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Management of the cleft lip deformity
Facial Plast Surg Clin N Am 13 (2005) 157 – 167
Management of the cleft lip deformity Jonathan M. Sykes, MD, Travis T. Tollefson, MD* Division of Facial Plastic and Reconstructive Surgery, Department of Otolaryngology – Head and Neck Surgery, University of California – Davis Medical Center, 2521 Stockton Boulevard, Suite 7200, Sacramento, CA 95817, USA
A congenital cleft lip is a deformity that has significant physical and psychologic impact. Successful repair of the cleft lip deformity is a challenging and rewarding task. Cleft lips can be unilateral or bilateral. They may be isolated or associated with cleft palate. Clefts of the lip and palate may also be associated with other congenital anomalies and may be a part of a genetic syndrome. Many surgical repairs have been proposed for reconstruction of unilateral cleft lip deformities, including straight-line repairs and various forms of geometric flap repair. This article classifies cleft deformities and describes the history and specific techniques of unilateral cleft lip repair. Understanding and application of these techniques can aid the cleft surgeon in maximizing function and appearance of a child born with a cleft lip deformity.
lip, the central maxillary alveolar arch and associated lateral and central incisors, and the hard palate anterior to the incisive foramen [1,2]. The secondary palate develops at approximately 8 to 12 weeks’ gestation, after the primary palate has completely fused. Development of the secondary palate occurs by inferior and medial growth and migration of the palatal shelves (medial projections of the maxillary processes). Because normal development of the lip (primary palate) and the palate (secondary palate) occur sequentially rather than coincidentally, cleft lip may or may not be associated with cleft palate. Deformities of the lips, palate, and nose result from interruptions in the normal developmental process. The severity of the deformity is related to the amount, timing, and location of the embryonic interruption [3].
Classification of cleft lips Embryology of cleft lips Normal development of the lip occurs between weeks 4 and 8 of intrauterine life. The primary palate forms at this time and is the initial separation between the oral and nasal cavities. The primary palate, or median palatine process, is formed by the fusion of the paired median nasal prominences (MNP). Fusion of the paired MNP gives rise to the central upper
This updated article was originally published in Facial Plastic Surgery Clinics of North America 9:1, February 2001. * Corresponding author. E-mail address: [email protected] (T.T. Tollefson).
Human cleft lip is caused by a failure of the MNP to make contact with the lateral nasal process (LNP) and the maxillary process (MXP) during embryogenesis [4]. The lip deformity may involve only the vermilion or may be full thickness, involving all tissue layers. A minor malformation of normal lip development may cause dehiscence of the orbicularis oris muscles with minimal or no overt clefting of the epidermis of the upper lip. This defect is known as a microform cleft lip (Fig. 1). An incomplete unilateral cleft lip involves a through-and-through defect of the skin, muscle, and mucosa of the lower aspect of the lip (Fig. 2). An incomplete cleft lip, however, spares some of the superior portion of the upper lip.
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Fig. 1. Patient with left microform cleft lip.
Fig. 3. Patient with right complete cleft lip, palate, and alveolus.
A complete unilateral cleft lip occurs when the deformity involves all layers of the upper lip, and it involves the entire height of the upper lip (Fig. 3). A complete cleft of the primary palate frequently is bridged by a bar of lip tissue, referred to as a Simonart’s bar or band [5]. This connection of soft tissue may lessen the underlying skeletal deformity in complete cleft lips. If disruption of both sides of lip development occurs, a bilateral cleft lip results. In the incomplete bilateral cleft lip, there is usually some skeletal continuity between the premaxilla and the palatal shelves and little or no protrusion of the premaxilla (Fig. 4). In complete bilateral cleft lips, the central premaxilla is totally detached from and protrudes anterior to each maxilla (Fig. 5).
could affect healing and safety. In 1966, Wilhelmsen and Musgrave [6] proposed the preoperative requirements in their ‘‘rule of 10’’: Weight—10 lbs Hemoglobin—10 g White blood cell count—less than 10,000/mm3 In 1957, Millard [7] proposed the ‘‘rule of order 10’’: Weight—over 10 lbs Hemoglobin—over 10 g Age—over 10 weeks
The decision to repair a cleft lip includes consideration of severity of the deformity, size, and health of the child, and other anomalies or problems that
In cases of narrow or moderate width clefts, the general principle of performing cleft lip repair at 3 months of age is a good one; however, several circumstances exist that alter the timing from the usual 3 months of age for definitive lip repair. In patients with associated congenital anomalies, potential airway problems, or other health problems, it is often wise to delay lip repair until an age when general anesthesia becomes safer. Many cleft lip deformities are not amenable to a single-stage
Fig. 2. Patient with right incomplete cleft lip.
Fig. 4. Patient with incomplete bilateral cleft lip and alveolus.
Timing of cleft lip repair
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Fig. 5. Patient with bilateral complete cleft of the lip, alveolus, and palate.
definitive repair. Examples of this include very wide unilateral complete cleft lips or wide complete bilateral cleft lips with a protrusive ‘‘locked out’’ premaxilla. In these cases, the lip adhesion procedure is performed at approximately 3 months of age, with definitive repair being performed at 5 to 6 months of age. The general timing of cleft procedures is outlined in Table 1.
Anatomy of the unilateral cleft lip A unilateral cleft of the upper lip involves alteration in all layers of the lip, including the skin, muscle, mucosa, and underlying skeleton. The external form of the defect is determined by the extent of the underlying muscular and skeletal deformity. The principle muscle of the lips is the orbicularis oris (Fig. 6). The fibers of this muscle encircle the oral orifice within the substance of the lips. The orbicularis oris muscle consists anatomically of two parts: the superficial and the deep layers. This muscle is not a true sphincter, with the superficial and deep
Fig. 6. The minetic muscles of the lower face. Note the insertion of the midface and lower facial muscles into the orbicularis oris muscles of the lip and mouth. (From Sykes J, Senders C. Pathologic anatomy of cleft lip, palate, and nasal deformities. In: Meyers AD, editor. Biological basis of facial plastic surgery. New York: Thieme Medical Publishers; 1993. p. 59; with permission.)
components arising as separate muscles from the modiolus at each oral commissure [8]. In the unilateral cleft lip deformity, there is discontinuity of the orbicularis oris muscle in the region of the cleft (Fig. 7). The muscles of the unilateral cleft lip have two distinct differences when compared with normally developed lip musculature [9]. First, the muscles are hypoplastic in the region of the cleft. Second, the muscles cannot cross the cleft gap and alternately travel along the margin of the cleft. The orbicularis oris muscles are prevented from attaching to their normal sites and find substitute insertions. In complete unilateral cleft lips, it is obvious that no muscle is present in the through and through cleft
Table 1 Timing of cleft repairs Procedure
Age
Cleft lip repair Tip rhinoplasty Tympanostomy tubes Palatoplasty T-tube placement Speech evaluation Velopharyngeal insufficiency workup and surgery (if necessary) Alveolar bone grafting Nasal reconstruction Orthognathic surgery (if necessary)
3 months
9 – 18 months 3 – 4 years 4 – 6 years
9 – 11 years 12 – 18 years At completion of mandibular growth (>16 years)
Fig. 7. The minetic muscles of a patient with left complete cleft of the lip and palate. Note the abnormal insertion of the orbicularis oris muscles, which insert along the long axis of the cleft. (From Sykes J, Senders C. Pathologic anatomy of cleft lip, palate, and nasal deformities. In: Meyers AD, editor. Biological basis of facial plastic surgery. New York: Thieme Medical Publishers; 1993. p. 61; with permission.)
