疗效相比有统计学意义,症状改善率分别为64%和90%,恶化19%和3%;术后脊髓空洞长度改变,两组比较有统计学意义.结论 枕大池成形术是治疗Chiari畸形合并脊髓空洞症较为合理的术式,疗效优于后颅窝成形术.     

Expansion of Chiari I-associated syringomyelia after posterior-fossa decompression

Chiari I malformation (CMI) is an abnormality that involves caudal herniation of the cerebellar tonsils into the foramen magnum. CMI has been shown to be closely associated with the development of syringomyelia (SM). Objects: Several theories have emerged to explain the apparent correlation between the existence of CMI with subsequent development of SM. However, the exact mechanism of the evolution of SM is still subject to controversy. We report here the case of a 12-year-old girl admitted to hospital with headache, vomiting, ataxia, and moderate pyramidal signs. Methods: Radiological evaluation revealed the presence of CMI, accompanied by a small SM. The patient underwent posterior fossa decompression and improved significantly. She was re-admitted 6 months later with clinical evidence of progressive spinal cord dysfunction. MR revealed gross expansion of the syrinx. Conclusions: This case raises questions regarding the pathophysiology of CMI and its association with SM. The case indicates the need for neurological and radiological follow-up for patients undergoing posterior fossa decompression due to CMI, even for those without an initial syrinx. This is the first report known to us of expansion of a syrinx following decompression of an associated CMI. Received: 16 January 2000     

Chiari畸形并脊髓空洞症的MRI分型及其治疗

对Ｃｈｉａｒｉ畸形并脊髓空洞症治疗方法及其效果的探讨。方法对１０３例Ｃｈｉａｒｉ畸形病例根据其ＭＲＩ征像分为Ａ、Ｂ、Ｃ三种类型。结果依据其类型采用不同的手术方式,并获得了较为满意的治疗效果。结论手术使延颈髓充分减压,可有效缓解临床症状,并对脊髓空洞症有明显的治疗作用。     

Chiari畸形并脊髓空洞的治疗与小脑扁桃体下疝分型

目的探讨Chiari畸形并脊髓空洞手术中,下疝的小脑扁桃体下舌分型与切除的标准。方法术中显微镜下观察32例下疝的小脑扁桃体压迫延髓的部位及其关系,结合MRI,提出下疝的小脑扁桃体分型,并根据分型切除小脑扁桃体下舌,切开脊髓中央管开口隔膜或活瓣,恢复第四脑室内脑脊液循环通畅。结果小脑扁桃体分型：①根据压迫部位分为：后方型27例,侧方型4例,侧前方型1例;②根据小脑扁桃体下疝程度分为：单侧压迫型11例,双侧压迫型21例。术后随访28例,MRI检查示下疝的小脑扁桃体均消失,第四脑室内脑脊液循环通畅,脊髓空洞明显缩小和消失;术后病人临床症状大多改善。结论下疝的小脑扁桃体根据其分型并切除,可为手术治疗Chiari畸形并脊髓空洞提供标准及依据。     

Chiari畸形合并脊髓空洞症的外科治疗

Chiari畸形又称小脑扁桃体下疝畸形.John Cleland于1883年首次描述了脑干、小脑移位变形和延髓空洞,Chiari分别于1891年、1895年将颅-椎移行区神经轴下移的程度进行了分类,1894年德国病理学家Arnold又作了更为详尽的描述,因此,1907年Schwalb和Gredig将此命名为ArnoldChiari畸形,而Sarnat和Williams则称其为Chiari畸形或描述表达为小脑扁桃体下疝畸形[1].     

Spontaneous resolution of isolated Chiari I malformation

Introduction Spontaneous resolution of Chiari I abnormality is very rare. In most patients, the radiological abnormality either stays unchanged with time or deriorates.Case report We present a male patient who was diagnosed at the age of 18 months as having radiological evidence of Chiari I malformation without syringomyelia, which had resolved 5 years later on a subsequent MR scan. At the time of initial diagnosis, he had been experiencing recurrent jerking movements of his body and was a sufferer of chronic renal failure.Discussion The symptoms were thought to be unrelated to the hindbrain hernia. Such spontaneous resolution of an isolated Chiari I malformation has only been described once more before, although resolution of hindbrain hernia associated with syringomyelia has been described before in several cases, albeit at single figures. The mechanism for such a natural evolution is not clear.Conclusion This patient demonstrates that surgical treatment should not be considered hastily in patients with radiological evidence of Chiari I in the absence of convincing associated clinical symptoms.     

Chiari畸形合并脊髓空洞症的MRI研究

目的 研究Chiari畸形(CM)/脊髓空洞症(SM)患者小脑扁桃体下疝的程度与空洞大小之间的关系.并探讨此综合征的发病规律。方法 回顾性分析我院MRI室自1992～1996年所做的CM/SM患者的术前MRI资料,测量小脑扁桃体下疝的程度(L)、空洞的长度(空洞沿椎体的节段数)和宽度、对所有的计量资料进行统计学处理。结果 162例,其中男68例,女94例,年龄14～61岁,平均37岁。L<5 mm,占13.58％,L在5～15 mm,占75.31％,L>15 mm,占11.11％。小脑扁桃体下疝的程度与空洞长度的相关系数为:r1=-0.096 1,P=0.351 7,与宽度的相关系数为:r2=-0.005 5,P=0.957 8。结论 小脑扁桃体下疝长度在5～15 mm之间者易伴发脊髓空洞,小脑扁桃体下疝的长度与空洞的大小无显著的线性相关。     

Winged scapula as the presenting symptom of Chiari I malformation and syringomyelia

Case report We report a 19-year-old girl with a 4-month history of an inability to fully elevate her upper extremity past the horizontal position. Physical examination revealed a winged scapula. MRI was demonstrative of a Chiari I malformation with a small cervical syrinx eccentrically placed to the same side as the dysfunctional extremity. Discussion We believe this to be the first report of dysfunction of the long thoracic nerve via a hindbrain hernia-induced syrinx with a resultant winged scapula. We would hypothesize that enough anterior horn motor neurons and their axons destined for the long thoracic nerve were injured by the syringomyelia to result in isolated deinnervation of the serratus anterior muscle. Conclusion The clinician may wish to include syringomyelia in the differential diagnosis of a winged scapula.     

Chiari畸形的分型与手术

目的：探讨Chiari畸形的分型与术式的关系。方法：根据MR和临床表现将病人分为3组：（1）脊髓前受压型：齿状突压迫延髓或脊髓的腹侧,造成延颈段脊髓严重成角畸形;四肢痉挛性瘫痪,长束征阳性,选用术式为枕枢髂骨固定术和经口齿状突磨除术。（2）小脑受压型：枕骨压迫小脑,延髓脊髓腹侧未受压;小脑和后组颅神经受损;选用术式为后颅窝减压术;（3）混合型：齿状突轻度压迫延髓脊髓腹侧,枕骨也轻度压迫小脑;四肢肌力轻度减低,肌张力可增高,腱反射可亢进;后组颅神经和小脑受损的表现也很轻微;选用术式为后颅窝部分减压术同时行枕枢髂骨固定术。结果：脊髓前受压型,小脑受压型和混合型分别随访9,30和22例,术后症状,体征改善或停止发展分别为8,24和18例,结论：Chiari畸形的分型有利于术式的选择。