xRead - Treatment of Cleft Lip and Cleft Palate (May 2025)

Facial Plastic Surgery & Aesthetic Medicine Volume 24, Number 5, 2022 ª American Academy of Facial Plastic and Reconstructive Surgery, Inc. DOI: 10.1089/fpsam.2021.0166

ORIGINAL INVESTIGATION

Craniofacial/Pediatric

Modified Rotational Advancement Technique for Primary Unilateral Complete Cleft Lip Repair Khaled Alkebsi, MSc, 1,2 Chenghao Li, PhD, 1 Bing Shi, PhD, 1, * Bassam Mutahar Abotaleb, PhD, 1,2 Wael A. Telha, MSc, 1 Karim Ahmed Sakran, MSc, 1,2 and Yang Li, PhD 1, *

Abstract Importance: Leveling Cupid’s bow while maintaining lip height and width in unilateral complete cleft lip patients with improved nose outcomes is a significant challenge for surgeons. Objective: This study aimed to measure the symmetry of the nasal and labial anthropometric points after using this modified cleft lip repair technique. Design, Setting, and Participants: In this retrospective study, 72 consecutive nonsyndromic unilateral com plete cleft lip patients underwent a modified rotational advancement technique. Photos of at least 1 year follow-up period were collected. Main Outcomes and Measures: The nose measurements included columella length, nostril height and width, ala width, nostril inclination, columella angle, and ala bases angle. The lip measurements included lip height and width, vermillion height; midline-philtrum angle, and angles of Cupid’s bow peaks. Descrip tive quantitative analysis of the lip and nose measurements, intraclass correlation coefficient, and ANOVA tests were conducted. Results: Well-aligned Cupid’s bows with symmetrical lip height and width were obtained with satisfactory aesthetic nasal outcomes. Conclusions: The present technique had leveled the Cupid’s bow while preserving the lip height and width, with resultant satisfactory nasal outcomes.

Introduction Several different techniques have appeared for cleft lip repair after the introduction of the straight-line closure of cleft lip repair in the 1840s. 1 The rotational advance ment (RA) technique has become the most popular method for cleft lip repair since Millard first described it in 1957. 2 So far, the majority of surgeons continue to use this technique in the treatment of cleft lip, 3 with * 84% of plastic surgeons performing modifications to the Millard technique. 3,4 The RA technique may result in deficient transverse width of the lateral lip owing to concern for vertical lip height at the expense of horizontal lip length. Therefore, the surgeons might use the inferiorly placed triangular flap. 5–7 Downloaded by Travis T Tollefson from www.liebertpub.com at 03/02/25. For personal use only.

Rotating the Cupid’s bow to the optimal position while maintaining symmetrical horizontal lip width on both cleft and noncleft sides is challenging for surgeons during cleft repair due to the lack of a model, which helps predict the postoperative outcomes. Treatment of the cleft lip requires a deep understanding of the anatomical and aes thetic features as well as geometrical design of any cleft lip repair to facilitate cleft lip treatment and give the sur geons the ability to predict postoperative outcomes. The outcome of cleft lip repair should be based on aesthetic improvement of the lip and nose. Although pre vious cleft lip repair techniques have achieved consider able progress in lip repair, the same improvement was not accomplished on the nose part of the procedure. 7 Draw ing upon many years of our institute’s experience, this

1 State Key Laboratory of Oral Diseases and National Clinical Research Center for Oral Diseases and Department of Oral and Maxillofacial Surgery, West China Hospital of Stomatology, Sichuan University, Chengdu, China. 2 Department of Oral and Maxillofacial Surgery, College of Dentistry, Ibb University, Ibb, Yemen. *Address correspondence to: Yang Li, PhD, Department of Oral and Maxillofacial Surgery, West China Hospital of Stomatology, Sichuan University No. 14, Section 3 of Renmin Nan Road, Chengdu 610041, China, Email: luciaya@163.com; Bing Shi, PhD, Department of Oral and Maxillofacial Surgery, West China Hospital of Stomatology, Sichuan University No. 14, Section 3 of Renmin Nan Road, Chengdu 610041, China, Email: shibingcn@vip.sina.com

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KEYPOINTS Question: Can new modifications in unilateral complete cleft lip repair improve the leveling of Cupid’s bow, short lip height and width, and short columella? Findings: Well-aligned Cupid’s bow with symmetrical lip height and width were obtained with satisfactory aesthetic nasal outcomes. Meaning: The new modifications to primary unilateral com plete cleft lip repair could improve the lip and nasal aesthetic results.

Fig. 1. (Left) important reference points in the SM. (Center) The shape and direction of the flaps after incision. Note the leveling of the Cupid’s bow to symmetrical position. (Right) postoperative scar.

study introduces a unique method including a geometri cal theory of Cupid’s bow rotation as described by Shi 8 and new modification in C-flap and superiorly based flap treatment to improve the lip and nose alignment and outcomes in unilateral complete cleft lip (UCCL) re pair. We performed postoperative anthropometric analy ses of lip and nose symmetry by labeling the points and measuring standard distances for evaluation of the Shi modifications (SM). This study was approved by the institutional review board of West China Stomatology Hospital of Sichuan Univer sity. This study included 72 consecutive patients with non syndromic unilateral complete cleft lip and palate between 2015 and 2019 who underwent cleft lip repair by SM. Data were derived from the hospital electronic medical record system, with the following data retrospectively collected: age at the time of operation, gender, cleft side, and follow-up. All surgeries were performed using SM without presurgical nasoalveolar molding. The last follow-up period’s photos were collected before any re vision intervention (follow-up ranged from 1 to 5 years). Surgical technique This modified RA passed through two stages until it reached its final form, as described in this study. In 2009, Shi introduced the bisector theory. 8 In this study, Shi added the superiorly based advanced flap on the cleft side, which helps increase the vertical lip height and fills the space created by the back-cut incision. Meanwhile, the C-flap is rotated to form the nasal sill and part of the columella on the cleft side (see Supplementary Video S1). The anthropometric points on the noncleft side are al most the same as that for the Millard technique (Fig. 1; left). The crucial difference between RA and SM in marking the noncleft side is the placement of the incision end. Mark point 1 at the low peak of Cupid’s bow. Points 2 and 3 are marked equally on both lateral peaks of Cupid’s bow. Points 11 and 12 are marked at the mouth’s commissures on the noncleft and cleft sides, respectively. Downloaded by Travis T Tollefson from www.liebertpub.com at 03/02/25. For personal use only. Materials and Methods Patients