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Fig. 8. The normal vasculature of the lips and nose. The main blood supply of the lips comes from the superior and inferior labial arteries, branches of the common facial artery. (From Sykes J, Senders C. Pathologic anatomy of cleft lip, palate, and nasal deformities. In: Meyers AD, editor. Biological basis of facial plastic surgery. New York: Thieme Medical Publishers; 1993. p. 60; with permission.)
gap. The incomplete cleft lip, however, contains soft tissue, which could contain muscle. Dissections on stillborn babies performed by Fara and colleagues [9] revealed that muscles in unilateral cleft lips were more hypoplastic on the medial side than on the lateral side of the cleft. These dissections also revealed that the muscles in incomplete clefts did not cross the cleft gap unless the skin bridge was at least one third of the height of the lip. When a small amount of muscle is present within an incomplete cleft lip, the orientation of the muscle invading the incomplete cleft skin bridge is still abnormal. The vascular supply to the lip in the unilateral cleft lip deformity is similar to the muscle abnormalities. The superior labial artery, a branch of the facial artery, courses obliquely along the margin of the cleft (Fig. 8). At the base of the nose, the superior labial artery anastomoses with the angular artery or the lateral nasal artery. The blood supply in the unilateral cleft lip deformity is stronger and more developed on the lateral side than it is on the medial side (Fig. 9). In the incomplete cleft lip deformity, a thin terminal branch of the superior labial crosses the skin bridge.
then wrapped with thread in a figure-eight fashion. Rose [11] and Thompson [12] described modification of straight-line closures for repair of the unilateral cleft lip deformity. All of these straight-line techniques closed the cleft defect adequately but often resulted in vertical scar contracture and notching of the lip. Several efforts have since been made to improve the aesthetic and functional results obtained with straight-line closure techniques. These include multiple types of geometric repairs with full-thickness lip flaps. These techniques were designed to irregularize the lip scars and prevent vertical scar contracture and notching of the upper lip. LeMesurier [13] described a quadrilateral fullthickness flap based laterally (from the cleft side). This inferior flap interrupted the scar at the vermilioncutaneous junction. Tennison [14] and Marcks and colleagues [15] introduced a triangular flap, which created a Z-plasty at the lower aspect of the lip. Subsequently, Randall [16] used the same design as Tennison but reduced the size of the triangular flap. In 1957, Millard [7] described the rotationadvancement flap technique for repair of the unilateral cleft lip deformity. This repair creates two major full-thickness flaps and is designed to place cleft scar in the philtral column. The rotation-advancement technique is presently the most commonly used procedure for unilateral cleft lip repair.
Philosophy of the rotation-advancement repair The primary goals of unilateral cleft lip repair are to reconstruct normal lip anatomy and restore lip
History of cleft lip surgery The first documented repair of a unilateral cleft lip was in approximately 390 ad in the Tang dynasty in China. The cleft lip edges were cut and sutured, and the child was instructed not to speak for 100 days. Ambroise Pare [10] repaired a unilateral cleft by freshening the cleft edges and skewering the two sides of the cleft with a long needle. The needle was
Fig. 9. The aberrant vasculature of a patient with a complete left unilateral cleft lip and palate. (From Sykes J, Senders C. Pathologic anatomy of cleft lip, palate, and nasal deformities. In: Meyers AD, editor. Biological basis of facial plastic surgery. New York: Thieme Medical Publishers; 1993. p. 62; with permission.)
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function [17]. Other goals include closure of the nasal floor, correction of nasal tip asymmetry, and narrowing of the alveolar cleft. The rotation-advancement flap technique involves two major full-thickness flaps that can be approximated to repair the cleft without notching of the lip. This design allows reconstitution and reorientation of the orbicularis oris muscles. The advantages and disadvantages of the Millard technique compared with geometric flap techniques are summarized in Table 2. Geometric flap techniques require premeasured geometric flap design. This allows the inexperienced surgeon to study specific flap design and become secure with precise flap shape and measurement. In general, however, geometric flap repair confines the surgeon and allows less flexibility during the surgical procedure. The main advantage of the rotation-advancement technique is its flexibility in application. The Millard procedure is a ‘‘cut as you go’’ technique, allowing continuous modifications during the design, incisions, and execution of the repair. Another advantage of the rotation-advancement technique is that the incisions are designed to place the scar in the new philtral column. Adherence to this known aesthetic subunit allows camouflage of the eventual scar. Most geometric flap design, on the other hand, violates the philtral subunit. The other advantages of the rotationadvancement technique are that minimal normal tissue is discarded and maximal repair of the muscle is accomplished. A disadvantage of the rotation-advancement flap technique is that the inexperienced surgeon may have difficulty without the aid of a premeasured flap design. Additionally, this technique requires extensive undermining and may have a tendency toward creating a small nostril on the cleft side. For this reason, the surgeon using this technique should attempt to make the cleft nostril slightly larger than the nostril on the noncleft side. It is easier to correct a large nostril than it is to repair nostril stenosis.
Table 2 Millard rotation-advancement repair Advantages
Disadvantages
Flexible Minimal tissue discarded Good nasal access Camouflaged suture line
Requires experienced surgeon Possible excessive tension Extensive undermining required Vertical scar contracture Tendency toward small nostril
From Ness JA, Sykes JM. Basics of Millard rotationadvancement technique for repair of the unilateral cleft lip deformity. Facial Plast Surg 1993;9:168; with permission.
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Fig. 10. The important reference points of the Millard rotation-advancement technique for repair of the unilateral cleft lip deformity. (From Ness JA, Sykes JM. Basics of Millard rotation-advancement technique for repair of the unilateral cleft lip deformity. Facial Plast Surg 1993;9:169; with permission.)
Measuring and design of incision The important reference points of the technique are shown in the following list and in Fig. 10. 1. Center (low point of Cupid’s bow—noncleft side) 2. Peak of Cupid’s bow—lateral noncleft side 3. Peak of Cupid’s bow—medial noncleft side 4. Alar base—noncleft side 5. Columellar base—noncleft side 6. X Back-cut point—noncleft side 7. Commissure—noncleft side 8. Commissure—cleft side 9. Peak of Cupid’s bow—cleft side 10. Medial tip of advancement flap—cleft side 11. Midpoint of alar base—cleft side 12. Lateral alar base—cleft side These points should be measured and marked before the infiltration of local anesthesia. This will prevent ballooning and distortion by the anesthetic/vasoconstrictive agent. Various measurements can be made to ensure accurate marking of the skin points. The following is a list of measurements for flap design: 1 2 2 3
to to to to
2 6 4 5
= = = +
1 8 8 x
to 3 = 2 – 4 mm to 7 = 20 mm to 10 = 9 – 11 mm = 8 to 9
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raised in the supraperiosteal dissection plane. A small skin flap, flap c, is the byproduct of the rotation incision at the columellar base, and it is elevated in the subcutaneous plane. The D flap is an alar flap, which is created by the perialar portion of the advancement incision. The designation for the skin and mucosal flaps is summarized in Fig. 12 and in the following list: A = rotation flap B = advancement flap c = columellar base soft tissue, noncleft side D = alar rim, cleft side m = medial mucosal flap l = lateral mucosal flap
Fig. 11. (A) Lengths of the rotation flap (3 to x) and the advancement flap (8 to 9). Note that the lengths of the advancement flap should always equal the length of the rotation flap. (B) Use of the paper clip technique to measure the curved rotation flap as being equal in length to the straight advancement flap. (From Ness JA, Sykes JM. Basics of Millard rotation-advancement technique for repair of the unilateral cleft lip deformity. Facial Plast Surg 1993; 9:171; with permission.)
