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1 J Korean Assoc Maxillofac Plast Reconstr Surg 2012;34(2): pissn: eissn: Review Article 편측구순열비의교정술 : Rotation Advancement 원칙에근거한 Mulliken 의방법 정영수ㆍ이규태ㆍ정휘동ㆍ John B. Mulliken 1 연세대학교치과대학 / 치의학전문대학원구강악안면외과학교실, 구강과학연구소, 1 Department of Plastic and Oral Surgery, Children's Hospital Boston Abstract Repair of Unilateral Cleft Lip and Nose: Mulliken's Modification of Rotation Advancement Young-Soo Jung, Gyu-Tae Lee, Hwi-Dong Jung, John B. Mulliken 1 Department of Oral and Maxillofacial Surgery, Oral Science Research Center, Yonsei University College of Dentistry, 1 Department of Plastic and Oral Surgery, Children's Hospital Boston This is a review regarding Mulliken's Modification using the Millard rotation-advancement principle for the repair of unilateral complete cleft lip and nasal deformity. All patients underwent prior labionasal adhesion and dentofacial orthopedics with a pin-retained (Latham) appliance used for infants with a cleft of the lip and palate. Technical variations concerning the operation are described. A high rotation and releasing incision in the columella lengthens the medial labial element and produces a symmetric prolabium with minimal transgression of the upper philtral column through the advancement flap. The orbicularis oris muscle is everted, from caudad to cephalad, to form the philtral ridge. A minor variation of unilimb Z-plasty is used to level the cleft side of Cupid's bow handle, and cutaneous closure proceeds superiorly from this junction. The dislocated alar cartilage is visualized though a nostril rim incision and suspended to the ipsilateral upper lateral cartilage. Symmetry of the alar base is addressed in three dimensions, including maneuvers to position the deviated anterior-caudal septum, configure the sill, and efface the lateral vestibular web. The authors believe the technical refinements described herein contribute favorably to the outcome of repair regarding unilateral cleft lip and nasal distortion. Key words: Unilateral cleft lip, Nasolabial repair, Rotation advancement 원고접수일 2012년 2월 14일, 원고수정일 2012년 3월 20일게재확정일 2012년 3월 20일책임저자정영수 ( ) 서울시서대문구연세로 50, 연세대학교치과대학구강악안면외과학교실, 구강과학연구소 Tel: , Fax: , ysjoms@yuhs.ac 133 RECEIVED February 14, 2012, REVISED March 20, 2012 ACCEPTED March 20, 2012 Correspondence to Young-Soo Jung Department of Oral and Maxillofacial Surgery, Oral Science Research Center, Yonsei University College of Dentistry 50, Yonsei-ro, Seodaemun-gu, Seoul , Korea Tel: , Fax: , ysjoms@yuhs.ac CC This is an open access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( by-nc/3.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

134 정영수 : 편측구순열비의 Mulliken 방법교정술 서론 최고로인정받는구순열수술외과의사중하나인 Children's Hospital Boston의 Dr. Mulliken은외과의사의성숙도는원칙 (principle) 과기법 (technique) 을구분할수있는가에달렸다고하였다.

