中华手外科杂志
中華手外科雜誌
중화수외과잡지
CHINESE JOURNAL OF HAND SURGERY
2014年
1期
37-40
,共4页
畸形,多发性%Apert综合征%早期诊断
畸形,多髮性%Apert綜閤徵%早期診斷
기형,다발성%Apert종합정%조기진단
Abnormalities,multiple%Apert syndrome%Early diagnosis
目的 探讨Apert综合征的临床表现、诊断及治疗.方法 第1次并指分离应在患儿9个月时开始,随后的治疗最好能在5岁前完成.分离手指从边缘的手指开始,再行中间手指.1岁以下患儿采用双侧同时手术.首先重建第1指蹼,随后再同时分离第2和第4指蹼.采用“Z”形切口分指,指间背侧矩形皮瓣重建指蹼,游离全厚皮片覆盖皮肤缺损.结果 2003年至2012年间5例患者诊断为Apert综合征,其中2例诊断后即行并指分离手术,术后未发生植皮坏死及感染,无病例最终分离5个手指,拇指桡偏畸形和指间关节僵硬均无进一步矫正.术后短期随访,手部外观和功能得以改善,家属对术后外观及功能改善满意,但长期结果有待进一步随访.结论 Apert综合征手并指严重,需要采取多次复杂的外科手术治疗.术后手部外观和功能得以改善.
目的 探討Apert綜閤徵的臨床錶現、診斷及治療.方法 第1次併指分離應在患兒9箇月時開始,隨後的治療最好能在5歲前完成.分離手指從邊緣的手指開始,再行中間手指.1歲以下患兒採用雙側同時手術.首先重建第1指蹼,隨後再同時分離第2和第4指蹼.採用“Z”形切口分指,指間揹側矩形皮瓣重建指蹼,遊離全厚皮片覆蓋皮膚缺損.結果 2003年至2012年間5例患者診斷為Apert綜閤徵,其中2例診斷後即行併指分離手術,術後未髮生植皮壞死及感染,無病例最終分離5箇手指,拇指橈偏畸形和指間關節僵硬均無進一步矯正.術後短期隨訪,手部外觀和功能得以改善,傢屬對術後外觀及功能改善滿意,但長期結果有待進一步隨訪.結論 Apert綜閤徵手併指嚴重,需要採取多次複雜的外科手術治療.術後手部外觀和功能得以改善.
목적 탐토Apert종합정적림상표현、진단급치료.방법 제1차병지분리응재환인9개월시개시,수후적치료최호능재5세전완성.분리수지종변연적수지개시,재행중간수지.1세이하환인채용쌍측동시수술.수선중건제1지복,수후재동시분리제2화제4지복.채용“Z”형절구분지,지간배측구형피판중건지복,유리전후피편복개피부결손.결과 2003년지2012년간5례환자진단위Apert종합정,기중2례진단후즉행병지분리수술,술후미발생식피배사급감염,무병례최종분리5개수지,무지뇨편기형화지간관절강경균무진일보교정.술후단기수방,수부외관화공능득이개선,가속대술후외관급공능개선만의,단장기결과유대진일보수방.결론 Apert종합정수병지엄중,수요채취다차복잡적외과수술치료.술후수부외관화공능득이개선.
Objective To discuss the clinical manifestation,diagnosis and treatment of Apert syndrome.Methods Separation of the fingers should start at 9 months of age and completed before 5 years old.Border digits were separated first,while the middle digits separated later.A bilateral approach was adopted in patients under 1 year old.The first web was reconstructed first,followed by the second and fourth webs that were done simultaneously.Z shape incision was made to separate the digits while a dorsal rectangular flap was adopted for reconstruction of the web.Skin defects were covered by fill thickness skin graft.Results Between 2003 and 2012,five Apert syndrome children were treated.Two of them underwent syndactyly separation as soon as being diagnosed.No skin graft necrosis or infection occurred after the operation.None of the 5 cases achieved separation of all 5 digits.The parents were satisfied with both esthetic and functional outcome of the surgery.Radial clinodactyly of the thumb and stiffness of the interphalangeal joint were not further corrected.Short-term follow-up revealed aesthetic and functional improvement of the hand.The long-term outcomes need to be reviewed after longer follow-up.Conclusion Apert hand is severe syndactyly that requires complex surgical management.Aesthetics and function of the hand can be improved after multiple operations.
