primary bilateral two stage cleft lip/nose repair: …...as we describe in part i, the dallas...

Copyright @ 2009 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.

Primary Bilateral Two<StageCleft Lip/Nose Repair: Part II

Haisong Xu, MD,*Þ Kenneth E. Salyer, MD, FACS, FAAP, FICS,Þþand Edward R. Genecov, DDS, FICD, FACD§

Abstract: The Dallas surgical protocol for primary bilateral cleftlip/nose repair depends on the anatomy of the deformity. In cases ofasymmetric bilateral clefts, an extremely small prolabium (G6 mm invertical high) or a displaced premaxilla, a 2-stage lip repair wasperformed. At the same time, assessment of the tissue available forthe columella determined the approach to the nose. In this part, thetechnique of 2-stage lip/nose repair of the bilateral cleft lip andpalate is reviewed, and the long-term outcomes are presented.

Key Words: Bilateral, primary cleft lip/nose repair, 2-stage repair,long-term outcomes, Dallas protocol, multidisciplinary

(J Craniofac Surg 2009;20: 1927Y1933)

Treatment of patients with bilateral cleft lip is more difficult thantreatment of patients with unilateral cleft lip,1 especially when a

bilateral cleft lip is accompanied by a asymmetry cleft of alveolusand palate, small prolabium, or a displaced projecting premaxilla.2

As we describe in part I, the Dallas surgical protocol for primarybilateral cleft lip/nose repair depends on the anatomy of thedeformity. The repair of bilateral complete symmetric cleft lip/nosewas performed simultaneously as a 1-stage procedure. In cases ofasymmetric bilateral clefts, an extremely small prolabium (G6-mmvertical height) or a displaced premaxilla, a 2-stage lip repair wasperformed. At the same time, assessment of the tissue available forthe columella determined the approach to the nose. So the lip/nosewas reconstructed in stages, but an attempt to complete it by 1 yearof age was the goal (Table 1). In this part, a 2-stage lip/nose repair ofthe bilateral cleft lip and palate is reviewed, and the long-termoutcomes are presented.

Two-stage bilateral lip repair is indicated in cases of a smallprolabium (e6-mm vertical height), asymmetric clefts, and cleftswith a displaced premaxilla. In cases of a small prolabium, closing 1side at a time stimulates growth and development of the prolabium

and prevents tension. Two-stage repair achieves symmetry withdifficulty in cases of asymmetric clefts and may improve the positionof the displaced premaxilla. Stage I lip repair is performed at age3 months; stage II lip repair was usually performed 6 to 8 weeks lateror when the first lip scar is soft and pliable and not contractedbecause it is totally freed and the second side is matched to it.

METHODS

Surgical TechniqueFigure 1 shows the design of the incisions on one side of the

bilateral cleft. The wider-side cleft is performed first. In this design,the peak of the Cupid’s bow was marked at the vermilion-cutaneousjunction on the lateral lip element at point A. Point C on the laterallip element indicates the junction between the incision line carriedon from point A toward the base of the alar. From this point, theincision is carried on laterally at the base of the ala and upwardinside the nasal cavity in front of and above the inferior turbinate. Onthe prolabium, the incision line is designed along the cutaneous-mucosal junction from point C¶ to point A¶. Point A¶ correspondswith point AVthe peak of the Cupid’s bow on the lateral lip element.The incision on the vermilion of the prolabium creates a bed for theinsertion of the vermilion flap (B) from the lateral lip element. PointB¶ indicates the line of incision on the prolabium where thevermilion flap from the lateral lip element will be inserted.

The mucoperiosteal flap on the prolabium is dissected fromthe bone, the vermilion flap is dissected on the lateral lip element,and the remaining incisions are made (Fig. 2). A lateral mucosa flapis sutured to the mucoperiosteal flap of the prolabium to create asulcus intraorally.

Stage I lip repair is completed (Fig. 3). The alar base isadvanced medially. The orbicularis muscles banked below theprolabium. The skin of the lateral lip element is sutured to the skin ofthe prolabium, and the vermilion flap from the lateral skin element isinserted under the mucosa of the prolabium.