The capital letter flaps A, B, and D are full-thickness flaps, whereas the small letter flaps c, m, and 1 are thin subcutaneous or submucosal flaps. The cutaneous c flap may be discarded or used to create the nasal floor. The mucosal m and 1 flaps may be discarded or used to help create an intraoral sulcus. After elevation of the major rotation and advancement flaps, an external alotomy is performed
These measurements are performed to add precision to the skin markings for the advancement flap and the rotation flap. The most important measurement is that the length of the rotation flap (3 to 5 + x) equals the length of the advancement flap (8 to 9) (Fig. 11). Although this measurement is often performed subjectively, objective analysis of the relative lengths can be made with a 26-gauge wire. The wire is first bent to correspond to the length of the rotation flap and then is straightened to measure the advancement flap. If these lengths are not equal, modification in the skin markings is made to equalize the two flap lengths. Incision and flap elevation The cutaneous incisions of the rotation and advancement flaps are made with a 15c-knife blade. Mucosal incisions are made with an 11-knife blade. The mucosal incisions create two thin mucosal flaps (m = medial, 1 = lateral). These flaps are raised in the submucosal plane. The major lip flaps (A = rotation, B = advancement) are full-thickness flaps and are
Fig. 12. (A) The major flaps of the Millard unilateral cleft lip repair. (From Ness JA, Sykes JM. Basics of Millard rotation-advancement technique for repair of the unilateral cleft lip deformity. Facial Plast Surg 1993;9:171; with permission.) (B) Intraoperative view of a patient after marking of the flaps for repair.
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determine if closure can be accomplished easily (Fig. 14). If too much tension on the closure exists, further dissection and undermining is performed to decrease wound tension. Active placement of the alar base
Fig. 13. Intraoperative view of a patient after elevation of the major rotation and advancement flaps before performance of an alotomy.
(Fig. 13). An internal alotomy is then accomplished to free the alar flap from the piriform aperture. Elevation of the skin and mucosa off of the orbicularis oris muscle is then performed. Lastly, dissection over the nasal tip is accomplished through the perialar and columellar base incisions. After all elevation is completed, single-pronged hooks are placed at the vermilion border of each lip flap to
After the alar flap is freed, active placement of the alar base is performed [18]. The width of the cleft side alar base should be slightly greater than that of the noncleft side to prevent nostril stenosis after wound healing. Closure of the nasal floor is accomplished with 4-0 chromic catgut sutures. Muscle closure It is important to isolate and precisely reconstruct the orbicularis oris muscles. This will relieve tension and make the lip function better. Careful muscle closure will also provide appropriate volume to the lip and prevent inversion (depression) of the cleft lip scar. The muscle is reapproximated with a 4-0 long-acting monofilament absorbable suture (Fig. 15). These muscle sutures are the strength of the lip closure. Skin closure After the alar base position is established and the orbicularis muscle is reconstituted, closure of the oral mucosa and the epithelium of the lip is performed. Intraoral mucosal incisions are closed with
Fig. 14. (A) Use of single-pronged hooks to test the advancement and rotation of the major flaps in the unilateral cleft lip repair. (From Ness JA, Sykes JM. Basics of Millard rotation-advancement technique for repair of the unilateral cleft lip deformity. Facial Plast Surg 1993;9:173; with permission.) (B) Intraoperative view depicting the use of this technique to assess tension on the repair.
Fig. 15. Closure of the orbicularis oris muscle before skin closure in the cleft lip repair. (From Ness JA, Sykes JM. Basics of Millard rotation-advancement technique for repair of the unilateral cleft lip deformity. Facial Plast Surg 1993;9:174; with permission.)
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5-0 chromic catgut sutures. The m and 1 mucosal flaps may be used to provide tissue to close the sublabial sulcus. Trimming of the mucosa before closure is usually necessary to provide the precise contour and volume to the vermilion of the lip. The external skin is approximated with 6-0 monofilament and 6-0 fast-absorbing gut sutures. Key sutures of 6-0 monofilament nonabsorbable sutures are placed at the base of the nose and at the vermilion-cutaneous junction. Tip rhinoplasty Undermining of the nasal tip is performed by dissecting the external skin from the underlying lower lateral cartilages (Fig. 16) [19]. The vestibular skin is not dissected from the underside of the alar cartilages to minimize the risk of circumferential alar (nostril) stenosis. After freeing the nasal skin from the lower lateral cartilages, tip rhinoplasty is performed. The goal of primary cleft rhinoplasty in the unilateral deformity is to improve nasal tip symmetry, definition, and projection. To minimize the chance of nasal growth inhibition, the lower lateral cartilages are not removed or incised. Instead, repositioning of the cartilages is accomplished with bolster sutures (Fig. 17). This corrects malpositioned cleft side lower lateral cartilage. If properly performed, primary cleft lip rhinoplasty will lessen the primary deformity and decrease the need for intermediate cleft rhinoplasty. Bilateral cleft lip repair The complete bilateral cleft deformity consists of the two lateral lip segments and a midline prolabium overlying the premaxilla. There are several key anatomical differences between the unilateral and bilateral cleft lip deformities that must be considered
Fig. 17. Intraoperative view after repositioning of the cleft lower lateral cartilages with bolster sutures.
before repairing the bilateral cleft lip (Table 3). Similar to the lateral lip segment in the unilateral cleft lip deformity, the aberrant orbicularis oris muscle insertions of the two lateral lip segments course superiorly toward the alar base. However, the central prolabium never contains muscle. Varying degrees of anterior premaxillary projection as well as rotation can occur. Some surgeons advocate either lip adhesion techniques or staged one-sided repairs to posteriorly reposition the premaxillary segment. Others argue that staged repairs can create asymmetry. This article describes a single-stage bilateral cleft lip repair and addresses the unique characteristics of the bilateral cleft lip deformity. Measurement and design First, the midline on the vermillion-cutaneous junction of the prolabial flap is marked. The philtral width, which is expected to widen with growth, is measured using a caliper to mark 2 to 2.5 mm on each side of the midline (total philtral width: 4 – 5 mm). Vertical markings are created to extend up to just superiomedial to the collumellar base. The lateral vermillion borders are then marked to create the prolabial forked flaps. Table 3 Keys to bilateral cleft lip repair
Fig. 16. lntraoperative view showing undermining of the nasal tip during cleft lip rhinoplasty. Note that the undermining occurs over the medial and lateral aspect of the lower lateral cartilages.