Point 4 is marked where 2-11 = 4-12. In the presence of a high discrepancy in the horizontal lip width between cleft and noncleft sides, we maintain horizontal symmetry by marking point 4 as medially as possible, resulting in a short vertical lip, which can be corrected by an advanced flap on the cleft side, allowing the downward rotation of point 4. Mark point 5 on the middle of the columella base. Mark point 6 on the line bisecting a 2-1-3 angle, where 1-2 = 1-3, 2-6 = 3-6. Since anywhere on the bisec tor will result in 2-6 = 3-6, point 6 can be localized any where on the bisector. In Millard’s technique, point 6 is localized superiorly and medially to the imaginary bisec tor line. 9 Ending the back-cut incision on the angle bisec tor will ensure the horizontal rotation of point 3 to be at the same horizontal level as point 2. Point 7 is marked at the columella level on the cutaneous-mucosal border to form the base of the C flap. Point 8 is marked at the ala level on the cutaneous mucosal border. Point 9 is located in the ala’s base on the cleft side so that 3-5 = 4-9, such that points 9 and 8 will form the base of the advanced flap. Point 10 is located in the ala’s base on the noncleft side. Point 13 is located where 8-9-13-4 = 10-2. Points 3-5-6 and 3-7 are connec ted to form the incision line on the noncleft side, and points 4-9 and 4-8 are connected to form the incision line on the cleft side. In our technique, the skin, muscle, and mucosa are incised separately at different levels. The skin is incised along with points 3-5-6, 3-7, 4-9, and 4-8 (Fig. 1; center). Then the skin is dissected and separated from the under lying orbicularis oris muscle on both sides. After that, the orbicularis oris muscle is dissected from the oral mucosa and horizontally on both sides paying more attention to the dissection of the muscle from the nasal spine to allow greater relief of the deviation of the columella and more controlled rotation of the muscle downward. The muscles are dissected from their abnormal attach ment to the maxilla on the cleft side. The incision on the vermillion is conducted as per Millard’s technique using Noordhoof’s modification. A horizontal incision on the oral mucosa from the vermillion level to the bisec tor line is needed if mucosa restricts the rotation.

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The muscle at the alar base on the cleft side is sutured with the muscle at the columella base to correct the nasal floor width, location of the nose base, and the columellar deviation. Then the whole muscle on both sides is sutured from top to bottom. In the Millard technique, the C-flap is rotated locally to fill the space created by the back-cut or the space made in the cleft side, whereas in the SM, the C-flap is rotated laterally to the nasal cavity to form the lateral columella wall and part of the nasal floor, resulting in increased columella length. On the cleft side, the ad vanced flap is rotated upward and medially and inserted to fill the back-cut space. The advanced flap on the cleft side also helps rotate point 4 to its natural position so that the lip height on the cleft side is lengthened while not sacrificing lip width. Then the skin at point 5 is subcutaneously sutured with the alar base at point 9 and the skin at points 3 to 4. Finally, the skin is com pletely sutured (Fig. 1; right). The nasal deformity is addressed by a Tajima incision on the ala of the cleft side. The subcutaneous dissection is performed along with the lower lateral cartilage to the lower edge of the nasal bone. To correct the collapsed nasal alar shape on the cleft side, the lower lateral carti lage is sutured reliably by a nonabsorbable suture with the ipsilateral upper lateral cartilage and with the contra lateral lower lateral cartilage. Measurement and analysis Frontal and basal views of standardized digital photo graphs taken by a specialist were imported into Adobe Photoshop CS6 software for measurements. 10–12 Then pixels ratios were used instead of the absolute distances to overcome the variations among the individual photo graphs. The lip and nose symmetry was assessed as ratios between the noncleft and cleft sides, considering a value close to 1 as symmetrical outcomes. The nose measure ments included five ratios and two angles (Fig. 2), whereas the lip measurements included five ratios and two angles (Fig. 3). Descriptive quantitative analysis of the lip and nose measurements, intraclass correlation coefficient (ICC) tests, and ANOVA were conducted using SPSS version 20 (Chicago, IL). Results In 72 patients, 44 males and 28 females (1:1.6) were trea ted by SM. Among them, 69.4% had left-side complete cleft lips and 30.6% right-side complete cleft lips. The age of the participants at the time of operation ranged between 3 months and 2 years (exception of one case aged 27 years) with a mean age of 11.8 – 3.6 months. The mean follow-up time was 1.899 – 1 years (range of 1–5 years). No dehiscence, necrosis, infection, and suture granuloma were observed during recovery time. Well aligned Cupid’s bow of the lip was shown by the cleft and noncleft side ratio of the vertical philtral height Downloaded by Travis T Tollefson from www.liebertpub.com at 03/02/25. For personal use only.

(VPh) 0.93. Considerable symmetry in horizontal lip width was also achieved (CPh-Ch ratio = 0.97). Columel lar height ratio and angle mean were 0.82 – 0.22 and 3.12 – 3.59, respectively. Satisfactory aesthetic lip and nose outcomes and ICC are shown in Table 1. An accept able straight scar resembling a normal philtral column Fig. 2. Nasal measurements (A) . CL; measured from highest to lowest points of the nostril. The measurement is performed at a position of 1 mm from columella on both sides, CA; angle between the long axis of columella and midline, NH; measured from highest to lowest points of the nostril, NW; measured from the widest lateral and medial points of nostril aperture, NA; the angle between vertical and horizontal axis of the nostril, AW; measured from the outmost alar point and columellar base. And (B) shows alar base angle; angle between the alar bases. AW, ala width; CA, columella angle; CL, columella length; NA, nostril angle; NH, nostril height; NW, nostril width; ABA, alar base angle.

