先天性脊柱侧凸患者中的脊髓畸形和脊椎畸形

目的:探讨先天性脊柱侧凸患者的各类脊髓畸形与脊椎畸形之间的关系。方法:对我院2003年～2005年收治的211例已确诊的先天性脊柱侧凸患者的临床资料进行回顾性分析,统计先天性脊柱侧凸患者脊髓畸形、脊椎畸形的发生率及其好发部位,观察两者间的关系。结果:211例先天性脊柱侧凸患者中脊髓畸形44例(20.85%),其中脊髓空洞症25例次(11.85%),脊髓纵裂30例次(14.22%),马尾终丝栓系28例次(13.27%),低位圆锥12例次(5.69%),其他类型少见,多种畸形常并发存在;脊髓畸形好发于脊柱胸段、胸腰段、腰段、腰骶段。44例伴有脊髓畸形的先天性脊柱侧凸病例中不良分节34例(77.27%),孤立性半椎体1例(2.27%),非孤立性半椎体21例(47.73%),脊柱裂30例(68.18%),混合型41例(93.18%);脊椎畸形好发于脊柱胸段、腰段、腰骶段。结论:先天性脊椎畸形常合并脊髓畸形,总体上脊髓畸形与脊椎畸形的好发部位相似,但各种脊髓畸形、脊椎畸形间缺少显著的、特定的对应关系。     

先天性脊柱侧凸患者中肋骨畸形和椎管内畸形的特点

目的 探讨先天性脊柱侧凸患者的各类脊椎畸形、肋骨畸形与椎管内畸形的特点及发生情况.方法 回顾性分析2010年1月至2011年3月手术治疗的先天性脊柱侧凸患者118例,男性52例,女性66例;年龄3～50岁,平均(14±7)岁.统计椎管内畸形、肋骨畸形的总体发生比例及各自的好发部位,运用x2检验分析不同类型脊椎畸形、肋骨畸形与椎管内畸形的伴发情况.结果 118例先天性脊柱侧凸患者合并椎管内畸形57例(48.3％),多种畸形常并发存在,以脊髓纵裂(32.2％)和脊髓空洞症(21.2％)最为多见.合并肋骨畸形69例(58.5％),以凹侧为多见(68.1％,47/69).椎管内畸形的发生率在混合型脊柱侧凸(58.3％,35/60)和多脊椎畸形(54.8％,51/93)的患者中较高(x2= 14.05和27.50,P＜0.01).伴有肋骨畸形的先天性脊柱侧凸患者中有椎管内畸形的42例(60.9％),无椎管内畸形的27例(39.1％),不伴有肋骨畸形的先天性脊柱侧凸中有椎管内畸形的15例(30.6％),两组相比椎管内畸形的发生率差异有统计学意义(x2= 10.5,P＜0.01).结论 先天性脊柱侧凸伴发的椎管内畸形以脊髓纵裂和脊髓空洞为多见;肋骨畸形多发生于混合型脊柱侧凸及多脊椎畸形患者,以凹侧多见.脊椎畸形的类型及是否伴有肋骨畸形对椎管内畸形的诊断有重要的提示意义.椎管内畸形与脊椎畸形的好发部位相似,但各种椎管内畸形、脊椎畸形间缺少显著的、特定的对应关系.     

