Comparison of operative and non-operative outcomes based on surgical selection criteria for patients with Chiari I malformations

Few studies have directly compared operative and non-operative outcomes in Chiari I patients. We evaluated risk factors for clinical improvement in 177 patients in order to help determine the optimal treatment of these often difficult to treat patients. The mean age at surgery for the operative treatment group was 29.9 years. The most common presenting signs and symptoms included cough headache (63.0%), migraine and non-cough type headaches (23.9%), paresthesias (32.1%), and abnormal reflexes or clonus (27.5%). The mean age of diagnosis for the non-operative treatment group was 30.2 years. The most common presenting signs or symptoms included migraine and other types of non-cough-associated headache (57.4%), paresthesias (45.6%), cough headache (44.1%), cerebellar signs or symptoms (41.2%), and dysphagia or apnea (15.7%). A propensity score was generated using cough headache, any headache, other headache, syrinx, abnormal reflexes or clonus, cerebellar symptoms, and miscellaneous symptoms as independent predictors of selection for surgery. The propensity score-adjusted odds of overall improvement for patients treated with surgery were 16.5 times the odds of overall improvement for patients treated conservatively (95% confidence interval 5.5–57.1, p < 0.0001). Overall 94.5% and 47.1% of operative and conservatively treated patients reported improvement, respectively. Only 26.5% of conservatively treated patients reported worsening of any of their symptoms. In conclusion, we provided further evidence for the use of cough headache as surgical indication for suboccipital decompression in patients with Chiari I malformation.     

前后路联合治疗颅底凹陷合并小脑扁桃体下疝患者的临床疗效及预后分析

目的分析前后路联合治疗颅底凹陷合并小脑扁桃体下疝（Chiari）畸形患者的临床疗效及预后。方法选取2009年2月~2016年2月诊治的12例颅底凹陷症合并Chiari畸形患者。分析患者术后的临床疗效、影像参数变化及预后。结果所有患者术后随访7 d至20个月,患者的临床症状、体征均有明显的改善;术后的JOA评分（14.14±2.04）分明显高于术前的JOA评分（6.65±1.23）分（P<0.05）;12例患者术后CL值、ML值、WL值、Klaus高度指数、延颈髓角及颅底角参数较术前明显改善（P<0.05）;术后并发症少。结论经前后路联合治疗颅底凹陷合并小脑扁桃体下疝患者的临床效果较好,对脊髓功能改善明显,并发症发生率少,短期预后良好。     

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。     

Percutaneous fetoscopic patch closure of human spina bifida aperta: advances in fetal surgical techniques may obviate the need for early postnatal neurosurgical intervention

Background  A percutaneous minimally invasive fetoscopic approach was attempted for closure of a spina bifida aperta in two fetuses with L5 lesions. The goal was to obviate the need for postnatal neurosurgery to manage this condition. Methods and Results  The percutaneous fetoscopic procedures were performed by a two-layer approach at respectively 22 ± 2 and 22 ± 4 weeks of gestation. The fetuses were delivered respectively at 32 ± 6 and 32 + 3 weeks of gestation. Their neural cords were completely covered although in small areas skin closure was incomplete. Postnatally, complete skin closure occurred beneath an occlusive draping within 2 to 3 weeks such that neurosurgical intervention was not required. Both neonates showed reversal of hindbrain herniation, near-normal leg function, and satisfactory bladder and bowel function. For one of the two fetuses, ventriculoperitoneal shunt insertion was not required. Conclusions  Percutaneous minimally invasive fetoscopic patch closure of spina bifida aperta offers a substantially less maternal trauma than open fetal surgical repair and currently may even obviate the need for postnatal neurosurgical repair. With a little further improvement in surgical techniques and a better understanding of incorporating surgical patches into the fetus, complete skin closure seems possible in the near future.     

Chiari畸形:临床与MRI分析

14例Chiari畸形(CM)包括CM Ⅰ 11例,CM Ⅱ 3例的临床和MRI特征进行了回顾性分析。临床表现与脑干受压、脊髓和小脑病变有关.主要决定于枕大孔的相对性狭窄和脑脊液循环障碍及其程度。78.6％病人可见脊髓空洞症,临床上均有脊髓症状。具有脑干和小脑体征的病人,扁桃体下疝都很显著。MRI是诊断CM的金标准,可无创显示扁桃体疝和脊髓空洞症,并直接探测出颅椎结合部的病理解剖。矢状和冠状面T1加权像最为有效。本文4例进行后颅窝减压和上颈椎板切开收到了显著的效果。     

Chronic migraine associated with the Chiari type 1 malformation

OBJECTIVE: Our objective was to assess the frequency and clinical characteristics of migraine in the patients with CM-1. METHODS: We analyzed migraine in 73 patients with CM-1. Migraine was classified according to the new International Headache Society criteria. We did not include patients who had intracranial, parenchymal, or cervical lesions other than CM-1 on brain and cervical magnetic resonance imaging. RESULTS: Of the 73 patients diagnosed as having CM-1, 11 (15.06%) had migraines; of them, 8 (10.95%) had chronic migraines, 2 (2.73%) had migraines with auras, and 1 (1.36%) had migraines without auras. The patients who had both migraines and CM-1 (group 1) were compared regarding clinical characteristics and demographic features to the control group having chronic migraines. The control group comprised subjects free of CM-1. Onset age of pain was earlier and the frequency of headache days per month, baseline pain intensity, exacerbation of pain intensity, nausea, vomiting, and pain aggravated by physical activity were significantly higher in group 1. CONCLUSIONS: Although we found the frequency of migraine to be similar to that in population-based studies, we detected a threefold increased frequency of chronic migraine in this special population. We believe that CM-1 may be a factor associated with chronic migraine.     

Chiari畸形Ⅰ型手术治疗的显微解剖基础

目的探讨研究Chiari畸形I型手术治疗的显微解剖基础。方法选取中山大学附属第一医院神经外科共31例Chiari畸形Ⅰ型患者分析随访。结果术中发现Chiari畸形Ⅰ型患者具有显微解剖异常,以此指导手术治疗取得较好效果。结论充分深入了解Chiari畸形Ⅰ型的显微解剖基础是获得良好治疗效果的保证。     

睡眠呼吸障碍为首发表现的Chiari畸形幼儿1例报道并文献复习

目的 探讨中枢性睡眠呼吸暂停的特点及其引起疾病的临床特征,提高Chiari畸形合并中枢性睡眠呼吸暂停的认识.方法 回顾性分析1例以睡眠呼吸障碍为首发表现的Chiari畸形患儿的临床资料,结合文献复习探讨Chiari畸形合并中枢性睡眠呼吸暂停(CSA)的特征.结果 患儿通过影像学及多导睡眠监测证实符合Chiari畸形Ⅰ型...