2 134 정영수 : 편측구순열비의 Mulliken 방법교정술 서론 최고로인정받는구순열수술외과의사중하나인 Children's Hospital Boston의 Dr. Mulliken은외과의사의성숙도는원칙 (principle) 과기법 (technique) 을구분할수있는가에달렸다고하였다. 수술의원칙은느리게개발되고확고한영구성을얻게되며주기적으로평가가필요하다하였고, 수술기법은빠르게드러나고자주변화되면서지속적인변형이필요하다하였다 [1]. Dr. Millard가편측성구순열수술에대한자신의방법을 1955 년에처음발표한이래, 다른어떤수술기법도이회전-신전법 (rotation advancement) 만큼인기를얻은적이없다. 이방법의요소들은이제편측성구순열수술의원칙으로확립되었으며, 50년이상을지나면서지속적으로기법이진화하고있다. 이전의다른수술법과달리이수술법은처음발표당시부터수술적해부학적목적을유지하면서다양한기법상변화를구사하여각각의파열부위요소들을외과의가다룰수있게하였다. 비록이기법은전세계적으로적용되고있지만성공적인실행은다양하고술자에크게의존된다. Millard 자신뿐만아니라많은다른외과의사들이각환자에맞게기법을조정하고, 몇가지잘못된점을수정하며, 새로운장점을얻도록기법상의변화를발표해오고있다 [2]. 1977년 Millard가 Children's Hospital Boston에방문교수로와있는동안 Mulliken은그의수술과강의에서영향을받아다년간편측구순열수술법과수술후문제점에대한문헌고찰과분석을통해회전-신전원칙을따르는몇가지기법상개발을해오고있다. 그는편측구순열수술목적을어느쪽이수술한쪽인지정상인입술과구분할수없게하는것이라고말할만큼열정적으로환자를치료하고있는데, 이번글에서는그기법들과배경들을소개하고자한다. 편측성구순열비기형의치료순서 (protocol for repair of unilateral cleft lip and nasal deformity) 아래에소개하는 Mulliken 의편측구순열치료순서는 definitive repair 시개열사이간극 (cleft gap) 을줄이고개열부위피부외측과내측의높이를맞추기위한준비과정이중요하게포함된다. 여기서는편측성완전구순열을기준으로설명하며, 불완전구순열인경우는개열사이간극과가용조직의양, 코변형의정도에따라일부과정을생략하기도한다. 서사용하고있는장치는 Millard와 Latham[3] 이함께완성한능동적장치를사용하고있다 (Fig. 1). 이장치는전신마취하에양측상악분절에핀으로고정한후집에서보호자가환아구강내장치의나사를돌리면양측상악분절의간극을좁혀수술하기좋은위치로이동시킬수있다 [4]. 2. 구순접합술과치은골막성형술 (labionasal adhesion and gingivoperioseoplasty [GPP]) 생후 3 4개월 Latham 장치를제거하면서시행한다. 이는 definitive repair 시 tension을줄이고구륜근피판의부피와내측입술의길이를증가시키며코의 lower lateral cartilage 위치를두번교정할기회를부여한다 [1]. 3. Rotation advancement and nasal repair (definitive repair) Labionasal adhesion 후 2개월내에는시행하지않는다. 즉, 생후 5 6개월에 definitive repair을시행한다. 이차구개 (secondary palate) 에파열이없으면서개열간극이넓지않은경우는술전악정형장치는생략하고생후 1개월에 labionasal adhesion을시행하고생후 3 4개월에 definitive repair을시행한다. 구순접합술과치은골막성형술 (labionasal adhesion and GPP) 1. Marking 개열부내측부위의정상적인 labial landmark는그대로두고 vermillion-cutaneous junction에서점막쪽 1 2 mm에 6 8 mm 선을그린다. 상응되는선을외측입술부분에그린다. 다른절개는 vestibular-piriform aperture 를따라표시하고외측 sul- 1. 술전악정형장치 (preoperative dentofacial orthopedics) 생후약 1개월에시작하여상악분절이수술하기에좋은정도로모아질때까지약 2개월정도진행한다. Mulliken 이실제임상에 Fig. 1. The Latham pin-retained presurgical orthopedic appliance. J Korean Assoc Maxillofac Plast Reconstr Surg

