Chiari I malformation with syrinx

The Chiari I congenital malformation is characterized by caudal displacement of the cerebellar tonsils through the cervical canal. Although this malformation is often asymptomatic, coexisting syringomyelia can result in neurologic symptoms. We report a case of progressive ataxia with brainstem dysfunction in an adolescent female manifesting a severe Chiari I malformation with syrinx. Chiari decompression 4 years after initial presentation led to rapid improvement in most of her long-term symptoms. This case demonstrates the importance of consideration of Chiari I with syringomyelia in the differential diagnosis of progressive ataxia and brainstem symptoms.     

Spinal cord syrinx expansion following acquired Chiari malformation decompression: Case report

Objective

Chiari malformation development after lumboperitoneal (LP) shunting for pseudotumor cerebri is a recognized phenomenon. Treatment options for an acquired Chiari malformation include observation, LP shunt revision or ligation, ventriculoperitoneal (VP) shunt placement, and suboccipital decompression. The authors describe a case of suboccipital decompression of an acquired Chiari malformation that resulted in the development of a spinal cord syrinx.

Clinical presentation

A 24-year-old woman presented with headaches, photophobia, and blurred vision. No ventricular enlargement was seen on a computed tomographic scan of the brain. The patient was diagnosed with pseudotumor cerebri based on the clinical presentation and a lumbar puncture with a high opening pressure. She underwent an LP shunt. At the time of her 10-month follow-up evaluation, she was noted to have a symptomatic acquired Chiari malformation without a spinal cord syrinx.

Intervention

The patient underwent a suboccipital decompression to treat her Chiari malformation. Postoperatively, she developed an enlarging, symptomatic spinal cord syrinx. The patient underwent LP shunt ligation and VP shunt placement and had subsequent resolution of her spinal cord syrinx.

Conclusion

An enlarging spinal cord syrinx can occur following the suboccipital decompression of an acquired Chiari malformation in pseudotumor cerebri patients. Careful consideration to VP shunting should be given prior to posterior fossa decompression in such cases in an attempt to avoid syrinx development.     

Isolated thoracic syrinx in children with Chiari I malformation

Introduction

Syrinx has been reported in 25–85 % of children with Chiari malformation type I (CMI), and it is most commonly cervical in location. As a result, cervical MRI is routinely included in an evaluation for CMI. Isolated thoracic syrinx without involvement of the cervical cord in this population is uncommon but clinically important because its presence may influence the decision to operate, surgical techniques employed, or interpretation of follow-up imaging. The purpose of this study was to determine the incidence of isolated thoracic syrinx in a large group of children evaluated for CMI.

Methods

We retrospectively reviewed all patients under 21 years of age who were evaluated for CMI at Columbia University/Morgan Stanley Children’s Hospital of New York from 1998 to 2013. All patients underwent MRI of the entire spine as part of the CMI evaluation, regardless of whether surgery was planned. The proportion of patients exhibiting isolated thoracic syrinx was determined. Presenting signs, symptoms, and imaging findings were then studied in an attempt to identify any clinical features associated with isolated thoracic syrinx.

Results

We identified 266 patients evaluated over the study period. One-hundred thirty-two patients (50 %) presented with a syrinx, and 12 patients (4.5 % of all patients evaluated and 9.1 % of all patients with a syrinx) had an isolated thoracic syrinx. Demographic variables, clinical presentation, and extent of tonsillar ectopia showed great heterogeneity in this group, and no factor was consistently associated with isolated thoracic syrinx.

Conclusions

Isolated thoracic syrinx is an uncommon but clinically significant finding in children with CMI. Our data demonstrate that the presence of a CMI-related thoracic syrinx cannot be reliably predicted clinically and is therefore likely to be missed in patients who do not undergo complete spinal cord imaging. MRI of the entire spinal cord should be considered for all children undergoing initial evaluation for CMI.