先天性腰骶部半椎体患者中脊髓畸形和脊髓外畸形的发生率

目的 :探讨继发于腰骶部半椎体的先天性脊柱侧凸患者中脊髓畸形及脊髓外畸形的发生率。方法 :回顾性分析2002年1月～2016年5月在我院收治的75例腰骶部半椎体患者,其中男35例,女40例,平均年龄13.8±9.1岁(3～41岁)。术前均行全脊髓MRI、心脏及泌尿生殖系统多普勒超声检查,统计腰骶部半椎体患者脊髓畸形及脊髓外畸形的发生情况。脊髓畸形包括脊髓纵裂、脊髓空洞、Chiari畸形、脊髓拴系、低位圆锥等。脊髓外畸形包括心脏异常、泌尿生殖系统异常、骶骨发育不良、双下肢不等长。分别按患者性别(男性与女性)、半椎体所处侧别(左侧与右侧)、半椎体分节情况(完全分节、半分节与未分节)及是否伴发其他部位半椎体(单发半椎体与多发半椎体)分组比较脊髓畸形及脊髓外畸形的发生率。结果:脊髓畸形的发生率为14.67%(11/75),其中脊髓纵裂6.67%(5/75),脊髓拴系5.33%(4/75),脊髓空洞4.0%(3/75),Chiari畸形1.33%(1/75),低位圆锥1.33%(1/75);3例(4.0%)患者同时合并两种脊髓畸形。按性别、半椎体所处侧别、半椎体分节情况及是否伴发其他部位半椎体比较脊髓畸形的发生率均无显著性差异(P0.05)。脊髓外畸形的发生率为20%(15/75),其中心脏异常6.67%(5/75),泌尿生殖系统异常4.0%(3/75),双下肢不等长4%(3/75),骶骨发育不良6.67%(5/75)。按性别、半椎体所处侧别、半椎体分节情况及是否伴发其他部位半椎体分组比较脊髓外畸形的发生率无显著性差异(P0.05)。结论:在腰骶部半椎体患者中,脊髓畸形的发生率为14.67%,脊髓外畸形的发生率为20%。骶骨发育不良、双下肢不等长、心脏及泌尿生殖系统异常是发生率较高的脊髓外畸形。     

脊椎裂后遗足踝畸形的临床分析

目的 分析脊椎裂后遗足踝畸形的发病情况、临床特点、畸形类别与矫形治疗状况.方法 对我科1990年1月至2009年7月接受手术治疗脊椎裂后遗足踝部畸形患者进行回顾性研究,对资料进行总结分析.结果 手术治疗脊椎裂后遗足踝部畸形共107例患者.其中男性44例,女性63例;手术年龄1.3～52.0岁,平均17.7岁,其中18岁以上者50.5%(54/107).隐性脊椎裂50.5%(54/107),显性(囊性)脊椎裂49.5%(53/107).1例为胸椎裂(T3～8),余106例为腰骶椎裂.单侧足受累49例(左侧22例,右侧27例),双侧足受累58例,共165足.畸形类型:内翻足76足,外翻足23足,夏科连枷足15足,其他类足畸形51足.合并其他受累部位:膝部屈曲或膝反屈畸形4例,髋部畸形17例(包括髋内收、屈曲或髋关节脱位,骨盆倾斜、双下肢不等长等),合并大小便功能障碍30例.本组54例隐性脊椎裂致足踝畸形患者中,29例既往在多个医院未能确诊发病原因,误诊或漏诊的比率高达53.7%(29/54).107例患者中,既往接受过足踝畸形矫形手术者仅26例.18岁以上既往未能实施合理矫形外科治疗遗留严重足踝畸形者50.5%(54/107).结论 脊椎裂继发畸形主要在足踝部.由于缺乏对此病的整体认识与矫形治疗经验,延误了对此类疾病的早期诊断、早期干预的治疗时机,遗留了很多未经矫形外科治疗的严重足踝畸形.     

小儿先天性脊柱侧弯合并椎管内神经畸形的临床研究

[目的]探讨小儿先天性脊柱侧弯合并椎管内神经畸形的发病情况和临床特征及其神经畸形与临床表现的相关性.[方法]回顾性研究122例先天性脊柱侧弯患儿,对患者的体格检查情况以及全脊柱CT、全脊髓MRI、双下肢肌电图等相关辅助检查结果进行整理分析.[结果]122例先天性脊柱侧弯有63例(51.6％)合并脊髓畸形,其中脊髓栓系(TCS) 69.8％(44/63);脊髓纵裂(SCM)占60.3％ (38/63),包括Ⅰ型脊髓纵裂占68.4％ (26/38),Ⅱ型纵裂占31.6％ (12/38);脊髓空洞27％ (17/63);脊膜膨出占20.6％ (13/63);椎管内囊肿占17.5％ (11/63);Chiari畸形4.8％ (3/63).[结论]小儿先天性脊柱侧弯合并椎管内神经畸形的发生率高,并且常表现为多种脊髓畸形同时存在.合并神经畸形的患儿常表现为腰背部中线处皮肤异常,足畸形和双下肢不对称,但对于不同神经畸形其与常见的临床表现具有不同的相关性.     