3 Young-Soo Jung: Repair of Unilateral Cleft Lip and Nose: Mulliken's Modification of Rotation Advancement 135 cus 안까지확장한다. 이선은외측입술부분절개를받침으로하는역 "T" 모양의덮개를형성한다 (Fig. 2). 2. Dissection 순측절개는근육을충분히노출시키기위해점막하조직 (submucosa) 을통해시행하는데, 구륜근 (orbicularis oris) 을박리하지않는다. 외측 sulcus 안절개선을통해외측입술부분을골막상방 (supraperiosteal plane) 에서박리하여상악으로부터들어올린다. 이때외측입술이내측전위시장력이없도록박리를확장한다. 점막 back-cut 은외측입술이전진되는것을도와준다 (Fig. 2). 가위를 medial crura 사이로넣어비첨 (nasal tip) 을넘어 cartilaginous dome 위로 supra-alar pocket 을형성하도록박리한다. 이박리를충분하게시행해야외측과내측입술부분이잘모이게되고, 콧방울만곡아치 (alar genu arch) 가타원형의콧구멍가장자리 (nostril rim) 를형성할수있도록한다. 3. Closure 악정형장치가성공적이면치은골막폐쇄 (gingivoperiosteal closure) 는가능하다. 외측입술부분을전방열구를따라전진시키고후방점막층을봉합한다. 서너개의 5-0 polydioxanone으로내외측근육을모아주는데, tied in series 로시행한다. 전방의 vermillion-mucosal 가장자리들은얇은흡수성실을이용하여외번되도록봉합한다. 만약콧구멍가장자리가꺼진다면콧방울연골 (alar cartilage) 을동측 upper lateral cartilage 에매달기위해동측코뼈 (nasal bone) 위로아주작게절개하여 polyglatin 봉합이묻히도록위치시킨다 (Fig. 2). 만약콧구멍가장자리가타원형이라면매달기매복봉합은불필요하다. 유아는절대적으로비호흡 ( 약생후 3개월까지 ) 을하기때문에기도확보 "stent" (Xeroform strip을 19-gauge polyethylene catheter 주위를감싼다.) 를이환측콧구멍에삽입한다. 이 "stent" 는일시적으로콧방울연골을지지하고전정부종을최소화시키며부착된부위를코분비물로부터보호한다. 이것은수술 48시간후에제거한다. Rotation advancement 원칙에근거한 Mulliken 의편측구순열비의교정술 (Mulliken's modification of rotation advancement and nasal repair) 1. Marking (Fig. 3) 1) Rotation incision Columella base로확장되는과도한곡선으로 rotation incision을설정하여내측입술부위에충분한길이를부여한다. 이절개선의모양이나길이가반대편인중선 (philtral ridge) 과맞게할필요는없다. Fig. 2. Labionasal adhesion. (Left) Adhesion incisions. Note back-cut in buccal sulcus. (Right) Suspension of slumped alar genu to ipsilateral above lateral cartilage (with permission from Mulliken JB, Martínez- Pérez D., 1999.). Vol. 34 No. 2, March 2012

4 136 정영수 : 편측구순열비의 Mulliken 방법교정술 Fig. 3. Markings for rotation-advancement repair. Rotation incision bows and extends in columellar base. Lateral triangular vermilion flap drawn to correct medial vermilion deficit (with permission from Mulliken JB, Martínez-Pérez D., 1999.). Fig. 4. Dissection, exposure, and midline translocation of anterior-caudal septum (with permission from Mulliken JB, Martínez-Pérez D., 1999.). 2) Advancement flap Alar base 아래로설정하는데, 외측으로넘어가도록확장하지는않는다. 이는이부위에서좋지않은반흔을피하기위해서이다. 3) Lateral triangular vermilion flap 이는내측입술의부족한 vermilion을보충하기위해설계한다 [5]. 2. Dissection 1) Medial crura 외측전정절개 (lateral vestibular incision) 를 alar-piriform junction 까지시행하고, membranous septal incision 은전정의내측까지시행한다. 이절개선을통해가위를이용하여 medial crura 를분리하고 nasal dome 위의피부를연골조직에서박리한다. 이때 lateral crus 위의피부와콧방울연골 (alar cartilage) 의안쪽은박리하지않는다. 2) Nasal septum C-flap을들어올리면서, septum의 anteriocaudal margin 부위의 mucopericondrium을절개하고비이환측으로변위된 septum을자유롭게하여 nasal spine의이환측골막에옮겨매달아코대칭성획득에도움이되게한다. Caudal septum이변위된채로있으면 columellar base는비이환부위로이동되고비정상적인수직적축과함께좁은 " 정상 " 콧구멍을야기한다. 이런전방중격의일차적위치수정은 columella 를바로세우고비이환측콧구멍의기형을수정하며파열측 alar base의균형적 Fig. 5. Hemicolumellar lengthening. (Left) Releasing incision in columellar base. (Above, right) Retrogression of C-flap to elongate hemicolumella. (Below, right) C-flap apposed to tiny opening in medial columella (with permission from Mulliken JB, Martínez- Pérez D., 1999.). 위치를확보하기위한근거를제공한다 (Fig. 4)[6-8]. 3) Back-cut 콧구멍을들어올리고내측입술부분을아래로당기면서 rotation incision 의최상방점에서 columella 피부에아주작은절개선을넣어장력을줄인다. 이는 Millard의 back-cut과수직인 "releasing incision" 개념을사용한것이다 (Fig. 5). 세가지 J Korean Assoc Maxillofac Plast Reconstr Surg