Characteristic
Surgical approach
Premaxilla may be ‘‘locked out’’ Prolabium contains no muscle
Lip adhesion or lip repair
Orbicularis oris muscle inserts superiorly Lateral lip has more volume than prolabium Wide, maloriented nasal tip, short collumella
Lateral muscle sutured at midline (Concentric orbicularis oris ring) Reoriented muscle under prolabium Mucosal flaps add volume Tip rhinoplasty
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intraoral sublabial incisions are extended intraorally to release the cheek – lip complex from the maxilla, with care to preserve the infraorbital neurovascular bundles. Lastly, the lateral lip advancement skin flaps are conservatively undermined from the reoriented orbicularis oris muscle fibers. The malpositioned lower lateral cartilages, including the shortened medial crus, are freed from the skin soft tissue envelope (Fig. 16). Fig. 18. Intraoperative view showing the marked prolabial flap (p) and forked flaps (f). The lateral lip advancement flaps (a) and mucosal flaps (m) have been elevated. The vertical heights of the prolabial and lateral lip advancement flaps are equal.
Next, the lateral lip markings are designed to approximate the vertical height of the prolabial flap. This vermillion marking begins where the mucosa begins to thin superior-medially. Marking and design of the mucosal flaps is similar to those in unilateral cleft repairs (Fig. 18). Bilateral sublabial incisions were designed to extend from the mucosal flaps to allow release of the cheek – lip complex from the maxilla. Incision and flap elevation With the prolabial flap incised and elevated, the inferiorly based prolabial mucosa is sutured under the prolabial flap to create a labioalveolar sulcus. The forked flaps are incised and elevated in a subcutaneous plane to be rotated laterally into the floor of nose as needed. Next, the mucosal flaps on the lateral lip segments are elevated with a conservative backcut into the nasal-alar groove (Fig. 19). The lateral
Fig. 19. Intraoperative view of the advanced mucosal flaps before elevation of the prolabial flap. The muscle layer closure will be performed anterior to the premaxilla, but deep to the prolabial flap. This minimizes tension on the skin closure and creates a concentric ring of orbicularis oris.
Nasal floor closure and alar base width The lateral lip segments’ mucosal closure over the premaxilla, but under the prolabial flap, is performed with 5-0 chromic catgut sutures. The forked flaps can be rotated laterally to add to the nasal floor closure, or these flaps can be discarded. Release of the lateral nasal wall mucosa can help obtain nostril size symmetry. The alar base width is actively placed with a 4-0 monofilament absorbable suture. Orbicularis oris closure The orbicularis oris muscle should be reoriented over the premaxilla to create a concentric muscular ring, as well as to prevent dynamic inferior displacement of the alar base with muscle contraction. The ‘‘key suture’’ (4-0 long-acting monofilament absorbable) is placed at the junction of the white and red lip to create vertical height symmetry and lip fullness. Skin closure After the mucosal and muscle approximation are completed, the Cupid’s bow is created by aligning the prolabial flap vermillion with the lateral lip vermillion using a permanent 6-0 monofilament suture (Fig. 20). Often, a back-cut at the vermillion border of the lateral lip segment is helpful. Other than the vertical lip height and horizontal symmetry, the third dimension to be considered must be lip fullness
Fig. 20. Intraoperative view of the final closure before lower lateral cartilage bolster placement.
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Fig. 21. (A) Intraoperative photograph of a 3 month old patient with a bilateral cleft lip and palate deformity. (B) Eight-month postoperative photograph of the same patient. (C) Ten-year postoperative photograph of the same patient.
(Fig. 21). A mucosal z-plasty can be used to distribute lip volume and prevent a ‘‘whistle’’ deformity that can be seen with central mucosal lip deficiencies.
with 4-0 permanent sutures. The shortened collumella can be addressed in subsequent surgeries if necessary.
Tip rhinoplasty The nasal tip skin elevation is performed before lip closure to allow the lower lateral cartilages to be repositioned. After lip closure is completed, the lower lateral cartilages are sutured in a more cephalic and medial position using two to four bolsters secured
Summary Repair of the unilateral cleft lip deformity is a challenging and rewarding procedure. Historically, many techniques have been described to reconstruct the unilateral cleft lip. These have included straight-
Fig. 22. (A) Preoperative view of a patient with a right complete unilateral cleft lip. (B) The same patient 3 months after repair of the cleft lip with the Millard rotation-advancement technique.
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line repairs and various geometric flap closures. The rotation-advancement flap technique of Millard is a reliable and versatile method for repair of the unilateral cleft lip deformity. This technique allows lip repair and tip rhinoplasty while camouflaging the scars in the newly formed philtral border. If properly applied, the rotation-advancement repair produces excellent functional and aesthetic results (Fig. 22).
References [1] Enlow DH. Facial growth. 3rd edition. Philadelphia7 WB Saunders; 1990. [2] McCarthy JG. Plastic surgery: cleft lip and palate and craniofacial anomalies, volume 4. Philadelphia7 WB Saunders; 1990. [3] Sykes JM, Senders CW. Facial plastic surgery: cleft lip and palate, volume 9. New York7 Thieme Medical Publishers; 1993. [4] Gaare JD, Langman J. Fusion of nasal swellings in the mouse embryo: surface coat and initial contact. Am J Anat 1977;150:461 – 75. [5] Simon G. Ueber die Uranoplastik mit besonderer Beruecksichtigung der Mittel zur Wiederserstellung einer reinen Sprache. Greifswalder Med Beitr 1866; 2:129 [in German]. [6] Wilhelmsen HR, Musgrave RH. Complications of cleft lip surgery. Cleft Palate J 1966;3:223 – 31. [7] Millard Jr DR. A primary camouflage of the unilateral harelook. Transactions of the 1st International Congress of Plastic Surgery, Stockholm. Baltimore (MD)7 Williams & Wilkins; 1957.
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[8] Burkitt AN, Lightoller GHS. The facial musculature of the Australian aboriginal. J Anat 1928;62:33 – 57. [9] Fara M, Chlumska A, Hrivnakova J. Musculis orbicularis oris in incomplete hare-lip. Acta Chir Plast 1965; 7:125 – 32. [10] Pare A. Dix livres de la chirurgie. Paris7 Jean Le Roger; 1564 [in French]. [11] Rose E. Udber den plastischen Ersatz des harten Gaumens aus der Lipp. Arch Klin Chir 1879;24:438 [in German]. [12] Thompson JE. An artistic and mathematically accurate method of repairing the defect in cases of harelip. Surg Gynecol Obstet 1912;14:498 – 505. [13] LeMesurier AB. Method of cutting and suturing lip in complete unilateral cleft lip. Plas Reconst Surg 1949; 4:1. [14] Tennison CW. The repair of the unilateral cleft lip by the stencil method. Plast Reconst Surg 1952;9:115 – 20. [15] Marcks KM, Trevaskis AE, DaCosta A. Further observations in cleft lip repair. Plast Reconstr Sug 1953;12:392. [16] Randall P. A triangular flap operation for the primary repair of unilateral clefts of the lip. Plast Reconstr Surg 1959;23:331. [17] Ness JA, Sykes JM. Basics of Millard rotationadvancement technique for repair of the unilateral cleft lip deformity. Facial Plast Surg 1993;9:167 – 76. [18] Sykes JM, Senders CW. Surgical treatment of the unilateral cleft nasal deformity at the time of lip repair. Facial Plast Surg Clin North Am 1995;3:1. [19] Sykes JM, Senders CW. Surgery of the cleft lip nasal deformity Operative techniques in otolaryngologyhead and neck surgery, volume 1. Philadelphia7 WB Saunders; 1990.
