马凡综合征脊柱侧凸的手术治疗

目的探讨马凡综合征脊柱侧凸的临床表现和手术方法,评价其疗效.方法回顾性分析1990年1月～2002年11月手术治疗的马凡综合征脊柱侧凸患者10例.评价手术前后侧凸冠状面、矢状面Cobb角、躯干偏移、顶椎旋转度(Nash-Moe法)及顶椎偏距等的变化.结果全部病例术后随访时间4个月～3年,平均15个月.躯干偏移术前平均2.17(0.5～8.0)cm,术后平均1.41(0.5～3.5)cm;顶椎旋转度手术后平均改善1°;顶椎偏距术前平均4.57(1～8.1)cm,术后平均2.14(0～5.5)cm.侧凸主弯冠状面Cobb角术前平均88°(49°～110°),术后平均42°(10°～90°),矫正率为46.38%(18.18%～81.54%),最后一次随访平均46°(11°～96°),平均丢失4°.胸椎后凸术前平均20°(-10°～52°),术后平均23°(0°～35°),最后一次随访平均24°(0°～35°).5例胸腰段后凸畸形Cobb角由术前的平均后凸85°纠正至术后的平均后凸10°.共有3例发生了术后并发症,1例发生假关节予密切随访,1例出现右股前区皮肤麻木经对症治疗1周后消失,1例腰段失代偿者行后路翻修术后取得满意效果.结论马凡综合征脊柱侧凸易产生术后并发症,三维矫形器械可以对马凡综合征脊柱侧凸进行有效地矫正.手术治疗的要点是充分考虑矢状面畸形的矫形、注意腰段或腰骶段的硬脊膜扩张、良好的植骨融合以及术后确切的外固定.     

马凡综合征性脊柱侧凸的治疗进展

马凡综合征是一种常染色体显性遗传病，患病率约为1／10000。最近的研究发现本病是由位于15号染色体长臂(15q21．2)上编码原纤维蛋白-1的基因-FBN1的突变所致。原纤维蛋白-1是一种大的糖蛋白，是细胞外基质中微原纤维的主要组成部分，而微原纤维参与构成弹力纤维。弹力纤维的异常可以解释马凡综合征的一系列临床表现。病变     

接受心脏手术的Marfan综合征患者合并脊柱侧凸的特点

目的:探讨接受心脏手术治疗的Marfan综合征患者合并脊柱侧凸的患病率、侧凸程度、类型及特点。方法:回顾性总结138例进行心脏外科手术的Marfan综合征患者的X线资料,测量冠状面和矢状面Cobb角,并对数据进行统计分析。结果:58例(42.03%)患者合并脊柱侧凸,男38例,女20例,男女患病率比例为1.18∶1,其中≤10岁6例,11～20岁12例,21～30岁19例,31～40岁11例,41～50岁7例,51～60岁3例;平均冠状面Cobb角为26.8°±27.8°;胸弯36例,胸腰弯11例,腰弯2例,双弯6例,三弯3例;单弯中顶椎凸向右侧38例,凸向左侧11例;矢状面胸椎后凸平均为14.3°±13.2°,其中胸椎前凸5例,胸椎后凸不足40例,胸椎正常后凸12例,仅1例胸椎后凸45°;11例患者冠状面Cobb角>40°,平均年龄15.9岁。结论:在接受心脏手术的Marfan综合征患者中脊柱侧凸患病率为42.03%;脊柱侧凸类型多样,冠状位畸形以胸弯和胸腰弯多见,胸椎凸向右侧发生率较高,矢状位畸形以胸椎后凸不足为主;在青少年表现较为严重,需要严密的随访及手术矫形。     

马凡氏综合征患者脊柱侧凸的矫治

目的:探讨马凡氏综合征(Marfansyndrome,MFS)患者脊柱侧凸的手术疗效。方法:对1997年9月至2002年1月使用脊柱三维矫形内固定系统后路手术治疗的8例MFS患者脊柱侧凸的矫正度、脊柱平衡及并发症等进行分析。结果:8例患者随访12~48个月,平均24个月。6例KingⅡ型患者胸弯及腰弯的矫正率分别为47%、57%,随访矫正率丢失分别为21%和16%;1例KingⅠ型患者胸弯及腰弯矫正率分别为25%及42%,随访矫正率丢失分别为21%和15%;1例KingⅢ型患者胸弯及腰弯的矫正率分别为58%、29%,随访矫正率丢失分别为9%和18%。所有患者躯干平衡得到改善,无神经并发症发生。2例出现交界性腰椎后凸,1例切口延迟愈合。结论:使用脊柱三维矫形内固定系统后路手术治疗马凡氏综合征患者脊柱侧凸可以获得满意疗效。     