Fig. 3. Lip measurement. Ab, alar base; BS, bisector; Ch, chelion; CPh, christa philtrum; HL, horizontal line; MCPh, middle christa philtrum; ML, midline; Sn, subnasal; VH, vermillion height; VPh, vertical pheltrum height; white triangle, angle between Cphs.

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Table 1. The aesthetic results and intraclass correlation coefficient test of the lip and nose Mean – SD ICC

3.89 – 3.86 0.93 – 0.10 0.92 – 0.11 0.87 – 0.10 1.03 – 0.24 0.97 – 0.22 1.28 – 0.36 4.95 – 4.24 0.82 – 0.22

ML-MCPh angle

0.954 0.911 0.957 0.961 0.908 0.932 0.947 0.919 0.957 0.884 0.988 0.913 0.984 0.968 0.965

VPh

Cph-Sn CPh-Ab

CPh-MCPh

CPh-Ch

VH

CPhs angle

CL NH NW AW NA

0.8228 – 0.14 1.28 – 0.21 1.10 – 0.13 0.74 – 0.15 3.12 – 3.59 1.95 – 1.52

CA

ABA

was obtained using SM. Furthermore, the null hypothesis (no difference between the authors’ measurements) was supported by ANOVA results ( p < 0.05). Discussion Significant progress has been made in cleft lip repair over the past century 13,14 : The SM showed satisfactory level ing of the Cupid’s bow and enhanced the lip height with preserving the lip width. Furthermore, the nasal symme try was satisfactory, as shown by the anthropometric measurements (Fig. 4). For many years, the short lip was observed after treat ment of the UCCL patients in our departments. This observation is consistent with what Lazarus et al. repor ted. 15 Other studies reported better lip and nose width with Millard technique as compared with Tennison– Randall technique. 12 Previous studies pointed out the possibility of using the inferiorly placed triangular flap to overcome the short lip height. 5,6 Therefore, our tech nique aimed to preserve the advantages and eliminate disadvantages inherent to Millard technique. In the Millard method, rotation of the Cupid’s bow to the symmetrical position is achieved by performing a back-cut. All points in the design of the Millard method are precisely localized. However, the back-cut incision length is not de termined and, therefore, it is highly dependent on the sur geon’s experience. Using the cut-as-you-go principle in the Millard method may result in an unexpected or unsuc cessful situation 16 and may lead to short lip height. 8,17 Incising the back-cut blindly without standard parameters may lead to either excessive rotation of the Cupid’s bow and thus increased lip height, or may lead to insufficient ro tation of the Cupid’s bow resulting in a short lip. 8 By analyzing the geometric rotation of the Cupid’s bow during cleft lip repair, together with the natural Ab, alar base; ABA, alar base angle; AW, ala width; CA, columellar angle; Ch, chelion; CL, columellar length; CPh, christa philtrum; ICC, intraclass correlation coefficient; MCPh, middle christa philtrum; ML, mid line; NA, nostril angle; NH, nostril height; NW, nostril width nostril width; Sn, subnasal, VH, vermillion height; VPh, vertical pheltrum height. Downloaded by Travis T Tollefson from www.liebertpub.com at 03/02/25. For personal use only.

Fig. 4. (Above) Preoperative views of 1-year old patient with left complete cleft lip. (Below) Postoperative views of 4 years after simultaneous primary repair of cleft lip and nasal deformity by SM showing good overall appearance. SM, Shi modifications.

shape of the upper lips, Shi shows that an incision on the noncleft side ends on a bisector of the angle 2-1-3 (representing the labial midline), ensures sufficient rota tion of the Cupid’s bow to its symmetrical position. 8,18 Bisector theory in cleft lip repair design helps guide clin ical practice and improves the ability to predict the post operative effect. Satisfactory repositioning of the Cupid’s bow was obtained using our method, as shown by the cleft and noncleft side ratio of the VPh 0.93. Marking the CPh in the cleft side could be performed either by mirroring the distance between the CPh commissures on the cleft and noncleft sides marking the point where the white roll disappears, or the vermil lion has the maximum thickness. 19 However, a short ver tical and/or horizontal lip may result despite the method used to mark the CPh on the noncleft side. Previous stud ies have reported that cleft lip surgeons should pay greater attention to lip width than vertical lip height. The reason behind this is the impossibility of correcting the short horizontal lip 20 and the ability to restore the short vertical lip height during revision. In contrast, re garding the aesthetic outcome, Gundlach et al. 21 have emphasized the significance of lip height symmetry be cause asymmetries in lip height are more likely to be no ticed by observers than asymmetries in lip width. Furthermore, it has been reported that a deficiency in lip width is normalized in growth 22 and that asymmetry in the lip height created on cleft lip repair is retained without relief. 17

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Authors’ Contributions Conceptualization, project administration, and supervi sion by B.S. and Y.L. Conceptualization, data collec tion, data curation, anthropometric measurements, statistic analysis, writing-original draft preparation, re view and editing by K.A. Data curation, methodology, and reviewing by C.L. Reviewing, editing, and final draft approval by A.B. Reviewing and editing, supervi sion by W.A.T. and K.A.S. We confirm that the article has been read and approved by all named authors and that there are no other persons who satisfied the criteria for authorship but are not listed. We further confirm that the order of authors listed in the article has been ap proved by all of us.