Young-Soo Jung: Repair of Unilateral Cleft Lip and Nose: Mulliken's Modification of Rotation Advancement 137 장점이있는데, 첫째 rotation incision 이중간을가로지르지않는다.

5 Young-Soo Jung: Repair of Unilateral Cleft Lip and Nose: Mulliken's Modification of Rotation Advancement 137 장점이있는데, 첫째 rotation incision 이중간을가로지르지않는다. 둘째윗입술에서개방이작아지고 C-flap이 hemicolumella 를연장시키기위해사용되도록한다. 셋째는 advancement flap 의첨부가 philtral column을가로지르고없애지않는다 [2]. 3. Closure 1) C-flap Releasing incision으로만들어진간극에 C-flap을돌리는것이아니라, columella와 membranous septum의 released medial edge에옆으로돌려 (side-to-side) 봉합한다 (Fig. 5). 즉, flap을 back-cut 부위로회전시키는것대신 hemicolumella 를역으로올린다. 이것은내측콧구멍 sill 이형성되는동안 columella를좀더안정적으로증가시키고자연스러운대칭을만들어준다. 2) Gingivolabial sulcus 정중부 labial frenum에서 releasing incision을가하여전진되는외측점막을넣을수있는 notch 를형성한후 gingivolabial sulcus 를봉합한다. 이러한점막부의 interdigitation 은피부에서 rotational gap으로들어오는 advancement flap의첨부와부합된다. 즉, 외측입술부위와점막이같은방향에서전진하게된다. 이것은 Mulliken 방법에서말하는세개의 unilimb Z-plasty 의첫봉합이자가장안쪽에위치된다. 이런내측점막면의 release 는입술이외번되게하고, 구강전정의내측면을길게하는효과가있다 [9]. 3) Muscle (functional closure) 개열의양쪽에서피부와점막으로부터근육다발을충분히박리한후하방깊은부위의 pars marginalis 부터 vertical mattress 봉합법으로하방에서상방으로진행하여 pars peripheralis가모아져서솟아오르게 (everting) 하여 philtral ridge가형성되게한다 (Fig. 6). 입술이앞으로기울게하고 infra-sill 부위에서생길수있는함몰이수정되도록상방근육을특히잘모아야한다. 마지막 suture는 nsalis와 depressor septi 근육을포함하는구륜근의상방가장자리를 anterior nasal spine 위의골막에위치시킨다 [10]. 상승을방지한다 ( 이왜곡은 levator labii superioris alaeque nasi 의반대적힘을받지않는것에의해야기된다. 즉, depressor alae nasi (musculus nasalis, pars alaris) 에의해아래방향으로당기는것이없기때문이다 ) [1]. 5) Nasal repair 콧구멍위쪽접힌피부를반달모양으로절제 (semilunar cutaneous excision) 한후이절개선을통해주저앉은 alar cartilage 를노출시킨다 (Fig. 7). Supraalar dissection이완성되면코의피부는연골조직에서분리된다. Alar cartilage를직접보면서동측의 upper lateral cartilage에매달아준다. Middle crura는 interdomal suture로반대편에묶어준다. Alar base 를닫고 cartilage 를매달고나면콧속외측전정부에물갈퀴 (web) 모양의주름이형성된다. 이것을 intercartilaginous line의가장자리에서반달형으로절제한후 suture하면, 함입된 lateral crus를올려주고외측전정부를확장시킨다 (Fig. 7). 6) Cutaneous closure 대칭적인 Cupid's bow를형성하기위해 vermilion-cutaneous junction에작은 unilimb Z-plasty를시행한다. 정상측 Cupid's bow handle의높이에맞게내측 vermilion-cutaneous junction에 releasing incision을주고이사이에들어갈삼각형피판을외측 white roll에만들어준다. 이과정에서 advancement flap의첨부와 rotation flap의가장자리를다듬어주어 philtral ridge가정상측과대칭이되도록조정한다 (Fig. 8). 또하나의 unilimb Z-plasty 는처음디자인한 lateral triangu- 4) Alar base 수직적수평적위치 (x-, y-axis) 외에콧속방향으로 (z-axis) 약간돌리는것이필요하다. Alar base flap의 tip과 C-flap의 tip을다듬어만나게하여 sill을형성한다 (Fig. 7). 그리고, alar base 깊은부위에 suture 를아래의근육이나골막에매달아줌으로써 sill에정상반곡선이형성되게하고, 콧속방향으로돌린 flap이외측으로뒤틀리는것을최소화하고, 웃을때 alar nasi 의 Fig. 6. Closure orbicularis oris muscle. Pars marginalis repaired first. Vertical mattress sutures "reef" muscular join to form philtral ridge (with permission from Mulliken JB, Martínez-Pérez D., 1999.). Vol. 34 No. 2, March 2012