重度脊柱侧凸的后路手术矫治

目的评价后路矫形内固定术治疗重度脊柱侧凸的疗效。方法重度脊柱侧凸患者16例,进行后路矫形内固定术治疗,术前主弯Cobb角71°-110°（84°±11°）,其中矢状面异常患者11例。结果手术时间3．4—5．1（4．1±0．6）h,出血量570—1120（778±178）ml,平均融合节段11（9—13）个椎体,术后主弯Cobb角24°-44°（31°±5°）,11例矢状面异常患者重新恢复了胸椎生理性后凸和腰椎生理性前凸,未发生感染、血气胸和神经系统等并发症。终末随访时,主弯Cobb角27°-45°（33°±5°）,矫正丢失率为0—13％（7％±5％）,固定范围内植骨全部融合,未发生术后失代偿和假关节形成。结论后路矫形内固定术是治疗重度脊柱侧凸安全有效的方法。     

后路凸凹侧双棒同步矫形在青少年特发性脊柱侧凸矫治中的应用

目的 探讨后路凸凹双侧矫形棒同步矫形技术在青少年特发性脊柱侧凸(AIS)外科矫治中的应用价值及疗效.方法 2006年2月至2008年8月采用后路凸凹侧双棒同步矫形技术治疗AIS 48例,其中男性16例,女性32例;年龄11～24岁,平均17.1岁.Lenke分型:Ⅰ型17例、Ⅱ型9例、Ⅲ型14例、Ⅳ型8例.行选择性胸弯融合27例,非选择性融合21例.观测手术前后冠状Cobb角、顶椎偏距、顶椎旋转、躯干偏移、尾端融合椎旋转、倾斜角、椎间角、矢状面平衡变化,评价侧凸矫正效果及脊柱平衡状况.结果 术后随访12～27个月,平均15.1个月.选择性融合患者末次随访胸、腰弯Cobb角平均矫正率分别为(76±11)%、(72±9)%.非选择性融合术患者末次随访胸、腰弯Cobb角平均矫正率分别为(74±15)%、(69±9)%.所有病例尾侧保留脊柱活动节段平均4.4个.1例因术中定位错误发生近端交界性后凸行翻修术.其余病例末次随访无脊柱失代偿,无假关节形成、神经损伤等并发症.结论 应用后路凸凹双侧矫形棒同步矫形治疗AIS,有助于提高矫形效果,重建和维持脊柱平衡,减少尾侧融合节段.     

特发性脊柱侧凸手术并发症分析

目的：探讨特发性脊柱侧凸手术并发症及预防方法。方法：2002年6月至2007年5月治疗特发性脊柱侧凸86例,男21例,女65例;年龄8～22岁,平均17.8岁。侧凸按Lenke分型,1型33例,2型10例,3型18例,4型5例,5型10例,6型10例。Risser征0～Ⅰ度5例,Ⅱ～Ⅲ度20例,Ⅳ～Ⅴ度61例。术前患者冠状位主侧凸Cobb角45°～85°,平均Cobb角60.35°,采用TSRH等钉棒系统进行全脊柱椎弓根螺钉三维矫形、融合固定技术。对手术后矫形情况及手术后并发症进行总结。结果：所有患者平均手术时间3.2h,平均出血1000ml（800～2400ml）,共置椎弓根螺钉924枚,术后平均Cobb角18.46°。全部患者均获随访,时间5～40个月,平均20.5个月。86例患者中,1例脊髓损伤;25枚螺钉偏位;2例神经根损伤;1例胸膜损伤;1例肠系膜上动脉综合征;3例手术切口感染;2例躯体失平衡;1例交界性后凸;3例内固定松动;2例假关节形成;1例曲轴现象;2例平腰畸形。结论：特发性脊柱侧凸手术有很多种并发症,正确的诊治方案是减少并发症的关键。     

重度脊柱侧凸的手术治疗

目的:评价重度脊柱侧凸的手术治疗方法和疗效。方法:65例重度脊柱侧凸患者接受手术治疗。特发性脊柱侧凸17例,先天性脊柱侧凸30例,其它类型脊柱侧凸18例。术前侧凸Cobb角82°~171°,平均108°,其中32例合并后凸,后凸Cobb角60°~108°,平均81.8°。冠状面C7垂线偏移骶中线21~62mm,平均29.7mm。所有患者均有不同程度的肺功能减退,最大肺活量平均为正常值的52%。术前均进行详细的临床和影像学检查评价。根据患者的畸形部位、程度和特点选择包括一期或分期前路松解、半椎体切除、椎体截骨、后路松解、内固定、融合术等进行矫正。结果:所有患者安全完成手术,无死亡及神经损伤并发症发生。术后侧凸矫正36°~102°,平均53°(49.1%),后凸矫正31°~68°,平均47°(58.4%),C7垂线偏移平均矫正21.2mm。随访12~72个月,平均32个月,侧凸和后凸平均丢失分别为12°和8°,无假关节形成。最后随访时81%的患者对治疗结果表示满意。结论:详细全面的术前检查和适当的手术方法选择是重度脊柱侧凸矫正手术的关键,术中和术后的严密观察和及时处理并发症是获得手术安全的保证。     