Figure 4 shows the design of the incision lines for stage II liprepair. The incision line on the lateral lip element is designed alongthe vermilion-cutaneous junction, with point C at the base of the alaand point A at the peak of the Cupid’s bow. Another incision isdesigned on the prolabium to match the repaired side.

The incision is carried through the skin, muscle, and mucosaalong the vermilion-cutaneous junction (Fig. 5), dissecting andfreeing the vermilion flap (marked B in Fig. 5) on the lateral lipelement. A minimal incision is made below the base of the ala, andanother is made intranasally in front of and above the inferiorturbinate. The vermilion flap, which was banked during stage I, isdissected and raised. It is matched to the other side to give fullnessand a tubercle in the vermillion containing a variable amount ofmuscle as needed to create a full vermillion. The orbicular muscle onboth sides is freed and sutured together if possible without tension. Ifnot, they are brought into the area below the prolabial skin and

ORIGINAL ARTICLE

The Journal of Craniofacial Surgery & Volume 20, Supplement 2, September 2009 1927

From the *Department of Plastic Surgery, Renji Hospital, Shanghai Jiao TongUniversity School of Medicine, Shanghai, China; and †World CraniofacialFoundation and ‡Departments of Biomedical Sciences and §Orthodontics,Baylor College of Dentistry, Texas A&M Health Science Center, Dallas,Texas.Received March 28, 2009.Accepted for publication March 29, 2009.Address correspondence and reprint requests to Kenneth E. Salyer, MD,

FACS, FAAP, FICS, World Craniofacial Foundation, 7777 Forest Lane,Suite C 616, Dallas, TX 75230; E-mail: [email protected]

This article did not require any sources of funding.The authors declare that they had no financial interests or commercial

associations during the course of this study.Copyright * 2009 by Mutaz B. Habal, MDISSN: 1049-2275DOI: 10.1097/SCS.0b013e3181b6cc77


allowed to heal, and scar is formed between the muscle. This can stillprovide an aesthetically good mobile lip.

After the sutures are placed, bringing the alar base medially insymmetry with the opposite side, and the skin of the lateral lipelement is sutured to the prolabium, the vermilion flaps are trimmedto achieve optimal symmetry and shape (Fig. 6). Special attention toachieve symmetry in matching the wet line, the peaks of the Cupid’sbow, and the vermilion-cutaneous junction is important in creating agood lip with a tubercle in the midline.

Two-stage lip repair is completed (Fig. 7). In most cases, thelip does not require secondary correction. Some do requiresecondary surgery later. However, the nose usually requires furthersurgery at 1 year and later.

As we indicated in part I of BPrimary Bilateral Cleft Lip/noseRepair,[ the nasal reconstruction is delayed for 1 year after the liprepair, which was considered as the primary procedure. Early lip/nose repair, followed by early palate closure, provides the foundationfor long-term good speech results and nasolabial aesthetics.3,4

RESULTSOne hundred fifty-two bilateral cleft lip/nose patients with

completed data treated from 1969 to 2007 were reviewed. Forty-three (28.3%) of 152 patients had 2-stage lip closure followed byearly nasal reconstruction.

Figures 8Y12 show the long-term outcomes of the 2-stageprimary bilateral cleft lip/nose repair with preoperative andpostoperative photographs.

DISCUSSIONBilateral cleft lip repair can be performed in 1 or 2 stages.5

The choice depends on the surgeon’s proficiency and experience;however, the type and severity of the cleft must also be considered.2

Complete or incomplete symmetric bilateral clefts in which thepremaxilla is within the alveolar arch or slightly protruding can besuccessfully treated with simultaneous lip repair on both sides. One-stage lip repair facilitates creation of a symmetric, balanced lip.When the cleft lip is asymmetric, it is necessary to determine if theasymmetry results from rotation or deviation of the premaxilla,either of which would shift the prolabium to one side, or if there is acomplete cleft on one side and an incomplete cleft on the other.Asymmetry of the bilateral cleft makes the design and performanceof simultaneous bilateral cleft lip repair more difficult than when thepremaxilla and prolabium are situated at midline. Such conditionsindicate use of 2-stage lip closure for most cases of asymmetricbilateral clefts.

