CAJ | 학술논문

目的探讨和分析Apert综合征手足畸形的形态学特点及治疗原则.方法回顾和总结2007年5月至2012年8月期间诊疗的12例Apert综合征患者,在Upton分型基础上,进一步根据指(趾)并连的范围和严重程度,将手部畸形分为Ⅰ、Ⅱ、Ⅲ、Ⅳ、Ⅴ型,足部畸形分为Ⅰ、Ⅱ型.依据分型确定手术治疗原则,Ⅰ型手部畸形行单次分指手术,Ⅱ～Ⅴ型依据多指并指分指原则行分期手术.拇指偏斜行近节指骨截骨术,Ⅰ型、Ⅱ型及Ⅲ型截骨术及分指术同时进行,Ⅳ型、Ⅴ型截骨术及分指术分期进行;足趾畸形分期行分趾手术.4例手部畸形患者接受全部手指分指及拇指近节指骨截骨手术治疗,6例手部畸形患者仍在治疗过程中,2例未治疗.2例足部畸形患者接受足趾分期分趾手术.结果所有分指(趾)手术均成功,无指(趾)坏死发生.术后发生指蹼瘢痕粘连3指(趾),瘢痕挛缩引起手指掌侧屈曲畸形4指.4例完成分指和截骨手术治疗的患者,术后患手可完成基本的抓、握、捏功能,2例完成足趾分趾手术的患者手术后足功能无明显改进.结论 Apert综合征是一种涉及手足严重形态学畸形的多器官畸形综合征,通过分指及指骨截骨手术治疗,在一定程度上改善了手功能和外形,仍残留其他畸形和功能障碍,需进一步研究和治疗.
목적 탐토화분석Apert종합정수족기형적형태학특점급치료원칙.방법 회고화총결2007년5월지2012년8월기간진료적12례Apert종합정환자,재Upton분형기출상,진일보근거지(지)병련적범위화엄중정도,장수부기형분위Ⅰ、Ⅱ、Ⅲ、Ⅳ、Ⅴ형,족부기형분위Ⅰ、Ⅱ형.의거분형학정수술치료원칙,Ⅰ형수부기형행단차분지수술,Ⅱ～Ⅴ형의거다지병지분지원칙행분기수술.무지편사행근절지골절골술,Ⅰ형、Ⅱ형급Ⅲ형절골술급분지술동시진행,Ⅳ형、Ⅴ형절골술급분지술분기진행;족지기형분기행분지수술.4례수부기형환자접수전부수지분지급무지근절지골절골수술치료,6례수부기형환자잉재치료과정중,2례미치료.2례족부기형환자접수족지분기분지수술.결과 소유분지(지)수술균성공,무지(지)배사발생.술후발생지복반흔점련3지(지),반흔련축인기수지장측굴곡기형4지.4례완성분지화절골수술치료적환자,술후환수가완성기본적조、악、날공능,2례완성족지분지수술적환자수술후족공능무명현개진.결론 Apert종합정시일충섭급수족엄중형태학기형적다기관기형종합정,통과분지급지골절골수술치료,재일정정도상개선료수공능화외형,잉잔류기타기형화공능장애,수진일보연구화치료.
Objective To analyze the morphologic characteristic of hand and foot deformities in Apert syndrome and explore the treatment strategy.Methods Twelve cases with Apert syndrome diagnosed and treated from May 2007 to August 2012 were retrospectively reviewed.Based on the severity and extent of finger (toe) syndactyly,the authors extended Upton's classification.Deformities of the hand were classified into type Ⅰ,type Ⅱ,type Ⅲ,type Ⅳ and type Ⅴ,while those of the foot into type Ⅰ and type Ⅱ.Under the guidance of the new classification,separation of the digits were performed by one-stage procedure in type Ⅰ and by multi-stage procedures in type Ⅱ,type Ⅲ,type Ⅳ and type Ⅴ.Deviated thumbs were corrected by proximal phalangeal osteotomies.Digit separation and osteotomy were done simultaneously in type Ⅰ,Ⅱ and Ⅲ cases.For type Ⅳ and Ⅴ cases,osteotomy and digit separation were done in two stages.Complete separation of the digits and proximal phalangeal osteotomy of the thumb were accomplished in 4 patients of hand deformities.Six patients are still in the different stages of surgical intervention,while 2 patients remain untreated.Complete separation of the toes was accomplished in 2 patients with foot deformities.Results All separations of the digits and toes were successful without any digit (toe) necrosis.Web adhesion occurred in 3 cases.Flexion contracture of the fingers due to scar contraction occurred in 4 fingers.The 4 patients who had completed digit separation and osteotomy were able to perform basic pinch and grasp.Function of the feet was not significantly improved.Conclusion Apert syndrome is one of the most severe deformity complex that involves the hands and feet.Surgical intervention,digit separation and osteotomy,could provide aesthetic and functional improvement of the hand.The residual anomalies still need further study and treatment to gain more aesthetic and functional improvement.