CAJ | 학술논문

目的本研究拟通过对先天性缩窄环综合征(congenital constriction ring syndrome,CCRS)分两期手术的治疗效果进行分析,探讨该病手术治疗的有效策略.方法 2007年1月至2012年1月,分两期手术治疗手部先天性缩窄环综合征患儿50例(195指).平均随访23.9个月.两期手术中,一期完整切除背侧的缩窄环,皮下筋膜瓣充填软组织缺损.远端手指无明显淋巴水肿时,行“Z”字成形；淋巴水肿明显时,行横行切口.二期在一期术后6个月于掌侧进行相同的术式.术后对手指外形、瘢痕形成及并发症情况进行观察.结果所有患儿均分两期手术.交叉并指发生率54％(27/50),淋巴水肿28％ (14/50).两期术后,78.5％ (153/195)的手指的缩窄环基本消失,无沙漏样畸形.88％ (44/50)的患儿及家长对术后手指外形及瘢痕满意.无皮瓣坏死及手指坏死等并发症.12％(6/50)的患儿需要进一步手术.结论分两期手术切除缩窄环避免了术后出现手指血运障碍的风险.完整切除缩窄环和皮下筋膜瓣充填软组织缺损是获得满意效果的关键步骤.根据手指淋巴水肿情况选择不同的切口,有助于改善术后手指的外观.
목적 본연구의통과대선천성축착배종합정(congenital constriction ring syndrome,CCRS)분량기수술적치료효과진행분석,탐토해병수술치료적유효책략.방법 2007년1월지2012년1월,분량기수술치료수부선천성축착배종합정환인50례(195지).평균수방23.9개월.량기수술중,일기완정절제배측적축착배,피하근막판충전연조직결손.원단수지무명현림파수종시,행“Z”자성형；림파수종명현시,행횡행절구.이기재일기술후6개월우장측진행상동적술식.술후대수지외형、반흔형성급병발증정황진행관찰.결과 소유환인균분량기수술.교차병지발생솔54％(27/50),림파수종28％ (14/50).량기술후,78.5％ (153/195)적수지적축착배기본소실,무사루양기형.88％ (44/50)적환인급가장대술후수지외형급반흔만의.무피판배사급수지배사등병발증.12％(6/50)적환인수요진일보수술.결론 분량기수술절제축착배피면료술후출현수지혈운장애적풍험.완정절제축착배화피하근막판충전연조직결손시획득만의효과적관건보취.근거수지림파수종정황선택불동적절구,유조우개선술후수지적외관.
Objective Congenital constriction ring syndrome (CCRS) is among the most common congenital deformities of the hand.However,few techniques have been described for correction of this anomaly,and none of these techniques could provide complete correction of the deformity.We describe our two-stage surgical strategy and report the treatment outcomes.Methods From January 2007 to January 2012,50 children (195 digits) with CCRS in the hand underwent two-stage surgery.The follow-up period averaged 23.9 months.At stage I,following excision of the dorsal fibrotic constriction ring,the resulting defect was filled with a subcutaneous fascial flap.In the absence of distal finger lymphedema,skin closure was done with Z-plasty.When there was obvious lymphedema,a transverse incision was used.Similar procedures were done on the volar aspect of the finger at stage Ⅱ 6 months after the first surgery.Appearance,scar formation and complications were observed postoperatively.Results All the patients underwent the two-stage operation.Acrosyndactyly was present in 54％ (27/50) of the patients.Lymphedema occurred in 28％ (14/50) of the cases.In 78.5％ (153/195) of the digits,a normal finger contour was obtained,with conplete elimination of the sandglass deformity caused by the constriction ring.The scars were found to be esthetically acceptable in 88％ (44/50) patients.There were no complications like flap loss or finger necrosis.Further surgical correction was deemed necessary in 6 cases (12％).Conclusion Two-stage surgical resection of the constriction ring can avoid the potential risk of circulation compromise.Complete excision of the constriction ring and filling the resulting circular groove with subcutaneous fascial flaps are key to achieving a normal contour.Selection of the incision depending on the degree of lymphedema helps to improve the results.