TABLE 1. Surgical Treatment Protocol

Patient Age/Stage Treatment

2-Stage primary bilateralcleft lip/nose repair3 mo Stage I lip closure5Y6 mo Stage II lip closure1 y Early nasal reconstruction

8 mo 2-Flap palatoplasty5 y Secondary minor lip and nose surgery7Y9 y Cancellous iliac bone graft to alveolar

cleft7-y Full-growth Distraction osteogenesis in selected

severe cases 912 mm, class IIIFull growth 40% Orthognathic surgery8Y18 y RhinoplastyVother soft tissue

FIGURE 1. Design of the incisions on 1 side of thebilateral cleft.

FIGURE 2. The incision is carried according to the design.

FIGURE 3. The appearance after stage I lip repair.

Xu et al The Journal of Craniofacial Surgery & Volume 20, Supplement 2, September 2009

1928 * 2009 Mutaz B. Habal, MD


Closure of the wider side first converts the asymmetricbilateral cleft to a unilateral cleft. The more precise techniques ofunilateral lip repair facilitate final lip reconstruction with bettersymmetry and balance. Closure of the wider side first is also bene-ficial because the narrower or incomplete cleft is left to be closedat the second stage. It is necessary to stress that 2-stage lip repairmakes it more difficult to obtain a well-proportioned philtrum andCupid’s bow than if simultaneous repair were performed.

Secondary correction is usually needed for satisfactory finalresults.6,7 The 2-stage closure of bilateral clefts prevents precisedefinition of the size of the future philtrum, so the entire prolabiummay be used to prevent tension. Consequently, the medial prolabiumis used in both stages. The medial portion of the lip may be too wide,necessitating a secondary correction to narrow it. Some surgeonshave expressed the opinion that asymmetry of the bilateral cleft lipdoes not prevent them from performing lip repair in 1 stage.8 But inour opinion, the type and severity of the cleft should be considered.

Indications for 2-Stage Lip Repair

1. a asymmetric bilateral clefts,2. an extremely small prolabium (G6 mm in vertical height), and3. a displaced premaxilla.

Advantages and DisadvantagesAdvantages

1. The bilateral cleft is transformed into a unilateral cleft.2. In the second stage, closure of the cleft is performed by applying

the designs used for unilateral cleft lip repair.3. Stimulation of growth of the prolabium by additional blood

supply from the lateral portion of the lip produces a largervolume of tissue for closure of the second side of the defectand facilitates reconstruction of the entire midportion of the lipwithout tension.

4. Excessive lip tension is avoided, especially in patients with asmall prolabium and a protruding premaxilla.

5. Reconstruction of the vermilion on the midportion of the lipseems to be more effective in 2-stage lip repair because thevermilion flap from 1 lip element is already in place. Adjustmentof the other side becomes less complicated.

6. This technique provides closure of a projecting premaxillaor a small prolabium without the use of active presurgicalorthopedics.

7. It provides foundation for a better nose because lip tension iseliminated.

FIGURE 4. The design of the incision lines for stage II lip repair.

FIGURE 5. The incision is carried, and the vermilion flaps areprepared.

FIGURE 6. Special attention to achieve symmetry inmatching the wet line, the peaks of the Cupid’s bow, andthe vermilion-cutaneous junction is important in creating agood lip with a tubercle in the midline.

FIGURE 7. The appearance after a 2-stage lip repair.