In SM, we transposed the CPh-commissures of the noncleft side without sacrificing the horizontal length of the lip at the expense of the vertical lip height by lat eralization of the PCh. Thus, we maintain the symmetry of the vertical lip height and horizontal lip width by en gaging the superiorly based advanced flap on the cleft side. This flap will allow the rotation of point 4 to the nor mal position without needing lateralization of point 4 to increase the vertical lip height, resulting in decreased lip width. The improvement in the lip width symmetry is shown by the CPh-Ch ratio (0.97). The nose is a significant element in the success of cleft lip repair. Several studies had consistently reported sig nificant outcomes when the nose repair was conducted at the time of primary lip repair. 23–25 Although several surgical techniques have adequately corrected the lips, the same success in correcting the nose cannot be achieved. 7 Suturing a part of the C-flap to the columella in the cleft side may increase its length, and this is shown by the CL ratio (0.82). However, future studies are rec ommended to find out the effect of this technique on nasal outcomes. In summary, we used the Millard method for > 30years to treat unilateral cleft lip patients; shortcomings such as insufficient lip width, height, and short columella were encountered in this method’s outcomes. To alleviate these drawbacks, in 2009, the design of the Millard method in the noncleft side was improved by advancing bisector theory to enhance the symmetry of the Cupid’s bow. In this study, we used the SM to treat UCCL pa tients in whom the C flap is rotated in to increase the col umellar length and form the nasal sill. Meanwhile, the back-cut gap is filled with an advanced flap from the cleft side, which helps improve the lip height and pre serving the lip width. These modifications will result in a straight instead of a curved scar, as shown in the Millard method (Fig. 1; right). Satisfactory labial and nasal out comes were demonstrated through quantitative analysis of the patients’ photographic measurements (see Supple mentary Figures S1–S7). For > 6 years, SM has been widely used to treat UCCL patients in the cleft lip and palate department at the West China Stomatology Hospi tal of Sichuan University, which is considered one of the largest centers for cleft lip and palate treatment in China. This study is a descriptive study of new modifications to Millard’s technique, focusing on these modifications’ in-depth description and objectives. Therefore, the lack of a comparison group could be a limitation of this study. However, future comparative studies are recommended for more details. Conclusion The present technique had leveled the Cupid’s bow while preserving the lip height and width, with resultant satis factory nasal outcomes. Downloaded by Travis T Tollefson from www.liebertpub.com at 03/02/25. For personal use only.

Author Disclosure Statement No competing financial interests exist.

Funding Information No funding was received for this article.

Supplementary Material Supplementary Figure S1 Supplementary Figure S2 Supplementary Figure S3 Supplementary Figure S4 Supplementary Figure S5 Supplementary Figure S6 Supplementary Figure S7 Supplementary Video S1

References 1. Mirault M. Lettres sur l’ope´ration du bec de lie`vre conside´re´ dans ses divers e´tats de simplicite´ et de complication. Acad Chir. 1844;2:256. 2. Millard DR, Jr. A primary camouflage of the unilateral harelook, in Transactions of the International Society of Plastic Surgeons, Skoog T, and Ivy RH, Eds., The Williams & Wilkins, Baltimore, Md, USA, 1957, p. 160. 3. Desrosiers III, AE, Kawamoto HK, Katchikian HV, Jarrahy R, Bradley JP. Microform cleft lip repair with intraoral muscle interdigitation. AnnPlast Surg . 2009;62(6):640–644. 4. Weinfeld AB, Hollier LH, Spira M, Stal S. International trends in the treatment of cleft lip and palate. Clin Plast Surg . 2005;32(1):19–23. 5. Mulliken JB, Mart´ınez-Pe´rez 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(5):1247– 1260. 6. Demke JC, Tatum SA. Analysis and evolution of rotation principles in unilateral cleft lip repair. J Plast Reconstr Aesthet Surg . 2011;64(3): 313–318. 7. Tan O. Triangular with Ala Nasi (TAN) II repair of unilateral cleft lips with severe nasal deformity. Ann Plast Surg. 2014;73(4):393–397. 8. He X, Shi B, Li S, Zheng Q. A geometrically justified rotation advancement technique for the repair of complete unilateral cleft lip. J Plast Reconstr Aesthet Surg . 2009;62(9):1154–1160. 9. Millard RD. Primary correction of unilateral cleft nose: a more accurate diagram. Plast Reconstr Surg . 1999;103(7):2094. 10. Cline JM, Oyer SL, Javidnia H, et al. Comparison of the rotation advancement and philtral ridge techniques for unilateral cleft lip repair. Plast Reconstr Surg . 2014;134(6):1269–1278. 11. Lu T-C, Lam WL, Chang C-S, Chen PK-T. Primary correction of nasal deformity in unilateral incomplete cleft lip: a comparative study be tween three techniques. J Plast Reconstr Aesthet Surg . 2012;65(4): 456–463. 12. Adetayo AM, James O, Adeyemo WL, Ogunlewe MO, Butali A. Unilateral cleft lip repair: a comparison of treatment outcome with two surgical

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19. Losee JE, Selber JC, Arkoulakis N, Serletti JM. The cleft lateral lip element: do traditional markings result in secondary deformities? Ann Plast Surg. 2003;50(6):594–599. 20. Han K, Park J, Lee S, Jeong W. Personal technique for definite repair of complete unilateral cleft lip: modified Millard technique. Arch Craniofac Surg . 2018;19(1):3. 21. Gundlach K, Schmitz R, Maerker R, Bull H. Late results following different methods of cleft lip repair. Cleft Palate J. 1982;19(3):167– 171. 22. Cutting CB, Dayan JH. Lip height and lip width after extended Mohler uni lateral cleft lip repair. Plast Reconstr Surg . 2003;111(1):17–23; discussion 24. 23. Salyer KE. Primary correction of the unilateral cleft lip nose: a 15-year experience. Plast Reconstr Surg . 1986;77(4):558–568. 24. Salyer K. New concepts in primary unilateral cleft lip-nose repair. Worldplast. 1995;2:83–97. 25. Salyer KE, Genecov ER, Genecov DG. Unilateral cleft lip-nose repair: a 33-year experience. J Craniofac Surg . 2003;14(4):549–558.