先天性椎管内畸形分类及手术处理

目的回顾性分析一组椎管内畸形病例的临床资料,探讨此类疾病的诊治策略和治疗效果。方法 2002年3月至2009年3月收治椎管内畸形患者80例,男36例,女44例,年龄1个月至71岁。脊柱裂70例,脊髓拴系综合征55例。脊髓纵裂11例,椎管内脂肪瘤19例。椎管内皮样囊肿5例。脊膜膨出14例,脊髓脊膜膨出20例,脂肪性脊髓脊膜膨出12例。合并有2种或2种以上畸形78例,仅2例为单纯椎管内畸形。所有病例分别按照脊膜膨出、椎管内脂肪瘤、脊髓纵裂的特殊方法进行手术。术前、术后随访均拍摄MRI作为研究资料。采用综合感觉、运动和排尿功能评估及手术操作是否达到目的作为疗效评估手段。结果结合MRI对80例患者进行病理性分析,大部分为脊柱裂合并的畸形。80%脊膜膨出和脊髓拴系病例获得功能改善。而椎管内脂肪瘤、二分脊髓近半数的病理未见明显效果。结论脊柱裂伴发椎管内畸形应早期发现,正确详尽诊断,综合评估,尽早医学介入,严格掌握手术适应证。     

小儿脊髓纵裂及其合并畸形的治疗

[目的]探讨小儿脊髓纵裂及其合并畸形的治疗方法。[方法]回顾性分析近10a获随访经手术治疗的脊髓纵裂患儿48例，男29例，女19例；年龄7d-14岁。均因腰背部异常、双下肢和（或）二便功能障碍做全脊柱x线、MRI、CT等检查。手术先切断终丝松解栓系，再打开硬膜直视下切除纤维束或骨软骨嵴，合并先天性脊柱侧弯者一期或二期半脊椎后路切除矫形。[结果]48例患儿均有脊髓低位，伴发脊髓栓系综合征34例，脊柱侧弯及后凸26例，脊髓脊膜膨出14例，脊髓空洞15例，椎管内脂肪瘤12例。依照脊髓纵裂Pang分型：Ⅰ型27例，Ⅱ型18例，混合型3例。位于颈段1例，胸段6例，胸腰段1例，腰段38例，骶段2例。术后改善28例，恢复正常3例，无效3例。后路半脊椎切除术矫治先天性脊柱侧弯效果满意。[结论]脊髓纵裂极易合并脊髓栓系及先天性脊柱侧弯，患儿应常规行脊柱x线、MRI、CT等检查，以提高脊髓纵裂、脊髓栓系及其他脊柱畸形的诊断率。早期切断终丝松解栓系，直视下切除纤维性、骨或软骨性间隔是治疗脊髓纵裂的必要手段。同时一期或二期后路半脊椎切除是早期治疗先天性脊柱侧弯的有效方法。     