The Journal of Craniofacial Surgery & Volume 20, Supplement 2, September 2009 Primary Bilateral Cleft Lip/Nose Repair: Part II

* 2009 Mutaz B. Habal, MD 1929


FIGURE 8. Completed case. A, Preoperative bilateral cleft lip/nose, alveolus, and palate at 3 months of age. D, Patients at6 months after right-side cleft lip closure. B, C, E, F, At 23 years of age showing long-term outcome after Le Fort I maxillaryadvancement, bilateral mandibular ascending ramus sagittal split osteotomy, and sliding genioplasty.

FIGURE 9. Completed case. A, Preoperative asymmetry bilateral incomplete cleft lip at 3 months of age. D, The patient at5 months after right-side cleft lip closure. B, C, E, F, At 18 years of age showing long-term outcome without orthognathic surgery.




FIGURE 10. Completed case. A, Preoperative asymmetry incomplete bilateral cleft lip and complete cleft palate at 3 monthsof age. D, Frontal view and submittal vertex view at 10 years old. B, C, E, F, At 19 years of age showing long-term outcomeafter Le Fort I maxillary advancement, bilateral mandibular ascending ramus sagittal split osteotomy, and bilateral malaraugmentation with demineralized bone graft.

FIGURE 11. Completed case. A, Preoperative asymmetry bilateral cleft lip/nose, alveolus, and palate at 3 months of age. D, Thepatient at 1 year after 2-stage lip closure and before columella lengthening. B, C, E, F, At 18 years of age showing long-term outcomeafter a 3-piece Le Fort I osteotomy with maxillary advancement plus bilateral mandibular ascending ramus sagittal split osteotomy.




8. This technique can easily be used today in developing countrieswhere orthodontic treatment is not available.

Disadvantages

1. Two operations are performed instead of one.2. Achieving a symmetric balance of the lip is more difficult than

in simultaneous closure of both sides.3. It may require addition revisions.

The success of surgical correction of the cleft lip and nasaldeformity is only partially related to staging the repair. Unquestion-ably, both approaches can be used effectively, depending on the typeand severity of the cleft. The surgeon must be able to judge and usethe advantages offered by both approaches.

Lip RepairIn the 2-stage lip repair, we attempt to reconstruct a sym-

metric, well-balanced lip with muscle continuity. The ultimate goalof muscle repair is to bring the orbicularis oris into the prolabium.Usually, it is possible to suture the muscle together beneath the skinof the prolabium. If this cannot be done, satisfactory esthetic andfunctional results can be achieved by bringing themuscle to the edgesof the prolabium without actually suturing them together at themidline. This maneuver prevents bulging of themuscle, so secondarycorrective procedures may not be necessary.

In some cases, when the tension created by a 1-stage pro-cedure appears to be excessive, a 2-stage procedure is used. Ingeneral, the 2-stage procedure produces a more pliable lip and lessscarring.9,10 The decision of whether to use a 1- or 2-stage procedure

depends on the surgeon’s experience and judgment. It is also dependson availability of orthodontic treatment.

In the case of a severely protruding premaxilla, simultaneousclosure of both sides may be achieved, but this necessitates wideundermining of the soft tissue on the face of the maxilla, at times asfar as the orbital rim. Unquestionably, this wide undermining resultsin increased scarring, which may affect midfacial growth. Under-mining above the periosteum is believed to be a safe procedure thatdoes not inhibit subsequent growth.11,12

The midportion of the vermillion is reconstructed by bringinglateral vermillion flaps below the prolabial tissue to create a newvermilion-cutaneous junction.Muscle is placed in the vermilion flapsto create a fullness of the vermilion, especially in the midportion ofthe lip, because the tissue deficiency in this area is usually mostpronounced.

Definitive lip closure was achieved using 4-0 chromic catgutfor the mucosal and muscle layers, 6-0 PDS for subdermal closure,and 6-0 nylon for the skin.

