先天性右侧腓骨发育畸形1例

1 病历简介 患者，男，16岁。右下肢跛行3年余，但未影响正常生活，未予足够重视，现感行走时跛行加重、偶有酸痛而入院。查体：生命体征正常，神志清楚，头颅、五官、躯体检查未见异常，发育良好、无畸形，肢体活动尚自如；心肺腹部检查未发现异常；神经系统检查阴性。专科检查：右侧小腿相对左侧稍细，小腿围较健侧约小5．2cm，触及轻压痛，皮温与健侧相同。X线右小腿正侧位片（图1）示：     

肝右叶缺如伴右侧胆管及右肾发育畸形一例

病例资料患者,女,43岁,右上腹疼痛数年,加重5 d。右上腹压痛,无反跳痛及移动性浊音,皮肤巩膜无黄染。多层螺旋CT平扫示肝脏体积明显缩小,胆囊位于肝脏右后方,在肝脏后方并可见迂曲的扩张的胆管,肝左叶胆管稍扩张,肝总管也明显扩张,右肾形态失常,体积明显缩小(图1)。增强扫描门静脉期见肝脏均匀强化,肝右叶未见明确显示,肝右叶胆管结构显示失常,门静脉主干及左支显示较清晰,右支未显示;右肾强化欠佳,体积明显缩小,形态不规整,皮质变薄,肾盂、肾盏变小,右肾动脉显示较细,左肾显示正常(图2、3)。剖腹探查显示肝右叶缺如,肝右胆管发育畸形扩张…     

先天性二尖瓣及三尖瓣发育畸形1例

患者男性．20岁。因憋喘及心前区杂音来诊。听诊：心尖区可闻及舒张期滚筒样杂音．并伴有震颤．肺动脉瓣区第二心音亢进。超声心动图显示：双房、右室扩大．左室腔内径偏小．右室壁增厚（约11mm）．室间隔及左室游离壁厚度及动度正常。     

肋骨骨样骨瘤1例

骨样骨瘤在临床上较多见,多发生于股骨、胫骨等长管骨,少见于短管骨、脊椎、跟骨等不规则骨,偶见于颅骨。肋骨发病者迄今未见报道,现报道一例经手术病理证实的肋骨骨样骨瘤。男,14岁。发现右肩胛骨下方硬性肿物1月余,伴间歇性疼痛,夜间稍重。肿物1月内逐渐增大,宽基底,质硬,不活动,皮肤无发热,颜色无改变。患者自行按摩及外敷红花油后疼痛非但不减轻,反而逐渐加重,未曾服用水杨酸类药物,遂来我院就诊。影像学检查:X线正位片见右侧第8后肋近腋缘处示环状高密度影,大小约4·2 cm×3·4 cm,边缘较光滑,高密度环较厚,厚度约为1·1 cm,中央区为…     

右侧肺动脉缺如1例

1 临床资料 患者为女性,30岁,主因间断咯血4个月余于2005年5月24日入院.患者4个月余前无明显诱因出现双眼花、右耳及右面部麻木感,右侧肢体无力伴胸闷,随后出现咯鲜血40～60 ml,伴意识障碍.     

右侧第6肋骨软骨粘液样纤维瘤1例

患者 女性,37岁。1月前,无明显诱因出现右胸背部疼痛,无发热、咳嗽、盗汗等,既往身体健康。查体：右胸第5、6胸椎旁压痛,其他未见阳性体征。X线胸片：右上纵隔旁可见一约3．0cm×2．0cm的团块状高密度影,外缘光滑,内侧欠清,第5胸椎右侧可见弧形压迹,第5、6肋骨骨质结构欠清（图1）。X线诊断：右上纵隔旁占位,建议进一步检查。逐行CT检查：右上后纵隔近第5、6胸椎处可见一约3．0cm×2．0cm的软组织肿块影,肋骨可见骨质破坏,     