techniques using quantitative (anthropometry) assessment. J Korean Assoc Oral Maxillofac Surg . 2018;44(1):3. 13. Koh KS, Hong JP. Unilateral complete cleft lip repair: orthotopic posi tioning of skin flaps. Br J Plast Surg . 2005;58(2):147–152. 14. Ritchie HP. Congenital cleft lip and palate: a muscle theory repair of the lip cleft. Ann Surg . 1926;84(2):211. 15. Lazarus DD, Hudson DA, van Zyl JE, Fleming AN, Fernandes D. Repair of unilateral cleft lip: a comparison of five techniques. Ann Plast Surg. 1998;41(6):587–594. 16. Romero R. The Millard rotation-advancement lip repair using accurate measurements. Oral Surg Oral Med Oral Pathol Oral Radiol Endodontol. 1997;84(4):335–338. 17. Saunders DE, Malek A, Karandy E. Growth of the cleft lip following a tri angular flap repair. Plast Reconstr Surg . 1986;77(2):227–238. 18. Shi B, Sommerlad BC. Cleft lip and palate primary repair jj features of complete unilateral cleft lip. 2013, DOI: 10.1007/978-3-642-38382 3(Chapter 2), 37–42.

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CME

Evidence-Based Practices in Cleft Palate Surgery

Sarah A. Applebaum, MD Sofia Aronson, MD Kareem M. Termanini, MD Arun K. Gosain, MD Chicago, IL

Learning Objectives: After studying this article, the participant should be able to: (1) Understand the embryologic origins, cause, and incidence of cleft pal ate. (2) Review the anatomy and common classifications of cleft palate and associated defects. (3) Describe surgical techniques for palatoplasty and under stand their respective indications. (4) Gain an awareness of general periop erative care considerations, timing of repair, and risk factors for and operative mitigation of complications. Summary: Cleft palate affects 0.1 to 1.1 per 1000 births, with a higher incidence in certain ethnic groups but affecting both sexes equally. Cleft palate may occur in isolation or in combination with cleft lip or in association with other con genital anomalies including various syndromes. The goals of cleft palate repair are to anatomically separate the oral and nasal cavities for normal feeding and improved speech and minimize the risk of oronasal fistulas, velopharyngeal dys function, and disruption of facial growth. This review discusses the incidence, causes, and classification of cleft palate; surgical techniques for palatoplasty and perioperative patient management; and complications of palatoplasty. (Plast. Reconstr. Surg. 153: 448e, 2024.)

CLEFT PALATE ANATOMY, EMBRYOLOGY/CAUSE, EPIDEMIOLOGY Epidemiology of Cleft Palate Cleft palate affects 0.1 to 1.1 per 1000 births 1 ; cleft palate alone has a slight female predomi nance but overall affects both sexes equally, and is more common in Asian, Latin, and Native American children and least common in people of African descent. 2–5 Cleft palate may occur in isolation or in combination with cleft lip and other orofacial clefts or in association with over 150 syndromes, including Stickler syndrome, Down syndrome, Treacher Collins syndrome, Beckwith-Wiedemann syndrome, syndromic craniosynostosis, and Pierre Robin sequence. 6,7 Children with cleft palate, especially those with syndromic associations, may have additional air way abnormalities that must be evaluated and considered during perioperative cleft manage ment. 8 Clefting may also occur as a result of exposure to teratogens such as alcohol, tobacco smoke, anticonvulsant drugs, and organic From the Division of Plastic Surgery, Ann & Robert H. Lurie Children’s Hospital, Northwestern University Feinberg School of Medicine. Received for publication October 15, 2022; accepted August 18, 2023. Copyright © 2024 by the American Society of Plastic Surgeons DOI: 10.1097/PRS.0000000000011035

solvents. 7 In addition, in a study of over 6 mil lion live births, cleft palate was found to be asso ciated with low maternal socioeconomic status, prepregnancy tobacco smoking, and maternal infections. 5 Relevant Anatomy Cleft palate refers to clefting specifically of the secondary palate, which includes the hard palate posterior to the incisive foramen and the soft palate or velum including the uvula. The primary palate is the triangular region anterior to the incisive foramen including the alveolus, and clefting here is semantically housed under the terms “cleft lip” or “cleft alveolus,” which are distinct from “cleft palate,” although they often occur concurrently. The soft palate con tains the palatal muscles responsible for eleva tion and used in phonation, including the paired tensor veli palatini and paired levator pala tini muscles. The levator muscles are normally

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Volume 153, Number 2 • Cleft Palate Surgery

Fig. 1. Cleft palate: relevant anatomy. ( Left ) Native alignment of the levator muscle sling with a transverse orientation of the paired muscles. ( Right ) Cleft palate results in aberrant insertion of the levator muscle on the posterior hard palate with a sagittal orientation of the paired muscles.

Fig. 2. Submucous cleft palate. ( Left ) A 1-year-old child who presents with a midline palatal lucency, called the “zona pellucida” ( arrow ) that is typically visible because of separation of the levator muscles. This patient also has a notch in the posterior hard palate representing absence of the posterior nasal spine. Patients may also present with a bifid uvula. ( Right ) Schematic showing the constellation of findings in submucous cleft palate, often including a notch in the posterior hard palate and bifid uvula.

oriented transversely to form a sling that elevates the palate during phonation and constitute an important functional mechanism of the velo pharyngeal sphincter (Fig. 1, left ). Clefting dis rupts the anatomical alignment of these muscles as they assume a more sagittal vector inserting on the posterior border of the hard palate, dis rupting normal muscle function and impacting speech resonance and the ability to create plosive sounds (Fig. 1, right ). 9 In submucous cleft palate, the levator muscle alignment is disrupted, but the oral and nasal mucosa containing the mus cles is generally preserved (Fig. 2). Despite the preservation of separate oral and nasal cavities, speech may be affected in submucous cleft pal ate because of the aberrant muscle orientation, and close follow-up is indicated to determine

whether palatoplasty will be of benefit to these patients during their speech development. Cleft Palate Embryology Palatogenesis occurs between 5 and 12 weeks of human embryogenesis. 7 By the fourth week, the frontonasal, maxillary, and mandibular prom inences are established and surround the oral cavity. The frontonasal prominences divide into paired medial and lateral nasal processes. During the sixth week of embryonic development, the upper lip is formed by the fusion of the medial and lateral nasal processes with the maxillary pro cesses. The philtrum and the primary palate are formed by the subsequent fusion of the paired medial nasal processes in the midline. Failure of these steps results in cleft lip or cleft of the primary

