Scoliosis associated with syringomyelia presenting in children

The clinical presentations and radiological features of scoliosis accompanying syringomyelia were analyzed in 14 cases of syringomyelia associated with a Chiari malformation in children. Scoliosis was the initial symptom in 11 out of 16 patients (64%) with syringomyelia and present in 14 (88%) at the initial examination. The scoliosis associated with syringomyelia was characterized by a higher incidence of a single curve (6 cases, 43%) and convexity to the left (7 cases, 50%) than seen in idiopathic scoliosis. The syrinx was shifted to the convex side of scoliosis on the axial section at the middle or lower thoracie level in patients with a single curve, and at the cervical or upper thoracic level in patients with a double curve. The authors think that the scoliosis develops in children as a result of damage done to the anterior horn, which innervates the muscles of the trunk, by an asymmetrically expanded syrinx.     

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.     

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.     

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…     

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].     

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。     

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。     

An efficacy analysis of posterior fossa decompression techniques in the treatment of Chiari malformation with associated syringomyelia

A retrospective study of the efficacy of posterior fossa decompression (PFD) was carried out in 132 patients with Chiari malformation (CM) with associated syringomyelia (SM). Of these 132 patients, 69 received extended PFD (large craniotomy group), and the other 63 patients received only local PFD (small craniotomy group). At the short-term postoperative evaluation (1–4 weeks) the extended PFD appeared to be more effective than the local PFD (p < 0.05). However, there was no significant difference in long-term analysis (6 months–11 years) (p > 0.05). In the large craniotomy group, there was no difference between the short-term and long-term efficacy (p > 0.05). However, in the small craniotomy group, long-term efficacy clearly improved (p < 0.05). Furthermore, patients who had undergone local PFD exhibited more obvious radiological improvement of SM (p < 0.05) and fewer postoperative complications compared to patients undergoing extended PFD (p < 0.05). Therefore, local PFD is preferable for the surgical treatment of CM with associated SM.     

Factors contributing improvement of syringomyelia and surgical outcome in type І Chiari malformation

Objective  The aim of our study was to compare pre- and postoperative radiologic data of posterior fossa and the improvement of syringomyelia after posterior fossa decompression (PFD) with and without tonsillar management in Chiari type І malformation (CM-I). Material and methods  A retrospective analysis was made of all patients who underwent PFD between Oct 1991 and March 2007 for CM-I. Fifty-seven patients treated for CM-I at a single institution were included in the study. Patients were divided into two groups according to the procedures used during their PFD: PFD vs. PFD with tonsillar management. To determine whether the tonsillar management or changes of posterior fossa volume relate with surgical outcome, we measure posterior fossa size and syringomyelia pre- and postoperatively using magnetic resonance imaging. Results  Forty patients (70.2%) received PFD and 17 patients (29.8%) received PFD with tonsillar management. The length of syringomyelia affected improvement of syringomyelia (alteration rate, A-rate). Clinical symptoms, craniectomy size, syringomyelia type, and the surgeon’s specialty did not affect A-rate. Tonsillar management has no significant effect on improvement of syringomyelia. Four patients need repeated surgery due to recurrence. Conclusion  We have shown that tonsillar management do not lead to improve A-rate, and the radiologic changes of posterior fossa volume do not relate with radiologic improvement of syringomyelia. PFD without tonsillar management is sufficient to improve syringomyelia. The longer syrinx, the more A-rate improve in our study. However, a wider craniectomy is unrelated to A-rate. In cases of recurrent patients, we obtained good results with tonsillar management or syringosubarachnoid shunt. An erratum to this article can be found at     

Segmental hyperhidrosis in syringomyelia with Chiari malformation

Segmental hyperhidrosis was seen in three cases of syringomyelia with Chiari malformation confirmed by magnetic resonance imaging. Using the iodine starch reaction, all three cases showed hyperhidrosis in the areas roughly corresponding to those of sensory disturbances. Two possible modes of pathogenesis are discussed: stimulation of preganglionic neurons and interference to the inhibitory tract.     