     

Surgical results of posterior fossa decompression for patients with Chiari I malformation

Introduction An increasing number of children with Chiari I malformations are coming to the attention of neurosurgeons today, although a consensus on the surgical approach to these lesions has yet to be found.Methods We present a retrospective analysis of posterior fossa decompression (PFD) performed at our institution on 96 patients from 1989 to 2001. Statistical analyses based on clinical and radiographic presentation and the types of surgical procedures used formed the basis for our review.Results Most of the patients with hydromyelia underwent duraplasty procedures with or without tonsillar manipulation. In contrast, most patients without hydromyelia underwent bony decompression with dural scoring and intraoperative ultrasound. PFD with bony decompression and dural scoring showed a 72% success rate, compared with 68% for duraplasty. Dural opening was not more likely to improve or arrest hydromyelia. The group subjected to duraplasty, however, had a significantly higher complication rate. Patients under the age of 8 fared better than their older counterparts.Conclusions Overall, we favor a tailored posterior fossa craniectomy with dural scoring as the initial surgical procedure in children with Chiari I malformation with or without a syrinx. This less invasive approach minimizes complications associated with dural opening and offers comparable success rates.     

后颅窝内容物内减压治疗Chiari畸形并脊髓空洞症

自2006年1月到2007年11月,作者对10例Chiari畸形并脊髓空洞,行小脑扁桃体下疝及脊髓中央管隔膜切除,使后颅窝内容积扩大,恢复脑脊液循环通畅,后颅窝骨瓣复位,称之为后颅窝内容物内减压。报告如下。     

Spontaneous resolution of syrinx: report of two cases in adults with Chiari malformation

Idiopathic syringomyelia is a disease with variable clinical course. We report here two cases of spontaneous resolution of cervical syrinx in adults previously diagnosed of Chiari–syringomyelia complex. They are added to the nine cases previously reported, and documented the need for careful surgical indication in this disease based on the radiological images of spinal cord cavitation.     

后颅窝减压术治疗Chiari畸形Ⅰ型合并脑积水疗效的Meta分析

目的 评价后颅窝减压术（PFD）治疗合并脑积水的Chiari畸形Ⅰ型的有效性。方法 计算机检索EBSCO、PubMed、EmBase、OVID等英文数据库以及中国知网、维普、万方等中文数据库,检索从建库到2015年5月31日发表的文献。采用STATA 13.0软件进行Meta分析。结果 共纳入3篇文献,86例,PFD治疗83例;PFD术后好转率为82.3%（95% CI 74.2%~90.4%）;合并脑积水的Chiari畸形Ⅰ型中,女性占62.9%（95% CI 52.6%~73.1%）。结论 合并脑积水的Chiari畸形Ⅰ型中,女性占大多数;PFD治疗合并脑积水的Chiari 畸形Ⅰ型具有较高的有效率。     

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。     

神经内镜下寰枕部减压手术治疗Chiari畸形

目的 探讨神经内镜下寰枕减压手术治疗Chiari畸形的手术方法和临床疗效.方法 对23例经MRI确诊为Chiari畸形但不伴有寰枢锥脱位和齿突凹陷的患者在神经内镜下进行了寰枕减压手术,并进行疗效观察.结果 23例术后随访0.5-3.0年,原有症状均有不同程度改善,感觉障碍程度减轻,肢体肌力有所增强.无手术后并发症.术前合并脊髓空洞症的16例患者中,术后7例空洞消失,8例范围缩小,1例范围无明显变化.结论 神经内镜下寰枕减压手术治疗Chiari畸形方法可行,手术安全、有效、创伤小、疗效满意.

Abstract：

Objective To investigate the methods and effectiveness to treat Chiari malformation with the surgical decompression of the foramen magnum under endoscope. Method 23 cases with Chiari malformation.(without atlanto- axialdislocation and basilar invagination) diagnosed by magnetic resonance imaging( MRI)were operated with the surgical decompression of the foramen magnum under the endoscope. Results 23 cases were followed- up 0. 5 -3. 0, years after operation, all the symptoms and signs were remarkably relieved after the operation. No complications was found. In 16 cases with spinal cavities,7 cases spinal cavities disappeared and scases deflated,l case no evident change. Conclusions The endoscope -assisted decompression of the foramen magnum is a safe and effective surgical method to treat Chairi malformation.     

