先天性上尺桡关节融合1例

先天性上尺桡关节融合是一种罕见的疾病，现将我院治疗的1例病人报告如下。     

先天性家族性指(趾)关节融合症23例报告

先天性家族性指趾关节融合症在临床上极为罕见,确切病因不明。我院发现同一家族２３例患有此种先天性畸形,并对该组病例进行调查和相应的系谱分析图１。临床资料该家族位于广西桂北山区,壮族,家族成员４６人,查出患有先天性近侧指（趾）关节融合症者２３例,其中男１１例,女１２例;年龄最大６５岁,最小４岁。指关节融合者２３例,其中包括既有指关节融合,又有趾关节融合者,但趾关节融合者数目不详。所有病例均是近侧指趾间关节受累,畸形指趾节指趾间横纹消失或变浅,近节和中节指趾连成一条形指趾节,指趾间关节强直,活动度为０…     

近端指间关节周围胶原沉积症2例

近端指间关节周围胶原沉积症(pachydermo-dactyly,PDD)是一种主要累及双手近端指间关节的慢性非炎症性疾病,好发于青少年男性.本病较为少见,文献多为个案报道,易误诊为幼年型类风湿关节炎(juvenile rheumatoid arthritis,JRA)、幼年特发性关节炎(juvenile idiopa...     

AO螺钉在远位指间关节融合中的应用

目的探讨采用AO螺钉进行手指远位指间关节(DIP)融合的手术技巧和临床应用价值。方法采用AO螺钉内固定进行DIP融合手术。结果本组获随访3个月～1年,15例(23指)DIP病变患者融合术后的DIP关节4周获骨性愈合14指,6周愈合4指,8周愈合1指,12周愈合3指,1例(1指)因末节指骨骨吸收,术后3个月融合关节未获骨性愈合,手术取出内固定物。结论应用AO螺钉进行手指DIP融合术具有操作简便、术后并发症少、疗效确切、可早期功能训练的优点。     

指间关节置换术治疗指间关节僵直五例

指间关节损伤，造成指间关节活动受限、僵直或屈曲畸形，直接影响病人日常工作和生活。虽然治疗方法较多，但效果不甚理想。笔者采用人工指间关节置换术治疗指何关节陈旧性损伤．关节僵直5例．效果满意。报告如下。     

先天性上尺桡关节融合：附1例报告

先天性上尺桡关节融合 ( Congenital Proximalradioulnar Synostosis)是一种上肢严重的畸形 ,前臂固定于旋前位不能旋后为其主要特征。前臂旋转功能丧失妨碍了手精细动作的完成 ,给患者的日常生活工作造成严重影响。本病在先天性骨骼畸形中极为少见 ,1 973年 Sandifort首先描述了这一先天性畸形 ,1 985年 Gleary通过文献检索认为该病全世界不足 30 0例[1] 。最近我们收治 1例 ,现报告如下。病例报告患儿 ,女 2岁。因出生后发现双前臂旋转功能障碍 2年而入院。无外伤史、无家族史 ,第二胎足月顺产儿 ,身高、体重发育无异常。下肢活动无障碍…     

AO微型钉板在指间关节融合术中的临床应用

目的探讨手指间关节融合术方法与疗效。方法采用AO微型钉板进行指间关节融合31例。结果本组获随访3～24个月,9例术后1.5个月达到骨性愈合,20例术后2.5个月达到骨性愈合,2例术后4.5个月达到骨性愈合。伤指术后疗效评定结果:良29例,可2例。结论 AO微型钉板应用于近指间关节融合中,伤指骨质愈合及活动有优势,但对于远指间关节(拇指除外),术后有钉板外露等并发症,手术需慎重。     

双手先天性近指间关节融合症1例

<正>患者,男,38岁,因摔伤致左手中、环指疼痛2 h就诊。查体:双手第3~5指近指间关节强直,主动、被动功能完全受限;左手中指肿胀、畸形,指背皮肤破裂出血;双手掌指关节、远指间关节及拇指活动均正常。X线检查提示:双手示、小指及右手环指近指间关节间隙消失,骨融合;右手中指近指间关节部分融合,关节间隙模糊;左手环指近指间关节骨融合处连续性中断伴脱位。见图1。追     

先天性上尺桡关节融合治疗2例

先天性上尺桡关节融合是一种较少见的先天性骨骼畸形,是桡骨与尺骨的近侧端形成骨性连接,并有不同程度的旋前畸形.目前,根据是否恢复上尺桡关节的旋转功能分为分离融合区重建术及截骨矫形术两大类,后者又根据单次矫形的程度分为旋转截骨术及截骨后Ilizarov外固定架矫形术[1].虽然近年来手术疗效逐步改善,但仍不十分满意.现就笔...     

手先天性并指畸形的治疗

目的 介绍手先天性并指畸形的治疗。方法 42例先天性并指畸形患者中，2指并指36例，多指并指6例；软组织并指30例，远节指骨骨性并指12例。均采用并指间背侧矩形皮瓣形成指蹼，指间掌背侧锯齿状皮瓣进行分指。皮瓣移位后指部外露创面用游离植皮覆盖，指端骨外露者用局部皮瓣覆盖。结果 3例的游离植皮部分坏死，创面经换药后愈合；39例的创面均I期愈合。术后1年12例失访，30例的指蹼坡度良好，指尖部软组织饱满，手术指的外展良好。12例术毕因指间关节侧弯，术后用支架纠正畸形，效果满意。结论 并指畸形的治疗关键是指蹼的形成，指间关节侧弯术后使用支架纠正为一良好的方法。     