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Plastic and Reconstructive Surgery • February 2024

palate. 10 The secondary palate forms from paired derivatives of the maxillary processes called the palatine shelves. These are initially oriented verti cally posterior to the primary palate and lateral to the tongue. During the eighth week of embryo genesis, the palatine shelves reorient into a hori zontal arrangement cephalad to the tongue and the paired palatine shelves begin to fuse at the midline. This process progresses from anterior to posterior and also allows the primary and second ary palates to fuse with one another. 11 These pro cesses establish distinct oral and nasal cavities, and failure of these processes results in cleft palate. 10 The degree of clefting corresponds to the time in development when fusion was interrupted. 7 Cleft Palate Classification Eighteen classification systems have been pro posed for cleft palate and are largely based on anatomical factors and cleft severity. 12,13 Much like cleft lip, cleft palate may be classified as unilateral or bilateral based on oronasal mucosal continu ity between the palate and the vomer, although this distinction is rarely noted. 14,15 Rather, one of the most common classification schemas for cleft palate is the Veau classification first described in 1931, and founded solely on anatomical distinc tions and deliberately omitting severity to main tain the simplest clinical groupings (Table 1). 12 Although this system is commonly used, it offers less detail than other systems that consider both anatomy and severity. One system that has gained popularity with clinicians across multiple special ties involved in cleft care is the LAHSHAL system, which is a simplified version of the “Striped-Y,” initially proposed by Kernahan and Stark in 1971 and has undergone multiple iterations of modi fications. Similar to the Striped-Y, the LAHSHAL system uses a schematic with letters corresponding to anatomical structures to more tangibly describe the location and extent of clefting (Fig. 3). 13,16 Although each system at its inception was cham pioned by its founders to be adopted widely, there remains a lack of consensus regarding the best classification system, and this poses a challenge

to establishing a shared language for communi cating cleft anatomy, severity, and the subsequent management implied by constellations of these factors. Binary clinical descriptors of cleft severity have also been established, with Bardach describ ing “wide clefts” as those greater than 1.5 cm, and “extremely wide clefts” as those greater than the width of the palatal shelves themselves. 15 Clefts within these categories carry a worse prognosis with respect to velopharyngeal outcomes, fistula rates following repair, and need for secondary surgery. 17 Given the variable size of patients at the time of repair, an absolute cutoff as proposed by Bardach may represent a different relative degree of cleft width among different patients. Instead, the palatal index has emerged as a tool to provide better relative data for a given patient, taking into consideration an individual patient’s total palatal width in relation to the width of their cleft. 18 Fig. 3. The LAHSHAL classification of cleft lip and palate. Classification system for describing the anatomical location of cleft lip and palate defects, in which “L” is the lip, “A” is the alveo lus, “H” is the hard palate, and “S” is the soft palate. (Reprinted with permission from Yao CA, Vartanian ED, Nagengast E, McCullough M, Auslander A, Magee WP. The smile index: part 3. A simple, prognostic severity scale for unilateral cleft palate. Plast Reconstr Surg Glob Open 2021;9:e3870.)

Table 1. Veau Classification of Cleft Palate Veau Classification

Structures Involved in the Cleft

Veau I Veau II

Soft palate

SURGICAL OPTIMIZATION AND TIMING

Soft and hard palate posterior to incisive foramen Soft and hard palate through alveolus unilaterally Soft and hard palate through alveolus bilaterally

Veau III

Preoperative Management The care of patients with cleft palate should ideally begin with prenatal diagnosis and

Veau IV

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Volume 153, Number 2 • Cleft Palate Surgery

Fig. 4. Suggested timeline of cleft palate care. Reprinted with permission from Comprehensive Cleft Care: Family Edition , edited by Joseph E. Losee, Richard E. Kirschner, Darren M. Smith, Christin R. Lawrence, and Amy Straub. Boca Raton, FL: CRC Press, Taylor & Francis Group; 2015.

Timing of Surgery and Sequence of Procedures Despite centuries of advancements in cleft palate repair, the timing (early versus late) and sequence of procedures (one- versus two-stage repair) remain widely debated and data exist to support both practices. 23,24 Regarding early versus late repair, the discussion has historically involved a negotiation between improving long term speech outcomes with a single-stage early repair around 12 months of age (level of evi dence: II), 25,26 and mitigating undesirable scarring that may restrict maxillary growth with a two-stage delayed repair (level of evidence: IV). 27 However, level II data suggest that there is no significant clinical difference in facial growth 28,29 or speech outcomes between the two approaches. In the United States, a single-stage repair between 6 and 12 months of age represents 85% of all cleft palate repairs performed by surgeons of the American Cleft Palate-Craniofacial Association. 30 Two recent reports from large databases have shed light on the impact of timing on early ver sus late complications (ie, 30 days 31 versus 2000 days, 32 respectively). Overall, it was found that early repair was associated with worse outcomes at both endpoints. NSQIP patients ( n = 3088) who underwent single-stage repair before 6 months of age experienced a two-fold increase in 30-day complication rates ( P = 0.04) and a four- to five fold increase in readmissions ( P = 0.02) and reop erations ( P = 0.04) within the same timeframe (level of evidence: III). 31 With respect to 2000 day complication rates, a large database project using the IBM MarketScan Commercial Database ( n = 3046) identified that patients were more likely to require a secondary procedure if pri mary repair occurred before 10 months of age. However, patients were 60% less likely to require a secondary procedure if initial repair occurred