Management of the cleft lip deformity Jonathan M. Sykes, MD, Travis T. Tollefson, MD* Division of Facial Plastic and Reconstructive Surgery, Department of Otolaryngology – Head and Neck Surgery, University of California – Davis Medical Center, 2521 Stockton Boulevard, Suite 7200, Sacramento, CA 95817, USA
A congenital cleft lip is a deformity that has significant physical and psychologic impact. Successful repair of the cleft lip deformity is a challenging and rewarding task. Cleft lips can be unilateral or bilateral. They may be isolated or associated with cleft palate. Clefts of the lip and palate may also be associated with other congenital anomalies and may be a part of a genetic syndrome. Many surgical repairs have been proposed for reconstruction of unilateral cleft lip deformities, including straight-line repairs and various forms of geometric flap repair. This article classifies cleft deformities and describes the history and specific techniques of unilateral cleft lip repair. Understanding and application of these techniques can aid the cleft surgeon in maximizing function and appearance of a child born with a cleft lip deformity.
lip, the central maxillary alveolar arch and associated lateral and central incisors, and the hard palate anterior to the incisive foramen [1,2]. The secondary palate develops at approximately 8 to 12 weeks’ gestation, after the primary palate has completely fused. Development of the secondary palate occurs by inferior and medial growth and migration of the palatal shelves (medial projections of the maxillary processes). Because normal development of the lip (primary palate) and the palate (secondary palate) occur sequentially rather than coincidentally, cleft lip may or may not be associated with cleft palate. Deformities of the lips, palate, and nose result from interruptions in the normal developmental process. The severity of the deformity is related to the amount, timing, and location of the embryonic interruption [3].
Classification of cleft lips Embryology of cleft lips Normal development of the lip occurs between weeks 4 and 8 of intrauterine life. The primary palate forms at this time and is the initial separation between the oral and nasal cavities. The primary palate, or median palatine process, is formed by the fusion of the paired median nasal prominences (MNP). Fusion of the paired MNP gives rise to the central upper
This updated article was originally published in Facial Plastic Surgery Clinics of North America 9:1, February 2001. * Corresponding author. E-mail address: [email protected] (T.T. Tollefson).
Human cleft lip is caused by a failure of the MNP to make contact with the lateral nasal process (LNP) and the maxillary process (MXP) during embryogenesis [4]. The lip deformity may involve only the vermilion or may be full thickness, involving all tissue layers. A minor malformation of normal lip development may cause dehiscence of the orbicularis oris muscles with minimal or no overt clefting of the epidermis of the upper lip. This defect is known as a microform cleft lip (Fig. 1). An incomplete unilateral cleft lip involves a through-and-through defect of the skin, muscle, and mucosa of the lower aspect of the lip (Fig. 2). An incomplete cleft lip, however, spares some of the superior portion of the upper lip.
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Fig. 1. Patient with left microform cleft lip.
Fig. 3. Patient with right complete cleft lip, palate, and alveolus.
A complete unilateral cleft lip occurs when the deformity involves all layers of the upper lip, and it involves the entire height of the upper lip (Fig. 3). A complete cleft of the primary palate frequently is bridged by a bar of lip tissue, referred to as a Simonart’s bar or band [5]. This connection of soft tissue may lessen the underlying skeletal deformity in complete cleft lips. If disruption of both sides of lip development occurs, a bilateral cleft lip results. In the incomplete bilateral cleft lip, there is usually some skeletal continuity between the premaxilla and the palatal shelves and little or no protrusion of the premaxilla (Fig. 4). In complete bilateral cleft lips, the central premaxilla is totally detached from and protrudes anterior to each maxilla (Fig. 5).
could affect healing and safety. In 1966, Wilhelmsen and Musgrave [6] proposed the preoperative requirements in their ‘‘rule of 10’’: Weight—10 lbs Hemoglobin—10 g White blood cell count—less than 10,000/mm3 In 1957, Millard [7] proposed the ‘‘rule of order 10’’: Weight—over 10 lbs Hemoglobin—over 10 g Age—over 10 weeks
The decision to repair a cleft lip includes consideration of severity of the deformity, size, and health of the child, and other anomalies or problems that
In cases of narrow or moderate width clefts, the general principle of performing cleft lip repair at 3 months of age is a good one; however, several circumstances exist that alter the timing from the usual 3 months of age for definitive lip repair. In patients with associated congenital anomalies, potential airway problems, or other health problems, it is often wise to delay lip repair until an age when general anesthesia becomes safer. Many cleft lip deformities are not amenable to a single-stage
Fig. 2. Patient with right incomplete cleft lip.
Fig. 4. Patient with incomplete bilateral cleft lip and alveolus.
Timing of cleft lip repair
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Fig. 5. Patient with bilateral complete cleft of the lip, alveolus, and palate.
definitive repair. Examples of this include very wide unilateral complete cleft lips or wide complete bilateral cleft lips with a protrusive ‘‘locked out’’ premaxilla. In these cases, the lip adhesion procedure is performed at approximately 3 months of age, with definitive repair being performed at 5 to 6 months of age. The general timing of cleft procedures is outlined in Table 1.
Anatomy of the unilateral cleft lip A unilateral cleft of the upper lip involves alteration in all layers of the lip, including the skin, muscle, mucosa, and underlying skeleton. The external form of the defect is determined by the extent of the underlying muscular and skeletal deformity. The principle muscle of the lips is the orbicularis oris (Fig. 6). The fibers of this muscle encircle the oral orifice within the substance of the lips. The orbicularis oris muscle consists anatomically of two parts: the superficial and the deep layers. This muscle is not a true sphincter, with the superficial and deep
Fig. 6. The minetic muscles of the lower face. Note the insertion of the midface and lower facial muscles into the orbicularis oris muscles of the lip and mouth. (From Sykes J, Senders C. Pathologic anatomy of cleft lip, palate, and nasal deformities. In: Meyers AD, editor. Biological basis of facial plastic surgery. New York: Thieme Medical Publishers; 1993. p. 59; with permission.)
components arising as separate muscles from the modiolus at each oral commissure [8]. In the unilateral cleft lip deformity, there is discontinuity of the orbicularis oris muscle in the region of the cleft (Fig. 7). The muscles of the unilateral cleft lip have two distinct differences when compared with normally developed lip musculature [9]. First, the muscles are hypoplastic in the region of the cleft. Second, the muscles cannot cross the cleft gap and alternately travel along the margin of the cleft. The orbicularis oris muscles are prevented from attaching to their normal sites and find substitute insertions. In complete unilateral cleft lips, it is obvious that no muscle is present in the through and through cleft
Table 1 Timing of cleft repairs Procedure
Age
Cleft lip repair Tip rhinoplasty Tympanostomy tubes Palatoplasty T-tube placement Speech evaluation Velopharyngeal insufficiency workup and surgery (if necessary) Alveolar bone grafting Nasal reconstruction Orthognathic surgery (if necessary)
3 months
9 – 18 months 3 – 4 years 4 – 6 years
9 – 11 years 12 – 18 years At completion of mandibular growth (>16 years)
Fig. 7. The minetic muscles of a patient with left complete cleft of the lip and palate. Note the abnormal insertion of the orbicularis oris muscles, which insert along the long axis of the cleft. (From Sykes J, Senders C. Pathologic anatomy of cleft lip, palate, and nasal deformities. In: Meyers AD, editor. Biological basis of facial plastic surgery. New York: Thieme Medical Publishers; 1993. p. 61; with permission.)