Pain improvement in patients with syringomyelia and Chiari I malformation treated with suboccipital decompression and tonsillar coagulation

Pain experienced by patients who have syringomyelia associated with Chiari I malformation (SACM) includes headache, cervicalgia and radiculalgia. We studied the correlation of clinical and imaging factors with the evolution of pain and surgical outcome at 12 months. We performed a prospective study of 13 patients who presented with SACM and who underwent suboccipital decompression, C1 laminectomy, tonsillar coagulation and duraplasty. The Bidzinski Outcome Scale was used. Post-operatively, pain improvement was observed in 11 patients and the syrinx resolved in 6 patients and reduced in 7. Eleven patients had a good or very good outcome, while 2 patients had a poor outcome. A post-operative medullary axial occupation of the syrinx less than 75% of the spinal canal was associated with improved post-operative pain. Longer duration of symptomatology was associated with a poor outcome and failed pain control. The size and form of the syrinx, as well as early surgical treatment, are the factors that most influence the control of pain post-surgery in patients with SACM.     

显微血管减压术后复发三叉神经痛的手术治疗

目的探讨显微血管减压术后复发三叉神经痛的手术治疗方法。方法1998年1月至2005年12月采用显微神经外科手术治疗37例显微血管减压术后复发三叉神经痛患者，30例患者行三叉神经感觉根部分切断术，单纯三叉神经显微血管减压术3例，显微血管减压术加行感觉根部分切断术4例。结果95％患者术中发现有CPA局部蛛网膜明显增厚粘连。全部患者获平均38．2个月的随访。随访期间总有效率97％。并发症：行三叉神经感觉根部分切断术者术后均有面部麻木，随访期间均见不同程度好转；术后发生听力障碍合并轻度面瘫1例，复视1例，随访期间好转；术后发生化脓性脑膜炎1例，出院时治愈；1例高龄患者术后发生小脑半球出血，量约5ml，经保守治疗后好转出院。结论CPA局部蛛网膜严重粘连是导致显微血管减压术后疼痛复发的最重要原因，二次手术时的术式选择应以三叉神经感觉根部分切断术为主。     

Surgical results of arachnoid-preserving posterior fossa decompression for Chiari I malformation with associated syringomyelia

We analyzed the outcome of posterior fossa decompression accompanied by widening of the cisterna magna, without disturbing the arachnoid, in patients with Chiari I malformation (CMI) associated with syringomyelia. Twenty-five adult patients with CMI and syringomyelia, who underwent surgery between October 2000 and December 2008, were enrolled in this study. All patients underwent foramen magnum decompression with C1 decompression, with or without C2 decompression. Three surgeons performed a dura opening with duraplasty in 20 patients, and another surgeon excised the outer layer of the dura without duraplasty in five patients. Clinical and radiological assessments were performed preoperatively and during the follow-up period. After surgery, 20 (80%) patients achieved a significant improvement in their clinical symptoms. However, four patients (16%) achieved only a stable state, and one patient's symptoms worsened. Radiological analysis showed that 17 patients (68%) had a favorable result; that is, a total collapse, or a marked reduction, of the syrinx. Seven patients (28%) were stable in terms of syrinx size. However, the syrinx enlarged in one patient who had undergone excision of the outer dura. Twenty-four patients achieved a widened cisterna magna with ascent of the cerebellar tonsils into the posterior fossa and acquisition of a more rounded shape. Postoperative complications included a transient headache and vomiting in three patients and transient motor weakness in one patient. Two patients developed a superficial wound infection. This study shows that arachnoid-preserving posterior fossa decompression is a safe and effective treatment for patients with CMI with associated syringomyelia.     

Microvascular decompression for recurrent trigeminal neuralgia

Recurrence of trigeminal neuralgia (TN) symptoms after microvascular decompression (MVD) is a challenge for neurosurgeons. This study evaluates the indication, efficacy and safety of re-do MVD. We retrospectively reviewed consecutive patients who underwent MVD for TN from January 2000 to June 2012. The parameters of study interest were pre-operative magnetic resonance tomographic angiography (MRTA) findings and operative findings. Pain outcome was scored using the Barrow Neurological Institute (BNI) grading scale. Twelve patients underwent re-do MVD following recurrence of pain. Vascular compression was detected on pre-operative MRTA images in eight patients, a small mass was found in two patients, and pre-operative imaging was negative in two patients. Of the eight patients with a positive finding of arterial conflict, a vascular loop was identified intra-operatively in five patients (62.5%); in two (25.0%) a small granuloma filled with Teflon fibers was found compressed the trigeminal nerve; and in one patient (12.5%) only dense arachnoid adhesions were found around the trigeminal nerve. For the two patients with mass compression on MRTA images, a granuloma was found during operation. Neurovascular compression was found in the two patients with negative preoperative MRTA images. Re-do MVD is a safe and effective treatment for recurrent TN when indicated by a prolonged pain-free period following the first surgery.     