先天性脊柱侧凸合并脊髓纵裂的临床特点及手术疗效

目的 :研究手术治疗先天性脊柱侧凸合并脊髓纵裂的患者的临床特点,评估其手术疗效及并发症发生情况。方法:回顾性分析2005年3月～2017年3月间我院收治并行手术治疗的先天性脊柱侧凸合并脊髓纵裂患者69例,其中女性41例,男性28例,平均年龄13.9±4.5岁(7～34岁)。所有患者术前均行全脊柱正侧位X线、CT及MRI检查,术后即刻及末次随访行全脊柱正侧位X线检查,测量影像学参数(主弯Cobb角、次弯Cobb角、主弯顶椎偏距、躯干偏移、胸椎后凸角及腰椎前凸角),分析先天性脊柱侧凸合并脊髓纵裂的影像学特点及临床表现,并评估脊柱侧凸矫形率及相关并发症[矫形率=(术前Cobb角-术后即刻Cobb角)/术前Cobb角]。结果:在69例脊髓纵裂患者中,单纯膜性纵裂50例,骨性纵裂4例,膜性合并骨性纵裂15例。合并椎板畸形38例,半椎体24例,肋骨畸形25例,37例同时存在其他椎管内畸形,椎管外畸形8例。临床表现主要有:背部毛发10例,跛行6例,腰背痛4例,截瘫2例。双下肢/双足异常8例,神经系统阳性体征20例。脊髓纵裂好发于下胸段及腰段,占72.4%(50/69),纵裂累及椎体节段平均为4.2±2.7个。所有患者中,1例在矫形前行骨嵴切除,余均未对纵裂做预防性切除,仅单纯行侧凸矫形内固定术。60例得到随访,随访率为86.9%。平均随访时间32.4±22.7个月(13～115个月)。术前主弯Cobb角平均为71.8°±29.4°,次弯Cobb角为46.4°±17.3°,胸椎后凸角为39.5°±36.1°,腰椎前凸角为50.4°±17.3°;主弯顶椎偏距为6.2±3.6cm,躯干偏移平均为2.8±3.0cm。术后即刻主弯Cobb角28.8°±21.6°,次弯Cobb角25.6°±14.5°,胸椎后凸角25.5°±19.1°,腰椎前凸角42.3°±15.4°;主弯顶椎偏距4.2±3.3cm,躯干偏移2.4±2.8cm,主弯顶椎旋转度所有患者术后即刻冠状位主弯矫形率为(59.9±22.0)%,末次随访时矫形率为(53.6±25.7)%。术后即刻与术前相比,主弯Cobb角、次弯Cobb角、胸椎后凸角、腰椎前凸角及主弯顶椎偏距均有明显统计学差异(P0.01),主弯顶椎旋转度及躯干偏移无明显统计学差异。末次随访时主弯Cobb角平均为33.3°±25.9°,次弯Cobb角为27.1°±16.9°,胸椎后凸角为25.1°±16.1°,腰椎前凸角为45.6°±17.6°;主弯顶椎偏距为4.9±6.0cm,躯干偏移平均为2.1±2.0cm,末次随访与术后相比,均无明显统计学差异(P0.05)。术后共6例出现神经系统并发症,发生率为8.7%,均为不完全神经损伤,无截瘫发生。内固定相关并发症3例,包括螺钉松动2例,内固定棒断裂1例。2例螺钉松动患者无任何临床症状,予以随访观察;1例内固定棒断裂患者手术翻修,未再次出现并发症。结论:先天性脊柱侧凸合并SCM手术治疗患者纵裂好发于下胸段及腰段,椎体畸形以混合型最多见;中下胸段肋骨畸形的伴发率最高。     

358例无脊柱畸形汉族儿童与青少年胸、腰椎脊椎数目的变异率及其分布

【摘要】 目的：分析江苏省3~18岁无脊柱畸形汉族儿童与青少年胸椎、腰椎脊椎数目变异的发生率及分布情况,增强脊柱外科医生对胸、腰椎脊椎数目变异的认识。方法：对我科2006年1月~2014年2月收集的358例江苏省3~18岁无脊柱畸形汉族儿童与青少年的站立位全脊柱正侧位X线片进行回顾性分析。其中男性176例,女性182例。对所有入选者的站立位全脊柱正侧位X线片上的胸椎和腰椎脊椎数目进行计数,并记录所有入选者脊椎数目变异的分布情况,以统计其发生率。将胸椎和腰椎中不同变异类型的发生率行统计学分析以明确是否存在差异。结果：共33例无脊柱畸形汉族儿童与青少年存在胸椎和腰椎脊椎数目变异,发生率为9.2%（33/358）。其中存在胸椎脊椎数目变异者19例（5.3%）,18例（5.0%）为11节胸椎,1例（0.2%）为13节胸椎;存在腰椎脊椎数目变异者18例（5.0%）,17例（4.7%）为6节腰椎,1例（0.2%）为4节腰椎。经卡方检验,11节胸椎和6节腰椎的发生率分别较13节胸椎和4节腰椎的发生率高（均P<0.05）。胸椎+腰椎合计16节的变异率为3.9%（14/358）,胸椎+腰椎合计18节的变异率为4.2%（15/358）。4例（1.1%）为混合型脊椎数目变异,均表现为11节胸椎和6节腰椎。结论：在江苏省无脊柱畸形汉族儿童与青少年中,9.2%的人群存在胸腰椎脊椎数目变异,其中11节胸椎及6节腰椎这两种变异情况比较多见。     