先天性膝关节脱位1例

1病例资料患儿,男,足月产,1月龄。产后即发现右膝关节过伸畸形,于2006年8月就诊。查体:被动屈曲明显受限,膝前部皮肤皱褶明显,腘窝皮肤皱褶消失,可触及髌骨,髌骨形态大小均正常;股骨髁向后,胫骨向前移位,被动屈膝时有弹性反抗,被动活动膝关节可达过伸30°以上,胫骨可以前后推移,股四头肌无挛缩,髌上囊触不清,无其他畸形。X线片示:胫骨向前移位。无家族史,其母亲无致畸药物服用史。治疗:经手法整复,右膝关节可恢复至屈曲80°左右,但有一定张力。用自制的铝板固定膝关节,每日按摩并加大关节屈曲角度,及时调节铝板屈曲度。28 d后摄X线片示:双…     

先天性分裂手畸形的手术治疗

目的 介绍治疗Ⅰ～Ⅲ型先天性分裂手畸形(Manske-Halikis分型法)的手术方法.方法 2005年3月至2010年7月,手术治疗Ⅰ～Ⅲ型先天性分裂手畸形共8例(9侧),另有分型外的特殊类型2例.其中采用Barsky法治疗Ⅰ型2例,Barsky法联合虎口开大全厚皮片植皮治疗ⅡB型1例,Ueba法治疗ⅡB型1例,Snow-Littler法治疗Ⅲ型3例,Ueba法治疗Ⅲ型1例2侧,术后早期虎口开大支具固定,并逐渐调整,持续固定8～ 12周.另有分型外的特殊类型2例,采用皮瓣转移、第五掌骨截骨小指移位固定第4或第3掌骨手术.结果 8例Ⅰ～Ⅲ型先天性分裂手畸形中,6例术后获得1～6年的随访(2例失访),裂口矫正后重建指蹼接近正常,外形满意.虎口角度为70°～90°,拇指可与其他手指对指,患手恢复了抓捏功能.2例特殊类型分裂手畸形的患儿,手掌的外形取得了明显的改善,小指功能不尽满意.结论 治疗先天性分裂手畸形的关键是通过皮瓣转移和骨转移来矫正裂开畸形和虎口重建,选择Snow-Littler法治疗Ⅲ型分裂手,可达到理想的外形和功能.     

先天性手部关节挛缩症的治疗

目的 探讨先天性多发性手部关节挛缩症手术方法的选择.方法 对8例(23指)先天性手部关节挛缩症的患儿,分别采用关节囊掌板松解、指浅屈肌腱止点切断、深浅肌腱交替术、皮片移植术等方法,术中以挛缩的关节能被动伸直为标准,采用克氏针内固定和术后石膏外固定相结合的方法进行治疗.结果 术后23指伤口均I期愈合.随访时间为12～25个月,关节功能及手指外形良好,除1例(4指)出现肌腱轻度粘连外,7例中14指(累及掌指关节1指,近指间关节13指)主、被动活动达到正常.其余手指背伸损害值V伸=5°～10°.结论 手部先天性多发性关节挛缩症根据组织的挛缩程度,通过上述方法可获得良好的治疗效果.     

A case of congenital midline cervical cleft with congenital heart disease

A congenital midline cervical cleft (CMCC) is a rare developmental anomaly. It may represent failure of the branchial arches to fuse in the midline and presents at birth with a ventral midline defect of the skin of the neck. Congenital heart disease along with CMCC is rarer, and most of the cases reported are associated with chest wall defects or thoracic ectopia cordis. The authors report a case of a 5-month-old girl with CMCC and an atrial septal defect (ASD) and discuss the clinical presentation, embryologic development, and treatment.     

A unique case of a giant congenital pulmonary hemangioma in a newborn

Congenital pulmonary hemangiomas are extremely rare lesions. We present a unique case of a giant congenital pulmonary hemangioma in an otherwise healthy newborn who presented with respiratory distress at birth. This was managed with resection after an unsuccessful trial of nonoperative management. The clinical, radiographic, and pathologic features of this unique case are discussed in the context of a review of the literature.     

Developmental biology and classification of congenital anomalies of the hand and upper extremity

Recent investigations into the mechanism of limb development have clarified the roles of several molecules, their pathways, and interactions. Characterization of the molecular pathways that orchestrate limb development has provided insight into the etiology of many limb malformations. In this review, we describe how the insights from developmental biology are related to clinically relevant anomalies and the current classification schemes used to define, categorize, and communicate patterns of upper limb malformations. We advocate an updated classification scheme for upper limb anomalies that incorporates our current molecular perspective of limb development and the pathogenetic basis for malformations using dysmorphology terminology. We anticipate that this scheme will improve the utility of a classification as a basis for diagnosis, treatment, and research.     

Non-vascularized free toe phalanx transfers in congenital hand deformities--the Great Ormond Street experience

Many options of varying complexity are available for the management of congenital short digits resulting from aphalangia in symbrachydactyly and constriction ring syndrome. We have used non-vascularized free toe phalanx transfers for these children when a vascularized toe transfer has been contraindicated. We describe our technique and experience with 22 children who underwent a total of 64 transfers of the proximal (35) or middle (29) toe phalanges (average 3 per child). The mean age at initial surgery was 15 months, and the mean follow-up was 5 years. Duration of time until epiphyseal closure could not be determined accurately, but total digital elongation averaged 6mm. Complications of this technique include joint instability, premature epiphyseal closure and, in one patient, infection and graft loss. Donor site deformity was determined according to measured growth deficit and toe function. This technique is a simple option for digital elongation and, if performed in the appropriate age group in short fingered and monodactylous subtypes of symbrachydactyly, has the potential to allow growth and function with minimal donor site deficit.