小脑扁桃体切除并脊髓中央管口松解术治疗合并脊髓空洞的Chiari畸形

目的探索外科治疗合并脊髓空洞的Chiari畸形的新方法。方法采用切除下疝小脑扁桃体,并脊髓中央管口假膜切开术,原位硬膜缝合法。结果18例手术治疗,15岁以下儿童12例,术中发现均有脊髓中央管口(闩部)假膜;成人6例,3例有假膜形成。全部病例术后脊髓空洞消失或明显缩小。结论脊髓中央管口假膜是造成Chiari畸形脊髓空洞的原因之一,切除下疝小脑扁桃体并脊髓中央管口假膜切开术,是手术治疗合并脊髓空洞的Chiari畸形最根本的方法。     

不同手术方式治疗Chiari Ⅰ畸形合并脊髓空洞的临床研究

目的通过同顾性比较枕大孔区减压硬膜成形术及枕大孔区减压环枕筋膜松解术对Chiari Ⅰ畸形合并脊髓空洞的治疗,明确两种不同术式治疗ChiariⅠ畸形合并脊髓空洞的疗效. 方法2002年1月至2004年4月对收治的62例ChiariⅠ畸形合并空洞患者行枕大孔区减压,其中46例患者剪开硬脑膜行硬膜成形术（硬膜成形组）,16例患者未剪开硬膜仅做环枕筋膜松解（筋膜松解组）.结果两组患者无一例死亡,硬膜成形组患者术后1年临床改善39例（84.78%）,脊髓空洞缩小30例,筋膜松解组临床改善9例（56.25%）,x^2=5.528,P=0.019,脊髓空洞缩小7例. 结论枕大孔区减压硬膜成形术是治疗ChiariⅠ畸形合并脊髓空洞症较为合理的术式,疗效优于枕大孔区减压环枕筋膜松解术.     

脊柱侧凸为首发症状的Chiari畸形临床研究

目的 研究以脊柱侧凸为首发症状的Chiari畸形的临床特征和治疗策略.方法 收集南京鼓楼陕院住院治疗的Chiari畸形患者60例,35例以脊柱侧凸为首发症状;25例末合并脊柱侧凸作为对照组,病例对照研究.结果 35例Chiari畸形合并脊柱侧凸患者,年龄3～33岁(平均13.75岁),97%合并脊髓空洞,不具有典型的Chiari畸形的临床症状和体征,以腹壁反射异常多见.两组之间手术方式和手术并发症无统计学意义.结论 Chiari畸形以脊柱侧凸为首发症状,多合并脊髓空洞,发病年龄轻,其临床症状和神经损害体征不明显,治疗策略上主张早期进行枕颈部枕骨大孔区减压和硬脑膜重建成形手术.     

两种手术方式对Chiari畸形Ⅰ型合并脊髓空洞的系统评价及meta分析

目的系统评价后颅窝减压(posterior fossa decompression,PFD)和后颅窝减压加硬膜成形术(posterior fossa decompression plus duraplasty,PFDD)在Chiari畸形Ⅰ型合并脊髓空洞患者中的有效性及安全性。方法检索Embase、Cochrane、Pubmed、Ovid、Medline、Science Direct、谷歌学术、万方、知网等数据库自建库到2019年的文献,筛选文中对PFD与PFDD进行了比较的随机对照研究或非随机对照研究,统计术后临床症状和影像学改善情况、并发症等指标,运用Revman(5. 3版本)进行数据分析。结果最终纳入10篇文献,共3 188例,PFDD组1 383例,PFD组1 805例。在脊髓空洞改善率(OR:5. 53; 95%CI:2. 86,10. 69)、症状缓解率(OR:2. 53; 95%CI:1. 30,4. 91)、并发症发生率(OR:3. 46; 95%CI:1. 40,8. 59)、脑脊液漏发生率(OR:9. 36; 95%CI:2. 63,33. 34)、假性硬脑膜膨出率(OR:1. 89; 95%CI:1. 28,2. 79)方面PFDD组高于PFD组(P 0. 05)。在切口感染发生率(OR:1. 44; 95%CI:0. 57,3. 59)、皮下积液发生率(OR:1. 71; 95%CI:0. 50,5. 80)方面两种术式无差异(P 0. 05)。结论针对Chiari畸形Ⅰ型合并脊髓空洞的患者,PFDD的有效性优于PFD。     