先天性脊柱畸形52例影像学诊断

目的：为探讨各种影像学检查方法在先天性脊柱畸形诊断价值。方法：52例先天性脊柱畸形病例进行x线平片拍摄、CT扫描、MRI等检查。结果：X线平片、CT检出的52例中：①椎体异常、包括多节颈椎体融合7例；半椎体25例；蝴蝶椎14例；椎体序列旋转畸形14例；②椎体附件椎弓、棘突缺如2例；③椎管内异常、脊髓纵裂畸形21例；④多层螺旋CT三维重建、MPR及椎畸形处，脊髓受压、变细、成角；⑤MRI检出脊髓纵裂畸形7例、脊髓栓系6例、脊髓空洞症3例、脊髓脊膜膨出症2例、伴脂肪瘤6例。11例进行了手术矫治。结论：影像学检查在先天性脊柱畸形的诊断中很有价值，X线平片作为初查；CT作为椎体、椎管内畸形检出首选；MRI检出脊髓原发性、继发性各种合并症必不可少。     

Patterns of presentation of congenital scoliosis

In analysing and investigating 66 consecutive cases of congenital scoliosis, we aimed to evaluate the clinical pattern of presentation, identify the underlying vertebral anomaly causing the deformity, and determine the frequency of associated intraspinal anomalies, cardiovascular anomalies and genito-urinary anomalies. Associated general anomalies were seen in 44% of the cases. The commonest pattern of deformity was a right-sided thoracolumbar curve. Hemivertebra was the most common vertebral anomaly (45%). Occult intraspinal anomalies were seen in 15% of cases, diastematomyelia being the commonest anomaly. Genitourinary anomalies and cardiovascular abnormalities were encountered in 6% and 15% of patients respectively. As decision making in the treatment of congenital scoliosis depends on the natural history of progression of the deformity and associated intra and extra spinal anomalies, identification of all associated anomalies is essential.     

Occult intraspinal anomalies and congenital scoliosis

Of 251 patients with congenital scoliosis, occult congenital intraspinal anomalies were diagnosed in forty-six (18.3 per cent). A diastematomyelia was the commonest anomaly (forty-one patients). Other less common anomalies, occurring alone or in association with a diastematomyelia, were: neurenteric, epidermoid, and dermoid cysts; teratoma; lipofibroma; absence of nerve roots; fibrous bands; and a tight filum terminale. Intraspinal anomalies were associated with all types and sites of congenital scoliosis by far the highest incidence (52 per cent) occurred in association with a unilateral unsegmented bar with contralateral hemivertebrae in the lower thoracic or thoracolumbar regions. Thirty of the patients with an intraspinal anomaly had neural abnormalities, which usually affected only one lower extremity, and in twenty-four patients a paralytic foot deformity developed. Neural deterioration occurred in nine of these patients before the age of five years and was halted by excision of the anomaly. An additional twelve patients (4.8 per cent) of the 251 with congenital scoliosis also had a unilateral neural deficit in the lower limb and a paralytic foot deformity, similar to those found in the patients with an intraspinal anomaly, but had no myelographic evidence of a structural anomaly.     