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Fig. 8. The normal vasculature of the lips and nose. The main blood supply of the lips comes from the superior and inferior labial arteries, branches of the common facial artery. (From Sykes J, Senders C. Pathologic anatomy of cleft lip, palate, and nasal deformities. In: Meyers AD, editor. Biological basis of facial plastic surgery. New York: Thieme Medical Publishers; 1993. p. 60; with permission.)
gap. The incomplete cleft lip, however, contains soft tissue, which could contain muscle. Dissections on stillborn babies performed by Fara and colleagues [9] revealed that muscles in unilateral cleft lips were more hypoplastic on the medial side than on the lateral side of the cleft. These dissections also revealed that the muscles in incomplete clefts did not cross the cleft gap unless the skin bridge was at least one third of the height of the lip. When a small amount of muscle is present within an incomplete cleft lip, the orientation of the muscle invading the incomplete cleft skin bridge is still abnormal. The vascular supply to the lip in the unilateral cleft lip deformity is similar to the muscle abnormalities. The superior labial artery, a branch of the facial artery, courses obliquely along the margin of the cleft (Fig. 8). At the base of the nose, the superior labial artery anastomoses with the angular artery or the lateral nasal artery. The blood supply in the unilateral cleft lip deformity is stronger and more developed on the lateral side than it is on the medial side (Fig. 9). In the incomplete cleft lip deformity, a thin terminal branch of the superior labial crosses the skin bridge.
then wrapped with thread in a figure-eight fashion. Rose [11] and Thompson [12] described modification of straight-line closures for repair of the unilateral cleft lip deformity. All of these straight-line techniques closed the cleft defect adequately but often resulted in vertical scar contracture and notching of the lip. Several efforts have since been made to improve the aesthetic and functional results obtained with straight-line closure techniques. These include multiple types of geometric repairs with full-thickness lip flaps. These techniques were designed to irregularize the lip scars and prevent vertical scar contracture and notching of the upper lip. LeMesurier [13] described a quadrilateral fullthickness flap based laterally (from the cleft side). This inferior flap interrupted the scar at the vermilioncutaneous junction. Tennison [14] and Marcks and colleagues [15] introduced a triangular flap, which created a Z-plasty at the lower aspect of the lip. Subsequently, Randall [16] used the same design as Tennison but reduced the size of the triangular flap. In 1957, Millard [7] described the rotationadvancement flap technique for repair of the unilateral cleft lip deformity. This repair creates two major full-thickness flaps and is designed to place cleft scar in the philtral column. The rotation-advancement technique is presently the most commonly used procedure for unilateral cleft lip repair.
Philosophy of the rotation-advancement repair The primary goals of unilateral cleft lip repair are to reconstruct normal lip anatomy and restore lip
History of cleft lip surgery The first documented repair of a unilateral cleft lip was in approximately 390 ad in the Tang dynasty in China. The cleft lip edges were cut and sutured, and the child was instructed not to speak for 100 days. Ambroise Pare [10] repaired a unilateral cleft by freshening the cleft edges and skewering the two sides of the cleft with a long needle. The needle was
Fig. 9. The aberrant vasculature of a patient with a complete left unilateral cleft lip and palate. (From Sykes J, Senders C. Pathologic anatomy of cleft lip, palate, and nasal deformities. In: Meyers AD, editor. Biological basis of facial plastic surgery. New York: Thieme Medical Publishers; 1993. p. 62; with permission.)
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function [17]. Other goals include closure of the nasal floor, correction of nasal tip asymmetry, and narrowing of the alveolar cleft. The rotation-advancement flap technique involves two major full-thickness flaps that can be approximated to repair the cleft without notching of the lip. This design allows reconstitution and reorientation of the orbicularis oris muscles. The advantages and disadvantages of the Millard technique compared with geometric flap techniques are summarized in Table 2. Geometric flap techniques require premeasured geometric flap design. This allows the inexperienced surgeon to study specific flap design and become secure with precise flap shape and measurement. In general, however, geometric flap repair confines the surgeon and allows less flexibility during the surgical procedure. The main advantage of the rotation-advancement technique is its flexibility in application. The Millard procedure is a ‘‘cut as you go’’ technique, allowing continuous modifications during the design, incisions, and execution of the repair. Another advantage of the rotation-advancement technique is that the incisions are designed to place the scar in the new philtral column. Adherence to this known aesthetic subunit allows camouflage of the eventual scar. Most geometric flap design, on the other hand, violates the philtral subunit. The other advantages of the rotationadvancement technique are that minimal normal tissue is discarded and maximal repair of the muscle is accomplished. A disadvantage of the rotation-advancement flap technique is that the inexperienced surgeon may have difficulty without the aid of a premeasured flap design. Additionally, this technique requires extensive undermining and may have a tendency toward creating a small nostril on the cleft side. For this reason, the surgeon using this technique should attempt to make the cleft nostril slightly larger than the nostril on the noncleft side. It is easier to correct a large nostril than it is to repair nostril stenosis.
Table 2 Millard rotation-advancement repair Advantages
Disadvantages
Flexible Minimal tissue discarded Good nasal access Camouflaged suture line
Requires experienced surgeon Possible excessive tension Extensive undermining required Vertical scar contracture Tendency toward small nostril
From Ness JA, Sykes JM. Basics of Millard rotationadvancement technique for repair of the unilateral cleft lip deformity. Facial Plast Surg 1993;9:168; with permission.
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Fig. 10. The important reference points of the Millard rotation-advancement technique for repair of the unilateral cleft lip deformity. (From Ness JA, Sykes JM. Basics of Millard rotation-advancement technique for repair of the unilateral cleft lip deformity. Facial Plast Surg 1993;9:169; with permission.)
Measuring and design of incision The important reference points of the technique are shown in the following list and in Fig. 10. 1. Center (low point of Cupid’s bow—noncleft side) 2. Peak of Cupid’s bow—lateral noncleft side 3. Peak of Cupid’s bow—medial noncleft side 4. Alar base—noncleft side 5. Columellar base—noncleft side 6. X Back-cut point—noncleft side 7. Commissure—noncleft side 8. Commissure—cleft side 9. Peak of Cupid’s bow—cleft side 10. Medial tip of advancement flap—cleft side 11. Midpoint of alar base—cleft side 12. Lateral alar base—cleft side These points should be measured and marked before the infiltration of local anesthesia. This will prevent ballooning and distortion by the anesthetic/vasoconstrictive agent. Various measurements can be made to ensure accurate marking of the skin points. The following is a list of measurements for flap design: 1 2 2 3
to to to to
2 6 4 5
= = = +
1 8 8 x
to 3 = 2 – 4 mm to 7 = 20 mm to 10 = 9 – 11 mm = 8 to 9
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raised in the supraperiosteal dissection plane. A small skin flap, flap c, is the byproduct of the rotation incision at the columellar base, and it is elevated in the subcutaneous plane. The D flap is an alar flap, which is created by the perialar portion of the advancement incision. The designation for the skin and mucosal flaps is summarized in Fig. 12 and in the following list: A = rotation flap B = advancement flap c = columellar base soft tissue, noncleft side D = alar rim, cleft side m = medial mucosal flap l = lateral mucosal flap
Fig. 11. (A) Lengths of the rotation flap (3 to x) and the advancement flap (8 to 9). Note that the lengths of the advancement flap should always equal the length of the rotation flap. (B) Use of the paper clip technique to measure the curved rotation flap as being equal in length to the straight advancement flap. (From Ness JA, Sykes JM. Basics of Millard rotation-advancement technique for repair of the unilateral cleft lip deformity. Facial Plast Surg 1993; 9:171; with permission.)