两种手术方式对Chiari畸形Ⅰ型合并脊髓空洞的系统评价及meta分析

目的 系统评价后颅窝减压（posterior fossa decompression,PFD）和后颅窝减压加硬膜成形术（posterior fossa decompression plus duraplasty,PFDD）在Chiari畸形Ⅰ型合并脊髓空洞患者中的有效性及安全性。方法 检索Embase、Cochrane、Pubmed、Ovid、Medline、ScienceDirect、谷歌学术、万方、知网等数据库自建库到2019年的文献,筛选文中对PFD与PFDD进行了比较的随机对照研究或非随机对照研究,统计术后临床症状和影像学改善情况、并发症等指标,运用Revman（5.3版本）进行数据分析。结果 最终纳入10篇文献,共3 188例,PFDD组1 383例,PFD组1 805例。在脊髓空洞改善率（OR：5.53;95%CI：2.86,10.69）、症状缓解率（OR：2.53;95%CI：1.30,4.91）、并发症发生率（OR：3.46;95%CI：1.40,8.59）、脑脊液漏发生率（OR：9.36;95%CI：2.63,33.34）、假性硬脑膜膨出率（OR：1.89;95%CI：1.28,2.79）方面PFDD组高于PFD组（P<0.05）。在切口感染发生率（OR：1.44;95%CI：0.57,3.59）、皮下积液发生率（OR：1.71;95%CI：0.50,5.80）方面两种术式无差异（P>0.05）。结论 针对Chiari畸形Ⅰ型合并脊髓空洞的患者,PFDD的有效性优于PFD。     

Chiari畸形手术方法的改良

目的探讨小脑扁桃体下疝畸形(Chiari畸形)的手术方法，提高治疗效果，减少并发症方法本院自1993年12月至2003年12月对收治的86例Chiari畸形及部分合并脊髓空洞症患者实施了改良的枕大孔区小骨窗骨性减压、局部硬膜外层剥脱术。术后82例得到随访，平均随访5．5年(1～9年)，其中50例MRI复查头颈部？结果患者症状明显改善59例，部分改善10例，无变化13例，有效率占随访者84．15％，无手术并发症。结论改良的手术方式，在枕颈交界区骨性减压后，保留枕大孔区内层硬膜结构的情况下，可改善局部蛛网膜下腔脑脊液循环，缓解小脑扁桃体对延颈髓的压迫，效果较显著，同时减少了并发症。     

Morphometric analysis of the craniocervical juncture in children with Chiari I malformation and concomitant syringobulbia

Introduction  Although very uncommon, Chiari I malformation (CIM) with syringomyelia may be associated with concomitant syringobulbia. We hypothesized that the anatomy of the craniocervical region may be different in CIM patients with syringomyelia who develop syringobulbia in conjunction with their syringomyelia compared to other patients with CIM with and without syringomyelia. The present study was conducted in order to prove or disprove such a theory. Materials and methods  A group of 189 children with operated CIM were reviewed for the presence of syringobulbia, and this cohort then underwent morphometric analyses of their craniocervical juncture. These measurements were then compared to both our prior patient findings and historic controls. Results  The current study did not identify any morphometrical peculiarities for patients with CIM and syringobulbia compared to other CIM patients with and without isolated syringomyelia. Conclusions  Based on our study, the mechanism behind such cerebrospinal fluid distention into the brain stem remains elusive with no single morphometrical difference in patients with CIM and syringobulbia compared to other patients with CIM. Perhaps, future testing aimed at identifying pressure gradients across the foramen magnum in patients with and without syringobulbia and concomitant CIM may be useful.     

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     

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.     

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.