Occult intraspinal anomalies in congenital scoliosis

Thirty consecutive patients with congenital spinal deformity underwent magnetic resonance imaging (MRI) to determine the incidence of occult intraspinal anomaly. These congenital spinal deformities included 29 cases of congenital scoliosis and one case of congenital kyphosis. Physical examination findings and plain radiographs were reviewed in an attempt to correlate these findings with subsequent intraspinal pathology. Nine patients had intraspinal anomalies identified on MRI consisting of five with tethered cord, four with syringomyelia, three with lipoma, and one with diastematomyelia. One patient required surgery for diastematomyelia; another underwent release of his tethered cord. Only one patient, with diastematomyelia associated with a syrinx and bifocal tethering, had his anomaly suggested by physical examination and plain radiographs. Two other patients had findings on plain radiographs previously associated with high prevalence of occult intraspinal anomalies; one patient with congenital kyphosis had a tethered cord, and one patient with a unilateral hemivertebrae associated with a contralateral bar had a tethered cord. Two of nine patients with occult intraspinal anomalies required surgery for their anomaly. In patients with a congenital spinal deformity, we found nine (30%) of 30 to have an associated anomaly within the spinal canal. Only three of these nine had plain radiographs and physical examination findings suggestive of their subsequent MRI findings. Given the poor correlation between findings on physical examination, plain radiographs, and subsequent occult intraspinal anomalies on MRI, we believe that MRI is helpful in evaluating patients with congenital spinal anomalies.     

A retrospective study of congenital scoliosis and associated cardiac and intraspinal abnormities in a Chinese population

Our objective is to assess the incidence of cardiac and intraspinal abnormities in Chinese congenital scoliosis (CS) patients and to study the relationship between the associated abnormities and the different CS types. Five-hundred and thirty-nine consecutive Chinese patients with CS were retrospectively studied, and the records of echocardiography, plain radiograph of the entire spine, magnetic resonance imaging of the entire spine and/or myelogram were reviewed. The results indicated that the incidence of cardiac and intraspinal abnormities in CS patients was 14.1 and 24.5%, respectively. There was no difference in the incidence of associated cardiac and intraspinal abnormities in different CS types (P > 0.05). The most common cardiac abnormities in CS patients was mitral valve prolapse, which was followed by congenital heart diseases, including atrial septal defect, ventricular septal defect, bicuspid aortic valve and patent ductus ateriosus. The cardiac abnormities were not likely to be concurrent with intraspinal abnormities in CS patients (P = 0.04). The intraspinal abnormities were more common in female and older patients (all P < 0.05). One or more abnormities mentioned above could be found in 36.8% CS patients and were more likely to be found in female patients (P < 0.01). We concluded that CS is not a simple abnormity, due to the high incidence of associated deformities of other organs, comprehensive assessment was strongly recommended before the surgical correction for CS patients.     

Intraspinal anomalies in scoliosis: An MRI analysis of 177 consecutive scoliosis patients

Background:

The association of intraspinal neural anomalies with scoliosis is known for more than six decades. However, there are no studies documenting the incidence of association of intraspinal anomalies in scoliotic patients in the Indian population. The guide lines to obtain an magnetic resonance imaging (MRI) scan to rule out neuro-axial abnormalities in presumed adolescent idiopathic scoliosis are also not clear. We conducted a prospective study (a) to document and analyze the incidence and types of intraspinal anomalies in different types of scoliosis in Indian patients. (b) to identify clinico-radiological ‘indicators’ that best predict the findings of neuro-axial abnormalities in patients with presumed adolescent idiopathic scoliosis, which will alert the physician to the possible presence of intraspinal anomalies and optimize the use of MRI in this sub group of patients.

Materials and Methods:

The data from 177 consecutive scoliotic patients aged less than 21 years were analyzed. Patients were categorized into three groups; Group A - congenital scoliosis (n=60), group B -presumed idiopathic scoliosis (n=94) and group C - scoliosis secondary to neurofibromatosis, neuromuscular and connective tissue disorders (n=23). The presence and type of anomaly in the MRI was correlated to patient symptoms, clinical signs and curve characteristics.