The capital letter flaps A, B, and D are full-thickness flaps, whereas the small letter flaps c, m, and 1 are thin subcutaneous or submucosal flaps. The cutaneous c flap may be discarded or used to create the nasal floor. The mucosal m and 1 flaps may be discarded or used to help create an intraoral sulcus. After elevation of the major rotation and advancement flaps, an external alotomy is performed
These measurements are performed to add precision to the skin markings for the advancement flap and the rotation flap. The most important measurement is that the length of the rotation flap (3 to 5 + x) equals the length of the advancement flap (8 to 9) (Fig. 11). Although this measurement is often performed subjectively, objective analysis of the relative lengths can be made with a 26-gauge wire. The wire is first bent to correspond to the length of the rotation flap and then is straightened to measure the advancement flap. If these lengths are not equal, modification in the skin markings is made to equalize the two flap lengths. Incision and flap elevation The cutaneous incisions of the rotation and advancement flaps are made with a 15c-knife blade. Mucosal incisions are made with an 11-knife blade. The mucosal incisions create two thin mucosal flaps (m = medial, 1 = lateral). These flaps are raised in the submucosal plane. The major lip flaps (A = rotation, B = advancement) are full-thickness flaps and are
Fig. 12. (A) The major flaps of the Millard unilateral cleft lip repair. (From Ness JA, Sykes JM. Basics of Millard rotation-advancement technique for repair of the unilateral cleft lip deformity. Facial Plast Surg 1993;9:171; with permission.) (B) Intraoperative view of a patient after marking of the flaps for repair.
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determine if closure can be accomplished easily (Fig. 14). If too much tension on the closure exists, further dissection and undermining is performed to decrease wound tension. Active placement of the alar base
Fig. 13. Intraoperative view of a patient after elevation of the major rotation and advancement flaps before performance of an alotomy.
(Fig. 13). An internal alotomy is then accomplished to free the alar flap from the piriform aperture. Elevation of the skin and mucosa off of the orbicularis oris muscle is then performed. Lastly, dissection over the nasal tip is accomplished through the perialar and columellar base incisions. After all elevation is completed, single-pronged hooks are placed at the vermilion border of each lip flap to
After the alar flap is freed, active placement of the alar base is performed [18]. The width of the cleft side alar base should be slightly greater than that of the noncleft side to prevent nostril stenosis after wound healing. Closure of the nasal floor is accomplished with 4-0 chromic catgut sutures. Muscle closure It is important to isolate and precisely reconstruct the orbicularis oris muscles. This will relieve tension and make the lip function better. Careful muscle closure will also provide appropriate volume to the lip and prevent inversion (depression) of the cleft lip scar. The muscle is reapproximated with a 4-0 long-acting monofilament absorbable suture (Fig. 15). These muscle sutures are the strength of the lip closure. Skin closure After the alar base position is established and the orbicularis muscle is reconstituted, closure of the oral mucosa and the epithelium of the lip is performed. Intraoral mucosal incisions are closed with
Fig. 14. (A) Use of single-pronged hooks to test the advancement and rotation of the major flaps in the unilateral cleft lip repair. (From Ness JA, Sykes JM. Basics of Millard rotation-advancement technique for repair of the unilateral cleft lip deformity. Facial Plast Surg 1993;9:173; with permission.) (B) Intraoperative view depicting the use of this technique to assess tension on the repair.
Fig. 15. Closure of the orbicularis oris muscle before skin closure in the cleft lip repair. (From Ness JA, Sykes JM. Basics of Millard rotation-advancement technique for repair of the unilateral cleft lip deformity. Facial Plast Surg 1993;9:174; with permission.)
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5-0 chromic catgut sutures. The m and 1 mucosal flaps may be used to provide tissue to close the sublabial sulcus. Trimming of the mucosa before closure is usually necessary to provide the precise contour and volume to the vermilion of the lip. The external skin is approximated with 6-0 monofilament and 6-0 fast-absorbing gut sutures. Key sutures of 6-0 monofilament nonabsorbable sutures are placed at the base of the nose and at the vermilion-cutaneous junction. Tip rhinoplasty Undermining of the nasal tip is performed by dissecting the external skin from the underlying lower lateral cartilages (Fig. 16) [19]. The vestibular skin is not dissected from the underside of the alar cartilages to minimize the risk of circumferential alar (nostril) stenosis. After freeing the nasal skin from the lower lateral cartilages, tip rhinoplasty is performed. The goal of primary cleft rhinoplasty in the unilateral deformity is to improve nasal tip symmetry, definition, and projection. To minimize the chance of nasal growth inhibition, the lower lateral cartilages are not removed or incised. Instead, repositioning of the cartilages is accomplished with bolster sutures (Fig. 17). This corrects malpositioned cleft side lower lateral cartilage. If properly performed, primary cleft lip rhinoplasty will lessen the primary deformity and decrease the need for intermediate cleft rhinoplasty. Bilateral cleft lip repair The complete bilateral cleft deformity consists of the two lateral lip segments and a midline prolabium overlying the premaxilla. There are several key anatomical differences between the unilateral and bilateral cleft lip deformities that must be considered
Fig. 17. Intraoperative view after repositioning of the cleft lower lateral cartilages with bolster sutures.
before repairing the bilateral cleft lip (Table 3). Similar to the lateral lip segment in the unilateral cleft lip deformity, the aberrant orbicularis oris muscle insertions of the two lateral lip segments course superiorly toward the alar base. However, the central prolabium never contains muscle. Varying degrees of anterior premaxillary projection as well as rotation can occur. Some surgeons advocate either lip adhesion techniques or staged one-sided repairs to posteriorly reposition the premaxillary segment. Others argue that staged repairs can create asymmetry. This article describes a single-stage bilateral cleft lip repair and addresses the unique characteristics of the bilateral cleft lip deformity. Measurement and design First, the midline on the vermillion-cutaneous junction of the prolabial flap is marked. The philtral width, which is expected to widen with growth, is measured using a caliper to mark 2 to 2.5 mm on each side of the midline (total philtral width: 4 – 5 mm). Vertical markings are created to extend up to just superiomedial to the collumellar base. The lateral vermillion borders are then marked to create the prolabial forked flaps. Table 3 Keys to bilateral cleft lip repair
Fig. 16. lntraoperative view showing undermining of the nasal tip during cleft lip rhinoplasty. Note that the undermining occurs over the medial and lateral aspect of the lower lateral cartilages.