In the case of a small prolabium, we encourage the patient’sfamily to perform vigorous massage to stimulate growth before lipclosure. Our experience indicates that preoperative massage ofthe prolabium and the lateral portions of the lip is beneficial forstimulating growth of the prolabium and growth and function of thelateral lip elements. It is interesting that no matter how small theprolabium is initially, it increases in volume as a result of stimulationfrom massage preoperatively and of an increased blood supply andrepaired lip function postoperatively.We followed some patients whoinitially had a very small prolabium and who underwent 1-stagesimultaneous bilateral lip closure. In these unique cases, there was adeficiency of tissue in the midportion, and addition operations were

FIGURE 12. Completed case. A, Preoperative bilateral complete cleft lip/nose, alveolus, and palate with small prolabium at3 months of age. D, The patient at 5 months after left-side cleft lip closure. B, C, E, F, At 18 years of age showing long-termoutcome after Le Fort I maxillary advancement and bilateral mandibular ascending ramus sagittal split osteotomy.




needed to reconstruct an adequate lip. The very small prolabium isprobably best treated by using a 2-stage lip procedure.

Columella Lengthening and NasalReconstruction

Our basic goal is to correct lip and nasal deformities at thetime of the primary procedures performed at 3 months to 1 year ofage. In the 2 stages for the lip, we attempt to reconstruct a sym-metric, well-balanced lip with muscle continuity and also suture thefloor of the nose to the alar base in a position that allows for nasalreconstruction at the next operation. After 2-stage cleft lip repair, theskeletal relationship is better, facilitating the nasal reconstruction.The nasal procedure is performed at 1 year of age. This allows 6 to9 months for maturity, and the softening of scar tissue, which isnecessary before the nasal reconstruction, can be performed. Thegoals of bilateral cleft nasal repair are the following:1. early correction of the complex nasal deformity to ensure more

normal anatomy, function, growth, and esthetic appearance ofthe infant nose;

2. columella lengthening;3. nasal tip definition;4. narrowing of the alar bases;5. alar cartilage repositioning and reshaping;6. reorientation of the nostrils from a horizontal position to a

vertical position;7. creation of a patent nasal airway; and8. complete primary nasal reconstruction by 1 year of age to allow

for more normal growth by placing the component nasalanatomic parts in as normal position and shape as is surgicallypossible at this time.

Our procedure was a modification of the technique of Croninand Denkler,13 in which early nasal deformity correction wasachieved. An extended columella incision makes it unnecessaryto make additional incisions along the alar cartilage to expose anddissect the lower lateral cartilage at the dome area. Through themidline columella incision, the lower lateral cartilage was dissectedfrom the skin, but remained attached to the nasal lining.

In our experience, trying to achieve perfect results at thisearly age may lead to a columella that is too long and estheticallyunappealing.14 This conservative approach allows for subsequentgrowth and development, creating a more normal columella and anose with tip definition and projection. Early reconstruction in thebilateral cleft nasal deformity has been performed as described overthe last 40 years, and we try to perform this by 1 year of age. Today,when possible, an open approach is used and recommended toeliminate any noticeable incision on the columella skin.13Y15 Weconsider this technique to be a primary procedure that is performedat a second stage when the tissues from stage I lip repair havebecome supple and able to accept additional surgery. The results inthe bilateral cleft patient are potentially not as good as in theunilateral deformity.1 This is due to the deformity and our inabilityto produce good results. No matter what technique or which surgeonperforms the correction, the bilateral cleft stigma remains. In theunilateral cleft lip, attractive faces with minimal deformity canconsistently be achieved.3,4,12,16 In the bilateral cleft deformity,further work is needed to achieve comparable results. In ourexperience, the technique described here gives the best possibleresult without the use of presurgical orthopedic treatment.

Today, nasoalveolar molding17,18 provides additional colu-mella skin and makes this technique outdated. However, thistechnique needs to be considered by all surgeons working wherenasoalveolar molding or other orthopedic treatment is not available.Pushing the premaxilla back to provide closure of the lip createsdental crossbite and contributes to midfacial growth abnormalities.19

This technique allows the face to grow in a more normal fashionwithout using abnormal forces that cause secondary deformity. Thisallows closure without creating alveolar tension.