Results:

The incidence of intraspinal anomalies in congenital scoliosis was 35% (21/60), with tethered cord due to filum terminale being the commonest anomaly (10/21). Patients with multiple vertebral anomalies had the highest incidence (48%) of neural anomalies and isolated hemi vertebrae had none. In presumed ‘idiopathic’ scoliosis patients the incidence was higher (16%) than previously reported. Arnold Chiari-I malformation (AC-I) with syringomyelia was the most common neural anomaly (9/15) and the incidence was higher in the presence of neurological findings (100%), apical kyphosis (66.6%) and early onset scoliosis. Isolated lumbar curves had no anomalies. In group-C, incidence was 22% and most of the anomalies were in curves with connective tissue disorders.

Conclusion:

The high incidence of intraspinal anomalies in presumed idiopathic scoliosis in our study group emphasizes the need for detailed examination for subtle neurological signs that accompany neuro-axial anomalies. Preoperative MRI screening is recommended in patients with presumed ‘idiopathic’ scoliosis who present at young age, with neurological findings and in curves with apical thoracic kyphosis.     

Neurenteric cysts--a spectrum

This review encompasses seven patients with clinically important cystic lesions of the gastrointestinal (GI) tract, exhibiting a wide range of vertebral anomalies and connections to the neural canal. Three patients had mediastinal masses connected to lower cervical and upper thoracic anomalous vertebrae with intraspinal extensions. In addition, one of these patients had a separate, juxtapancreatic intestinal duplication cyst. One infant with colonic duplication had a lumbar vertebral anomaly and an epithelial-lined tract between the two. Another patient had a presacral cystic mass which was the site of recurrent infections and meningitis until a connection with the rectum was divided. A newborn baby had a completely split notochord syndrome with a large dorsal enteric fistula. Finally, one patient had a dorsal enteric cyst with a direct intraspinal connection. Four of the seven patients had associated significant congenital anomalies, two of whom died early in the neonatal period. The rest of the patients did well. This broad range of enteric lesions with associated vertebral and intraspinal abnormalities suggests that the clinical spectrum of neurenteric cystic lesions is much wider than is generally appreciated.     

体感诱发电位检查在先天性脊柱侧凸神经功能评估中的价值

目的 探讨体感诱发电位(SEP)检查在伴脊髓发育畸形的先天性脊柱侧凸(CS)中的诊断价值.方法 回顾性分析2001年9月到2007年9月诊治的187例CS患者临床资料,其中男性85例,女性102例;年龄3～22岁,平均13.8岁.所有患者均行全脊髓磁共振检查判断是否存在脊髓发育畸形.分析术前SEP的峰潜伏期及左、右侧峰潜伏期差值.SEP波形消失、峰潜伏期延长及峰潜伏期不对称定义为SEP异常.比较有无脊髓发育畸形患者的临床特征及SEP异常发生率的差异.结果 共有32例患者伴脊髓发育畸形.CSⅢ型(混合型)伴脊髓发育畸形比例(30.8%)高于Ⅰ型和Ⅱ型(P＜0.05).伴脊髓发育畸形组平均侧凸Cobb角大于无脊髓发育畸形组(P＜0.05),而两组平均后凸Cobb角差异无统计学意义(P＞0.05).伴脊髓发育畸形组SEP异常率与无脊髓发育畸形相比,差异有统计学意义(x2=4.70,P＜0.05).结论 SEP检查可以评估CS患者的神经功能状态,对CS伴脊髓发育畸形具有辅助诊断价值.     

Chest wall deformity in patients with repaired esophageal atresia

Chest wall deformities developed after thoracotomy for esophageal atresia, in 77 of 232 patients (33%) who did not have a congenital vertebral anomaly. Anterior chest wall asymmetry was present in 47, scoliosis in 18 and a combination of both in 12 patients. Scoliosis was convex away from the incision in two thirds of those affected. Anterior chest wall deformity was more common in patients greater than 25 years of age, and scoliosis was more common in patients who had had multiple thoracotomies. Breast surgery to minimize inequality was required in three female patients, and spinal surgery in one patient. Twenty-two of 53 patients with a congenital vertebral anomaly developed scoliosis, eight of whom required surgery. The scoliosis was probably the result of the vertebral anomaly in these patients, who are particularly at risk for progressive deformity.