马凡综合征所伴脊柱侧凸的自然史及干预措施

马凡综合征(Marfan syndrome,MFS)是一种常染色体显性遗传的结缔组织异常,累及多个系统,包括眼、心血管、骨骼系统,其人群发病率约为0.02%～0.03%[1].     

Superior mesenteric artery syndrome associated with scoliosis treated by a modified Ladd procedure

A 16-year-old asthenic girl had idiopathic adolescent scoliosis and superior mesenteric artery (SMA) syndrome. After a Harrington rod procedure, the SMA syndrome produced a complete duodenal obstruction that did not resolve with nasogastric decompression, positioning, or peripheral intravenous nutrition. A complete derotation of the duodenum and the colon and stabilization of their mesentery (Ladd procedure with the Bill modification) resolved the obstruction and was considered the preferred operative treatment for this problem.     

Severe scoliosis associated with Costello syndrome: a case report

Costello syndrome is characterised by dwarfism, unique cutaneous lesions, a distinct facial gestalt, and mental retardation. There have been no detailed reports of severe spinal deformities requiring surgical treatment as a complication of Costello syndrome. We report a case of a 10-year-old girl with progressive scoliosis associated with Costello syndrome. She underwent anterior release and posterior surgical correction and fusion from T5 to L2 using a third generation hook and rod system plus spinous process wiring. Congenital portal vein deficiency and coagulopathy were other major complications. At 15-month follow-up, the patient had good balance and no evidence of instrumentation failure.     

Anesthetic management of a child with Klippel-Feil syndrome associated with severe scoliosis]

We experienced an anesthetic management of a female child with Klippel-Feil syndrome complicated with severe scoliosis. This patient showed airway problems in anesthetic management because of limited neck movement due to fused cervical supine and winding airway due to scoliosis. A laryngeal mask (LM) was useful for ventilation but we fitted LM carefully because of her orolaryngeal anomaly. Intubation was very difficult through LM because the axis of LM was not on the laryngeal axis and the tip of the tracheal tube caught the epiglottis and arytenoid cartilage.     

Surgical treatment of scoliosis associated with Marfan syndrome by using posterior-only instrumentation

Surgical treatment of scoliosis associated with Marfan syndrome poses a challenge to spine surgeons. This retrospective study was undertaken to determine whether posterior-only surgery with instrumented fixation and fusion addresses the correction of scoliosis and maintains curve correction. Twelve consecutive patients with Marfan syndrome were treated between 2002 and 2007 for scoliosis by posterior segmental instrumentation using pedicle screws or hybrid thoracic-hook and lumbar-screw constructs. Their preoperative Cobb angle averaged 66 ± 10° (range: 55-90°). The average operation time was 252 ± 36 min (range: 200-300 min) and the average blood loss was 690 ± 117 ml (range: 550-920 ml). No significant complications were found. All the patients were followed for a minimum of 2 years (range: 2.4-6.8 years). The average Cobb angle was corrected to 23 ± 8° (range: 13-35°) immediately after surgery and 28 ± 9° (range: 14-43°) with a correction rate of 58 ± 13% at final follow-up. The results indicate that posterior-only surgery with instrumented fixation and fusion is effective and safe for the treatment of scoliosis in selected patients with Marfan syndrome.     