CONCLUSIONSCleft form and severity of the cleft determine the approach

and surgical technique. The surgeons should find out their ownoptimal protocol that in their hands with their abilities will give themthe best results in their countries or situation. This technique shouldbe considered in the treatment of all bilateral clefts depending on theanatomy and team availability.

REFERENCES1. Brown JB, McDowell F, Byars LT. Double clefts of the lip. Surg Gynecol

Obstet 1947;85:202. Bardach J, Salyer KE. Bilateral cleft lip repair. In: Bardach J, Salyer KE,

eds. Surgical Techniques in Cleft Lip and Palate. Chicago, IL: YearBook Medical Publishers, 1987:96Y137

3. Salyer KE, Sng KW, Sperry EE. Two-flap palatoplasty: 20-yearexperience and evolution of surgical technique. Plast Reconstr Surg2006;118:193Y204

4. Salyer KE. Excellence in cleft lip and palate treatment. J CraniofacSurg 2001;12:2Y5

5. Bardach J, Salyer KE. Atlas of Craniofacial and Cleft Surgery.Philadelphia, PA: Lippincott-Raven Publishers, 1999

6. Yoshimura Y, Nakajima T, Nakanishi Y, et al. Secondary correctionof bilateral cleft lip deformity with simultaneous Abbe flap and nasalrepair. J Craniomaxillofac Surg 1998;26:17Y21

7. Good PM, Mulliken JB, Padwa BL. Frequency of Le Fort Iosteotomy after repaired cleft lip and palate or cleft palate. CleftPalate Craniofac J 2007;44:396Y401

8. Mulliken JB. Bilateral cleft lip. Clin Plast Surg 2004;31:209Y2209. Bardach J, Mooney MP, Giedrojc-Juraha ZL. A comparative study of

facial growth following cleft lip repair with or without soft tissueundermining: an experimental study in beagles. Presented at the 40thAnnual Meeting of the American Cleft Palate Association, Indianapolis,IN, May 1983

10. Wolfe SA, Ghurani R, Mejia M. Use of staged rotation-advancementprocedures for the treatment of incomplete bilateral clefts of the lip.Ann Plast Surg 2004;52:263Y268; discussion 269

11. Sumiya N, Ito Y, Otani K, et al. Correction of the bilateral completecleft lip: transformation to a unilateral incomplete cleft lip closure.Ann Plast Surg 2001;46:369Y374

12. Salyer KE, Genecov ER, Genecov DG. Unilateral cleft lip-noserepairVlong-term outcome. Clin Plast Surg 2004;31:191Y208

13. Cronin TD, Denkler KA. Correction of the unilateral cleft lip nose.Plast Reconstr Surg 1988;82:419Y432

14. McComb H. Primary repair of the bilateral cleft lip nose: a 15-yearreview and a new treatment plan. Plast Reconstr Surg 1990;86:882Y889;discussion 890Y893

15. Trott JA, Mohan N. A preliminary report on one stage open tiprhinoplasty at the time of lip repair in bilateral cleft lip and palate: theAlor Setar experience. Br J Plast Surg 1993;46:215Y222

16. Salyer KE, Genecov ER, Genecov DG. Unilateral cleft lip-nose repair:a 33-year experience. J Craniofac Surg 2003;14:549Y558

17. Cutting C, Grayson B, Brecht L, et al. Presurgical columellar elongationand primary retrograde nasal reconstruction in one-stage bilateralcleft lip and nose repair. Plast Reconstr Surg 1998;101:630Y639

18. Lee CT, Garfinkle JS, Warren SM, et al. Nasoalveolar molding improvesappearance of children with bilateral cleft lipYcleft palate. PlastReconstr Surg 2008;122:1131Y1137

19. Berkowitz S, Mejia M, Bystrik A. A comparison of the effects of theLatham-Millard procedure with those of a conservative treatmentapproach for dental occlusion and facial aesthetics in unilateral andbilateral complete cleft lip and palate: part I. Dental occlusion. PlastReconstr Surg 2004;113:1Y18



primary bilateral two stage cleft lip/nose repair: …...as we describe in part i, the dallas...

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