138 정영수 : 편측구순열비의 Mulliken 방법교정술 Fig. 7. Symmetric positioning of alar base and formation of sill. (Left) Alar base flap trimmed and positioned in three dimensions; note minor rotation along z axis.

6 138 정영수 : 편측구순열비의 Mulliken 방법교정술 Fig. 7. Symmetric positioning of alar base and formation of sill. (Left) Alar base flap trimmed and positioned in three dimensions; note minor rotation along z axis. Suture secures base to muscle and forms normal depression of lateral sill. (Center, above) Cutaneous overhang in superior nostril rim excised as a crescent. (Right) Genu (junction middle and lateral crus) elevated and secured to ipsilateral upper lateral cartilage. (Center, below) Lenticular excision of web elevates encroaching lateral crus and effaces vestibular ridge (with permission from Mulliken JB, Martínez-Pérez D., 1999.). Fig. 8. Adjustment of Cupid's bow handle. Incision at medial vermilion-cutaneous junction lowers bow peak; resulting triangular mortise is filled with lateral white roll-cutaneous tenon (usually 1.5 to 2 mm at base). Note medial vermilion-mucosal releasing incision for inset of lateral vermilion flap (with permission from Mulliken JB, Martínez-Pérez D., 1999.). Fig. 9. Philtral closure. Eccentric placement of intradermal sutures (higher on medial edge) lowers philtrum. Note cymal excision of superior margin of advancement flap at join of constructed sill, contingent on height of lateral lip, measured from alar base to Cupid's bow peak (with permission from Mulliken JB, Martínez-Pérez D., 1999.). lar vermilion flap으로내측 vermilion-mucosal junction에 releasing incision을주어생긴 notch에전진되어들어가도록하여 median tubercle의부족분을보충하게한다 [2]. Philtral closure는아래에서부터위로 suture를시행하는데 intradermal suture는 rotation 측이높게해주어 philtrum을낮게한다 (Fig. 9). 흔하지는않지만 advance flap의위쪽 margin을다듬어야할때에는 alar base 위치에맞추어반곡선형으로잘라낸다 (Fig. 9). 봉합은 alar groove, sill 아래의주름, phitral column의두드러짐을강조하기위해외측에서내측으로 시행하고, 마지막에 advancement flap 의끝을다듬어장력없이들어가도록한다. 7) Internal resorbable splint 1997년부터골절편고정에쓰이는흡수성판을이환측코내부에 splint 로삽입하고있다. 약 15 mm 길이로잘라정상측 nostril dome 모양으로구부려기존의코연골재위치를위해형성했던절개선안으로넣어 cartilage 위에위치하도록한다. 흡수성이므로나중에제거하지않는다 (Fig. 10)[11]. J Korean Assoc Maxillofac Plast Reconstr Surg

Young-Soo Jung: Repair of Unilateral Cleft Lip and Nose: Mulliken's Modification of Rotation Advancement 139 이번에소개한 Mulliken 의치료법이환자들과외과의사들에게많은도움이되기를바란다. References Fig. 10.