I期后路全脊椎切除治疗重度胸腰椎畸形的神经系统并发症

目的 探讨应用I期后路全脊椎切除治疗重度胸腰椎畸形的神经系统并发症,并分析相关危险因素.方法 2000年2月-2010年9月接受I期后路全脊椎切除治疗的重度胸腰椎畸形患者67例,男29例,女38例;年龄14～62岁,平均31.4岁.其中青少年(年龄<18岁)21例,成人(年龄≥18岁)46例.侧凸畸形11例,平均冠状面主弯Cobb角90.4°;侧后凸畸形25例,冠状面主弯Cobb角94.5°,后凸角度平均65.5°;角状后凸畸形28例,平均后凸角74.3°;圆弧状后凸3例,平均后凸角91.1°.初次手术患者59例,翻修患者8例.采用主弯区顶椎全脊椎切除,全节段椎弓根螺钉内固定矫形和360°植骨融合术,统计神经系统并发症的发生情况.结果 平均随访时间14个月(3～69个月),出现神经系统并发症者共8例(11.9%),其中严重神经并发症3例,发生率4.5%,包括1例大量失血血容量灌注不足导致完全性脊髓损伤.轻度神经并发症患者5例,发牛率7.5%.胸椎全脊椎切除的神经损伤发生率要明显高于腰椎(P<0.05).多个椎体切除的并发症发牛率显著增加(P<0.05).术前已经伴有或者不伴有神经损害表现患者的神经并发症发生率分别为33.3%和7.3%(P<0.05),翻修手术的并发症发生率明显增加(P<0.05).差异虽无统计学意义(P>0.05),但出现神经系统并发症的8例患者术前均合并有严重的后凸畸形(>60.).结论 I期后路全脊椎切除是外科治疗重度胸腰椎畸形有效手术方式,但神经并发症应引起关注.相关神经损伤危险因素包括术中操作不当、大量失血、术前已经有神经受损表现、胸段截骨、多个椎体切除、翻修手术和严重后凸.

Abstract：

Objective To analyze the neurological complications in treatment of severe thoracolumbar spinal deformity with one stage posterior vertebral column resection (pVCR) and discuss the related risk factors. Methods There were 67 patients with severe thoracolumbar spinal deformity who underwent one-stage pVCR from February 2000 to September 2010.There were 29 males and 38 females at an average age of 31.4 years old(range,14-62 years).There were 21 patients at age less than 18 years old and 46 at age more than 18 years old.Patients were divided into four pathological types:severe scoliosis group(n=11,mean Cobb angle 90.4°),kyphoscoliosis group(n=25,mean scoliosis 94.5°,and mean kyphosis 65.5°),angular kyphosis group(n=28,mean kyphosis 74.3°)and global kyphosis group(n=3,mean kyphosis 91.1°).of all the patients,59 patients underwent primary surgery and eight underwent revision surgery.Surgical methods included posterior apex vertebral column resection,segemental pedicle screw fixation and correction as well as 360° bone fusion.Neurological complication was statistically analyzed. Results The average follow-up was 14 months (range,3-69 months),which showed severe neurologic complication in eight patients(11.9%)after surgery.Severe neurologic complication occurred in three patients (4.5%),among whom one patient presented delayed complete paraplegia 23 hours after surgery.Five patients had mild neurologic deficits(7.5%),the incidence of which was higher than 23.1%for thoracic osteotomy (P<0.05).Multilevel pVCR had high rate of neurological complications (P<0.05).The incidence rate was 33.3% for patients with preoperative neurologic compromise and 7.3%for patients mthom preoperative neuroiogic compromise (P<0.05).The incidence rate was increased in the revision surgery (P<0.05).Eight patients with neurological deficits had kyphotic angle of raore than 60°although there was no statistical difference (P>0.05). Conclusions pVCR is an effective surgical method for the correction of severe thoracolumbar spinal deformity.The neurological complications,however,should be paid attention to the surgeons.The risk factors for neurologic complications include improper manipulation,massive blood losing,preoperative neurologic compromise,osteotomy at thoracic rein,multi-level vertebrectomy,revision surgery and severe kyphosis.