Characteristic
Surgical approach
Premaxilla may be ‘‘locked out’’ Prolabium contains no muscle
Lip adhesion or lip repair
Orbicularis oris muscle inserts superiorly Lateral lip has more volume than prolabium Wide, maloriented nasal tip, short collumella
Lateral muscle sutured at midline (Concentric orbicularis oris ring) Reoriented muscle under prolabium Mucosal flaps add volume Tip rhinoplasty
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intraoral sublabial incisions are extended intraorally to release the cheek – lip complex from the maxilla, with care to preserve the infraorbital neurovascular bundles. Lastly, the lateral lip advancement skin flaps are conservatively undermined from the reoriented orbicularis oris muscle fibers. The malpositioned lower lateral cartilages, including the shortened medial crus, are freed from the skin soft tissue envelope (Fig. 16). Fig. 18. Intraoperative view showing the marked prolabial flap (p) and forked flaps (f). The lateral lip advancement flaps (a) and mucosal flaps (m) have been elevated. The vertical heights of the prolabial and lateral lip advancement flaps are equal.
Next, the lateral lip markings are designed to approximate the vertical height of the prolabial flap. This vermillion marking begins where the mucosa begins to thin superior-medially. Marking and design of the mucosal flaps is similar to those in unilateral cleft repairs (Fig. 18). Bilateral sublabial incisions were designed to extend from the mucosal flaps to allow release of the cheek – lip complex from the maxilla. Incision and flap elevation With the prolabial flap incised and elevated, the inferiorly based prolabial mucosa is sutured under the prolabial flap to create a labioalveolar sulcus. The forked flaps are incised and elevated in a subcutaneous plane to be rotated laterally into the floor of nose as needed. Next, the mucosal flaps on the lateral lip segments are elevated with a conservative backcut into the nasal-alar groove (Fig. 19). The lateral
Fig. 19. Intraoperative view of the advanced mucosal flaps before elevation of the prolabial flap. The muscle layer closure will be performed anterior to the premaxilla, but deep to the prolabial flap. This minimizes tension on the skin closure and creates a concentric ring of orbicularis oris.
Nasal floor closure and alar base width The lateral lip segments’ mucosal closure over the premaxilla, but under the prolabial flap, is performed with 5-0 chromic catgut sutures. The forked flaps can be rotated laterally to add to the nasal floor closure, or these flaps can be discarded. Release of the lateral nasal wall mucosa can help obtain nostril size symmetry. The alar base width is actively placed with a 4-0 monofilament absorbable suture. Orbicularis oris closure The orbicularis oris muscle should be reoriented over the premaxilla to create a concentric muscular ring, as well as to prevent dynamic inferior displacement of the alar base with muscle contraction. The ‘‘key suture’’ (4-0 long-acting monofilament absorbable) is placed at the junction of the white and red lip to create vertical height symmetry and lip fullness. Skin closure After the mucosal and muscle approximation are completed, the Cupid’s bow is created by aligning the prolabial flap vermillion with the lateral lip vermillion using a permanent 6-0 monofilament suture (Fig. 20). Often, a back-cut at the vermillion border of the lateral lip segment is helpful. Other than the vertical lip height and horizontal symmetry, the third dimension to be considered must be lip fullness
Fig. 20. Intraoperative view of the final closure before lower lateral cartilage bolster placement.
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Fig. 21. (A) Intraoperative photograph of a 3 month old patient with a bilateral cleft lip and palate deformity. (B) Eight-month postoperative photograph of the same patient. (C) Ten-year postoperative photograph of the same patient.
(Fig. 21). A mucosal z-plasty can be used to distribute lip volume and prevent a ‘‘whistle’’ deformity that can be seen with central mucosal lip deficiencies.
with 4-0 permanent sutures. The shortened collumella can be addressed in subsequent surgeries if necessary.
Tip rhinoplasty The nasal tip skin elevation is performed before lip closure to allow the lower lateral cartilages to be repositioned. After lip closure is completed, the lower lateral cartilages are sutured in a more cephalic and medial position using two to four bolsters secured
Summary Repair of the unilateral cleft lip deformity is a challenging and rewarding procedure. Historically, many techniques have been described to reconstruct the unilateral cleft lip. These have included straight-
Fig. 22. (A) Preoperative view of a patient with a right complete unilateral cleft lip. (B) The same patient 3 months after repair of the cleft lip with the Millard rotation-advancement technique.
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line repairs and various geometric flap closures. The rotation-advancement flap technique of Millard is a reliable and versatile method for repair of the unilateral cleft lip deformity. This technique allows lip repair and tip rhinoplasty while camouflaging the scars in the newly formed philtral border. If properly applied, the rotation-advancement repair produces excellent functional and aesthetic results (Fig. 22).
References [1] Enlow DH. Facial growth. 3rd edition. Philadelphia7 WB Saunders; 1990. [2] McCarthy JG. Plastic surgery: cleft lip and palate and craniofacial anomalies, volume 4. Philadelphia7 WB Saunders; 1990. [3] Sykes JM, Senders CW. Facial plastic surgery: cleft lip and palate, volume 9. New York7 Thieme Medical Publishers; 1993. [4] Gaare JD, Langman J. Fusion of nasal swellings in the mouse embryo: surface coat and initial contact. Am J Anat 1977;150:461 – 75. [5] Simon G. Ueber die Uranoplastik mit besonderer Beruecksichtigung der Mittel zur Wiederserstellung einer reinen Sprache. Greifswalder Med Beitr 1866; 2:129 [in German]. [6] Wilhelmsen HR, Musgrave RH. Complications of cleft lip surgery. Cleft Palate J 1966;3:223 – 31. [7] Millard Jr DR. A primary camouflage of the unilateral harelook. Transactions of the 1st International Congress of Plastic Surgery, Stockholm. Baltimore (MD)7 Williams & Wilkins; 1957.
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[8] Burkitt AN, Lightoller GHS. The facial musculature of the Australian aboriginal. J Anat 1928;62:33 – 57. [9] Fara M, Chlumska A, Hrivnakova J. Musculis orbicularis oris in incomplete hare-lip. Acta Chir Plast 1965; 7:125 – 32. [10] Pare A. Dix livres de la chirurgie. Paris7 Jean Le Roger; 1564 [in French]. [11] Rose E. Udber den plastischen Ersatz des harten Gaumens aus der Lipp. Arch Klin Chir 1879;24:438 [in German]. [12] Thompson JE. An artistic and mathematically accurate method of repairing the defect in cases of harelip. Surg Gynecol Obstet 1912;14:498 – 505. [13] LeMesurier AB. Method of cutting and suturing lip in complete unilateral cleft lip. Plas Reconst Surg 1949; 4:1. [14] Tennison CW. The repair of the unilateral cleft lip by the stencil method. Plast Reconst Surg 1952;9:115 – 20. [15] Marcks KM, Trevaskis AE, DaCosta A. Further observations in cleft lip repair. Plast Reconstr Sug 1953;12:392. [16] Randall P. A triangular flap operation for the primary repair of unilateral clefts of the lip. Plast Reconstr Surg 1959;23:331. [17] Ness JA, Sykes JM. Basics of Millard rotationadvancement technique for repair of the unilateral cleft lip deformity. Facial Plast Surg 1993;9:167 – 76. [18] Sykes JM, Senders CW. Surgical treatment of the unilateral cleft nasal deformity at the time of lip repair. Facial Plast Surg Clin North Am 1995;3:1. [19] Sykes JM, Senders CW. Surgery of the cleft lip nasal deformity Operative techniques in otolaryngologyhead and neck surgery, volume 1. Philadelphia7 WB Saunders; 1990.