7 Young-Soo Jung: Repair of Unilateral Cleft Lip and Nose: Mulliken's Modification of Rotation Advancement 139 이번에소개한 Mulliken 의치료법이환자들과외과의사들에게많은도움이되기를바란다. References Fig. 10. Illustration showing splint in subcutaneous pocket overlying suspended alar cartilage (with permission from Wong GB, Burvin R, Mulliken JB., 2002.). 요약 (summary) 모든환자들은구순접합술을시행받았고구순및구개열유아들은악정형장치인 Latham을사용하였다. 수술의기술적변화들은앞서설명하였다. Columella 부위의높은 rotation 과 releasing incision은내측입술부위를충분히길게해주고, advancement flap이 phitral column 상방으로최소로침범되게하여균형적인입술을만들수있다. 또한구륜근을외번시켜 philtral ridge 를형성하고, 작은 unilimb Z-plasty 을구순측 Cupid's bow handle 높이에맞게시행후, vermilion-cutaneous junction에서부터상방으로 cutaneous closure 시행한다. 변위된 alar cartilage는 nostril rim incision을통해동측 upper lateral cartilage에매달며, Alar base는 anterior-caudal septum의위치, sill의설정그리고외측 vestibular web 제거를포함하여 3차원적으로설계하여치료해야한다. 1. Mulliken JB, Martínez-Pérez D. The principle of rotation advancement for repair of unilateral complete cleft lip and nasal deformity: technical variations and analysis of results. Plast Reconstr Surg 1999;104: Stal S, Brown RH, Higuera S, et al. Fifty years of the Millard rotation-advancement: looking back and moving forward. Plast Reconstr Surg 2009;123: Millard DR Jr, Latham RA. Improved primary surgical and dental treatment of clefts. Plast Reconstr Surg 1990;86: Chan KT, Hayes C, Shusterman S, Mulliken JB, Will LA. The effects of active infant orthopedics on occlusal relationships in unilateral complete cleft lip and palate. Cleft Palate Craniofac J 2003;40: Noordhoff MS. Reconstruction of vermilion in unilateral and bilateral cleft lips. Plast Reconstr Surg 1984;73: Tajima S. The importance of the musculus nasalis and the use of the cleft margin flap in the repair of complete unilateral cleft lip. J Maxillofac Surg 1983;11: Anderl H. Simultaneous repair of lip and nose in the unilateral cleft (a long term report). In: Jackson IT, Sommerlad BC, editors. Recent advances in plastic surgery. Vol. 3. Edinburgh: Churchill Livingstone; p Matsuo K, Hirose T, Otagiri T, Norose N. Repair of cleft lip with nonsurgical correction of nasal deformity in the early neonatal period. Plast Reconstr Surg 1989;83: Koch J. The reconstruction of the vestibule of the mouth in cleft lip and palate surgery. Dtsch Stomatol 1970;20: Delaire J. Theoretical principles and technique of functional closure of the lip and nasal aperture. J Maxillofac Surg 1978;6: Wong GB, Burvin R, Mulliken JB. Resorbable internal splint: an adjunct to primary correction of unilateral cleft lip-nasal deformity. Plast Reconstr Surg 2002;110: Vol. 34 No. 2, March 2012

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untitled J Korean Assoc Maxillofac Plast Reconstr Surg 2013;35(3):178-183 pissn:1225-4207 eissn: 2233-7296 Case Report 편측성불완전소구순열의치료 박혜정ㆍ정휘동ㆍ John Butler Mulliken 1 ㆍ정영수 연세대학교치과대학구강악안면외과학교실, 1 Department of Plastic