establishment of care with a multidisciplinary team that will follow up the child until completion of facial growth. 19–21 Beginning at 3 weeks of age, we institute early feeding evaluation, followed by regular orthodontic surveillance and care through a cleft and craniofacial team. Many patients with clefts are identified prenatally. This is more com mon in patients with cleft lip and palate, as cleft palate alone is more difficult to identify on prena tal ultrasound. Once a cleft palate is identified, we like to see the patient within the first 3 weeks of life. The timeline for care of a child who presents to our multidisciplinary cleft team with isolated cleft palate is shown (Fig. 4). Following adequate pre surgical preparation and clearance of any concur rent medical comorbidities, we prefer cleft palate repair at age 11 to 12 months to provide maximum time for facial growth without compromising speech development. Patients will subsequently be followed up by a multidisciplinary cleft team annually to monitor progression of speech, den tal and orthodontic development, and maxillary growth. Because of the substantial concordance between cleft palate and other congenital anoma lies, it is important for these children to be fully screened for coexisting diseases and syndromes and to obtain medical clearance for surgery, with special consideration of cardiac anesthesia needs and careful management of any airway anomalies. There is a 1.5 times higher risk of cardiac arrest in infants under general anesthesia between the ages of 1 and 12 months, suggesting that timing and medical optimization are important. 22 In addition, it is important for children to be not only medi cally but also nutritionally optimized, and if orally fed, they must be tolerating adequate oral intake with implementation of expected postoperative feeding restrictions on breast and bottle feeding before they ever undergo surgery.

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Plastic and Reconstructive Surgery • February 2024

between 10 and 14 months of age ( P < 0.001) and 29% less likely if initial repair occurred at more than 14 months of age ( p = 0.002) (level of evi dence: III). 32 Another recent retrospective cohort study identified that patients with more extensive fea tures (ie, Veau IV cleft palate) had significantly fewer adverse events when surgery was performed after 250 days of age, whereas infants with Veau I or II cleft palate had fewer events after 125 days of age (level of evidence: IV). 24 Coexisting Disease There is a wide variation of the incidence and prevalence of coexisting disease among neonates with cleft palate, and all presentations should be fully evaluated in the 9 to 12 months preceding typical cleft palate repair, and appro priately planned for from an anesthetic stand point. American Society of Anesthesiologists Physical Status Classification class III and IV (severe systemic disease limiting day-to-day activ ity), bronchopulmonary dysplasia/chronic lung disease, and cerebral palsy are some of the great est predictors of poor outcomes after cleft pal ate repair (level of evidence: IV). 33 In addition, if the patient has a history of prematurity, it is our institutional preference to postpone primary palatoplasty until after 1 year of age to account for both, corrected postnatal age and for the increased risk of postanesthetic apnea that can be observed up to 60 weeks postnatal in prema ture infants. Airway Anomalies Patients with cleft palate often present with concurrent airway and respiratory anomalies that must be assessed for and managed appropri ately. These are more often observed in patients with syndromic clefting and include anatomical and neuromuscular abnormality. Most patients are referred for evaluation because of the clini cal observation of snoring. Several studies have sought to characterize the incidence of sleep- disordered breathing and sleep apnea in patients with cleft palate, and have found that sleep disordered breathing is evident in 37% to 75% of patients. 34 Compared with children without clefting who also have sleep apnea, children with cleft palate were more likely to demonstrate cen tral apnea episodes, suggestive of variable mech anisms for control of breathing during sleep. 34,35 These findings have prompted clinicians to rec ommend polysomnographic evaluation for all

children with cleft palate. Other options for eval uation include nasal endoscopy and bronchos copy to assess for anatomical obstructions in the airway. Perioperatively, flexible nasal endoscopy may be considered to assist with intubation. 36 Subspecialty Anesthesia Considerations The senior author (A.K.G.) suggests that it is incumbent on the surgeon to ascertain that the anesthesia team is equipped and trained to man age the difficult pediatric airway. In resource rich environments, cleft surgery should involve an anesthesiologist with subspecialty training in pediatric anesthesia. On medical mission trips abroad, it may be more critical to use an anesthe siologist with pediatric experience than a surgeon with pediatric training. 36 Patients at high risk for respiratory collapse may benefit from opiate min imization strategies and nonopiate multimodal analgesics, such as nerve blocks and local anes thetic administration, which have been shown to provide excellent analgesia and decrease postop erative opiate use. 37 Perioperative Steroids Perioperative steroids have been used suc cessfully to mitigate airway risks such as postop erative edema and subsequent respiratory distress and have also demonstrated benefits in prevent ing postoperative fever. The standard protocol used by the senior author (A.K.G.) is 0.5 mg/kg of dexamethasone initiated preoperatively and continued for up to 24 hours. A retrospective cohort study of 118 patients undergoing Furlow palatoplasty with and without steroid use demon strated no airway distress in patients with steroid administration and comparable rates of fistulas in both groups, despite prior concerns from sur geons regarding adverse impacts of steroids on wound-healing. 38 Objectives of Repair The main objectives of cleft palate correction are to reposition velar muscles to recreate anatom ical sling and maximize palatal length to achieve functional speech and restore anatomical separa tion of oral and nasal cavities for normal feeding. Repair techniques must aim to also mitigate the risks of palatoplasty, such as avoidance of oronasal fistula formation by means of tension-free closure, avoidance of maxillary growth restriction by limit ing the presence of denuded bone, and avoiding SURGICAL TECHNIQUES