The characteristics of thoracic insufficiency syndrome associated with fused ribs and congenital scoliosis

1. Thoracic insufficiency syndrome is the inability of the thorax to support normal respiration or lung growth. 2. The rare condition of fused ribs and congenital scoliosis may result in a three-dimensional thoracic deformity with adverse effects on thoracic growth and function with development of thoracic insufficiency syndrome. 3. The normal thorax is defined by two characteristics: normal, stable volume and the ability to change that volume. Volume depends on the width and depth of the rib cage, and the thoracic spine provides height. The ability to change volume, termed thoracic function, is provided by the diaphragm and the secondary muscles of respiration. 4. On radiographs, the loss of the vertical height of the lung of the concave, restricted hemithorax is defined by the percentage of space available for the lung. 5. Spine rotation causes a windswept thorax, with both restriction of the volume of the convex hemithorax and restriction of the motion of the involved ribs. 6. Constrictive three-dimensional deformity of the thorax may cause extrinsic, restrictive lung disease. 7. Progressive thoracic insufficiency syndrome is diagnosed on the basis of clinical signs of respiratory insufficiency, loss of chest wall mobility as demonstrated by the thumb excursion test, worsening indices of three-dimensional thoracic deformity on radiographs and computed tomography scans, or a relative decline in percent predicted vital capacity due to thoracic "failure to thrive," as demonstrated by pulmonary function tests. 8. Treatment of progressive thoracic insufficiency syndrome should provide an acute increase in the thoracic volume with stabilization of any flail chest-wall defects and maintain these improvements as the patient grows, without the need for spine fusion.     

Sagittal balance in scoliosis associated with Marfan syndrome: a stereoradiographic three-dimensional analysis

Purpose Marfan syndrome (MFS) is a genetic disease often marked by the presence of scoliosis. There is no three-dimensional analysis of the deformity in the literature. Our aim was to determine what kind of sagittal balance defines scoliosis associated with MFS, namely a flexion deformity, as it is in scoliosis associated with Chiari I or an extension deformity, as in adolescent idiopathic scoliosis (AIS). To address this issue, we compared the presence or absence of a thoracic scoliosis with the presence or absence of a segment in extension in the thoracic spine. Methods In our series, 30 patients diagnosed with Marfan syndrome were prospectively included. In each patient, personalized three-dimensional reconstruction from T1 to L5 of the spine was made using stereoradiography. The patients were first separated based on the presence or absence of thoracic scoliosis, in order to compare this with the presence or absence of a segment in extension in the thoracic spine. They were then classified into two groups based on the presence or absence of the segment in extension (meaning containing negative values of inter-vertebral sagittal rotation) in the thoracic spine. Results Among scoliotic patients with a thoracic scoliosis (17 cases), there were 13 (76.5% cases) with a segment in extension in the thoracic spine and 4 with no segment in extension. Conclusions Our results showed that scoliosis associated with MFS is somehow original, demonstrating a sagittal balance in extension (as AIS) in about 80% of thoracic curves, but without this characteristic feature in about 20%.     

一期后路矫形治疗脊柱侧弯合并无症状脊髓栓系患者的临床研究

[目的]评价一期后路矫形治疗脊柱侧弯伴无症状脊髓栓系患者的手术疗效。[方法]收集中南大学湘雅医院脊柱外科2009~2014年因脊柱侧弯行矫形手术的脊柱侧弯伴无症状脊髓栓系的手术患者17例,男5例,女12例,年龄9~17岁,平均14.8岁。通过对比患者术前、术后及随访时矢状面X线片,测量脊柱冠状面Cobb角,并行SRS-22评分及m JOA评分。[结果]本组患者术前平均冠状面Cobb角(58.4±12.6)°,术后为(23.8±6.4)°,侧弯得到明显矫正(P<0.01);末次随访时平均Cobb角为(22.4±6.8)°,与术后相比无明显矫正度数丢失(P>0.05)。术前SRS-22总分(69.82±4.86)分,其中包括:功能活动评分(18.65±2.34)分、疼痛评分(20.94±1.74)分、自身形象评分(14.47±2.18)分、精神健康评分(15.76±2.05)分;术后1年随访患者SRS-22总分(86.53±4.14)分,其中包括:功能活动评分(20.71±1.73)分、疼痛评分(21.88±1.36)分、自身形象评分(18.06±2.28)分、精神健康评分(18.94±1.98)分。患者手术满意程度评分(7.94±1.34)分。与术前相比,患者各方面评分均有所提高(P<0.05),其中患者的自身形象评分及精神状况评分明显提高。本组患者术前改良日本骨科学会评分(m JOA-score)总分为(23.65±1.77)分,其中包括:主观症状评分(7.00±0.87)分;临床症状评分(5.41±0.62)分;日常活动评分(11.59±0.94)分;膀胱功能评分(-0.35±0.99)分。术后患者评分改善至总分(26.35±1.53)分,其中主观症状评分(8.65±0.49)分;临床症状评分(5.76±0.44)分;日常活动评分(12.47±0.94)分;膀胱功能评分(-0.53±1.17)分,除膀胱功能(P=0.668)无明显改善外,其余各项均有所改善(P<0.01)。[结论]一期后路矫形治疗脊柱侧弯合并无症状脊髓栓系是一种安全有效的手术方法。