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     

Posterior fossa decompression in syringomyelia associated with a Chiari malformation: a retrospective analysis of 22 patients

BACKGROUND: The technique of posterior fossa decompression, nowadays preferred by most neurosurgeons as the first surgical option in the treatment of hindbrain associated syringomyelia, was reintroduced in our center in 1989. We present a retrospective analysis of 22 patients with this pathology who underwent the procedure since then. METHODS: In all patients diagnosis was made by MRI of the craniospinal junction. The operation consisted of a suboccipital craniectomy, removal of the posterior arc of C1, laminectomy of C2 or C3 when necessary and a wide duraplasty at the end. The intradural manipulations (opening of the arachnoid membrane, coagulation or resection of the tonsils) were not uniform. Postoperatively short- and long-term clinical outcome and MRI findings were assessed. RESULTS: Sixteen out of 21 patients (76%) experienced an improvement in the early follow-up period. In the late follow-up period 13 out of 19 patients (68%) were improved, whereas five patients (26%) experienced a marked deterioration. There was no unequivocal effect on all symptoms and signs. Postoperative MR images showed a favorable result in 16 out of 20 patients (80%), consisting of syrinx collapse or reduction of the syrinx diameter. CONCLUSIONS: We conclude that decompression of the posterior fossa is a safe procedure with a considerable chance of clinical improvement. Although total syrinx collapse is not as frequently seen as in syrinx shunting procedures, the clinical outcome may be better. Moreover, there seemed to be no unequivocal correlation between clinical outcome and postoperative syrinx size in the present study.     

Chiari畸形合并脊髓空洞症术中不同大小后颅窝减压窗近期疗效的比较

目的 探讨不同大小后颅窝骨性减压窗对Chiari畸形合并脊髓空洞症患者手术近期疗效的影响。方法 回顾性分析2007~2010年收治的117例Chiari畸形合并脊髓空洞症患者的临床资料,均采用环枕减压硬膜成形术,空洞横径>脊髓50%者行空洞穿刺减压术。根据后颅窝减压窗面积分为2组:Ⅰ组,87例,减压面积为12 cm2（3 cm×4 cm）;Ⅱ组,30例,减压面积<6 cm2。术后2周采用Tater评分评定疗效。结果 117例患者手术后症状均无加重,症状改善97例（82.9%）,无变化20例。Ⅰ组症状改善76例（87.4%）,Ⅱ组为21例（70%）;两组症状改善率差异显著（P<0.05）。>结论 Chiari畸形合并脊髓空洞症患者的后颅窝减压面积以不小于12 cm2的近期疗效较好。     

22例Chiari畸形Ⅰ型合并脊髓空洞症的疗效分析

目的探讨后颅窝减压术合并枕大池重建术治疗Chiari畸形Ⅰ型合并脊髓空洞症的手术疗效。方法回顾性分析22例Chiari畸形Ⅰ型合并脊髓空洞症患者的临床资料。结果手术后1周内症状消失或改善的19例;随访术后6个月~2年,症状消失或改善的14例;其中脊髓空洞症减小或消失10例。结论后颅窝减压合并枕大池重建术是临床治疗Chiari畸形Ⅰ型合并脊髓空洞症安全有效的手术方法。     

面肌痉挛微血管减压再手术病例的选择与疗效

目的探讨微血管减压(microvascular decompression,MVD)手术治疗面肌痉挛手术失败或复发再手术病例的选择和疗效。方法 2001年11月至2010年5月期间,在首次手术失败或复发的面肌痉挛患者中,根据其要求手术的愿望,结合术前影像学和首次手术录像、记录等资料综合判断后,选择了8例首次手术失败和20例术后复发患者进行MVD再手术治疗。结果经过平均34月的随访,仅1例患者术后症状无明显改善。其余27例(96.4%)患者术后症状缓解程度达75%以上,其中21例(75.0%)术后症状完全消失。24例(85.7%)患者术后自我疗效评价达到"优"和"良"。所有患者术后无严重病残和死亡发生,仅一例术后发生HBS 3级面瘫。结论选择合适的病人、恰当的手术时机、耐心仔细的显微操作,是面肌痉挛微血管减压再手术取得良好疗效的保证。