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Volume 153, Number 2 • Cleft Palate Surgery

velopharyngeal dysfunction through relapsed migration of levator muscles out of their anatomi cal alignment. Hard Palate Repair Techniques The most commonly used techniques in cleft palate repairs that involve the hard palate include von Langenbeck palatoplasty, 39 Veau-Wardill Kilner pushback, 23,40–42 and Bardach two-flap palatoplasty. 43,44 Two-Flap Palatoplasty The most common technique used for one stage repair in a survey reported in 2009 was the Bardach style (two-flap palatoplasty) (Fig. 5) com bined with intravelar veloplasty or the Furlow pal atoplasty. 30 A modification of the von Langenbeck palatoplasty, the Bardach two-flap palatoplasty involves extending the lateral incisions along the anterior aspect of the secondary palate to meet in the region of the incisive foramen, thereby elevating bilateral unipedicled mucoperiosteal flaps from the hard palate and vascularized by the greater palatine arteries. 43 The senior author (A.K.G.) modifies the anterior incision to extend into the alveolar margin, separating the palatal and labial gingival layers at the cleft margin. [ See Video 1 (online) , which displays two-flap palato plasty: part 1. See Video 2 (online) , which displays two-flap palatoplasty: part 2.] This modification allows for gingivoperiosteoplasty on closure of the palate. However, if the patient had a gingivoperi osteoplasty at the time of initial cleft lip repair, one would follow the traditional Bardach tech nique and not reenter the gingiva at the time of cleft palate repair. Modifications by Salyer et al. implemented a bilayer closure of the hard pal ate involving both nasal and oral mucoperiosteal flaps, recreating a functional levator sling with velar muscle dissection and repair, and incorpo rating vomerine flaps to facilitate nasal lining closure. 45 Two-flap palatoplasty is a useful technique for closure of complete unilateral or bilateral clefts. Dissection of mucoperiosteal flaps off the greater palatine artery achieves greater mobility in flap positioning and can close anterior hard palate fistulas in unilateral complete clefts. There is also less denuded bone relative to V-Y push back and less detrimental effects on maxillary growth. Beagle studies conducted by Bardach et al. showed no interference with facial growth. 46,47 This procedure does not, however, have any effect on palatal lengthening and requires a soft palate repair to reposition the velar musculature.

Fig. 5. Two-flap palatoplasty. ( Above ) Incisions for the two-flap palatoplasty are designed along the cleft margin between the oral and nasal mucosa and along the palatal aspect of the max illary alveolus. Incisions are bounded anteriorly by the incisive foramen. ( Center ) Bilateral mucoperiosteal flaps are ( Continued )

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Plastic and Reconstructive Surgery • February 2024

Von Langenbeck Palatoplasty Originally described in 1861, this technique involves repair of an incomplete cleft by elevat ing bipedicled mucoperiosteal flaps (Fig. 6). [ See Video 3 (online) , which displays von Langenbeck palatoplasty.] In comparison to the Bardach two flap palatoplasty, this technique maintains the pal atal soft-tissue attachments to the anterior margin of the alveolus, making it a bipedicled flap. 48 The incisions are created along the edges of the cleft in the midline and lateral relaxing incisions that begin posterior to the maxillary tuberosity and follow the posterior portion of the alveolar ridge. Once both bipedicled mucoperiosteal flaps have been elevated, they are advanced to the midline and repaired primarily. In the original descrip tion, the lateral raw surfaces were left to heal by secondary intention. However, the senior author (A.K.G.) feels that the lateral surfaces should be either closed primarily or covered with a vascular ized tissue layer, such as buccal fat flaps, to mini mize subsequent contracture and maxillary growth restriction. As with the two-flap palatoplasty, this primary hard palate closure is combined with intravelar veloplasty or Furlow double-opposing Z-plasty for complete cleft closure. 49 Veau-Wardill-Kilner (V-Y Pushback) Palatoplasty A modification of the von Langenbeck pala toplasty, this technique is useful for closure of incomplete clefts (Fig. 7). In comparison to the von Langenbeck palatoplasty, the V-Y pushback raises mucoperiosteal flaps off the greater pala tine artery and divides the anterior pedicle. The V-to-Y incision made on the hard palate allows for the mucoperiosteal flaps to be pushed back and positions the levator muscles in a more ana tomical, posterior position. 23 In essence, this tech nique is a modification of von Langenbeck’s with increased posterior palatal lengthening at the expense of wider undermining and increased Fig. 5. (Continued). shown elevated from the hard palate and isolated on their pedicles, the paired greater palatine arteries. The nasal mucosal layer has been closed from the alveolus ante riorly to the tip of the uvula posteriorly. The muscles depicted in shaded gray are no longer inserted on the posterior border of the hard palate, but rather reoriented into anatomical align ment horizontally to recreate the levator sling in the posterior most soft palate. ( Below ) The oral mucosal layer is repaired last to complete the two-flap palatoplasty. [Reprinted with per mission from van Aalst JA, Kolappa KK, Sadove M. MOC-PSSM CME article: nonsyndromic cleft palate. Plast Reconstr Surg . 2008;121(Suppl):1–14.]

denuded palatal bone. The outcomes of V-Y pushback techniques have resulted in improved speech outcomes but also adverse facial growth and increased fistula rates. Fig. 6. Von Langenbeck palatoplasty. Incisions for the von Langenbeck palatoplasty are designed similar to the two flap palatoplasty with medial incisions along the cleft mar gin between the oral and nasal mucosa. However, with this approach, the anterior hard palate mucoperiosteum is kept intact, and thus both oral mucoperiosteal flaps are function ally bipedicled. [Reprinted with permission from van Aalst JA, Kolappa KK, Sadove M. MOC-PSSM CME article: nonsyndromic cleft palate. Plast Reconstr Surg . 2008;121(Suppl):1–14.] Introduced be Leonard Furlow in 1986, the double-opposing Z-plasty or “Furlow palatoplasty” is a technique used to close cleft palates by way of creating two soft palate Z-plasties that are mir ror images of each other (Fig. 8). 50 [ See Video 4 (online) , which displays Furlow palatoplasty: part 1. See Video 5 (online) , which displays Furlow palatoplasty: part 2.] The posteriorly based oral and nasal flap contain the levator muscle and position the levator sling posteriorly after closure. The palate is lengthened through the central limb of the Z-plasty. This procedure theoretically allows for hard palate closure without relaxing incisions, therefore minimizing denuded bone along the posterior alveolar ridge and maxillary growth restriction. However, given the Z-plasty closure relies on bringing in lateral soft palate tissue, wide clefts are sometimes not amenable Soft Palate Repair Techniques Furlow Palatoplasty

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