CME Primary Repair of Cleft Lip and Nasal Deformity Laura A. Monson, M.D. Richard E. Kirschner, M.D. Joseph E. Losee, M.D. Pittsburgh, Pa.; and Columbus, Ohio

Learning Objectives: After studying this article, the participant should be able to: 1. Describe the relevant anatomy and embryology that contribute to the cleft lip and nasal deformity. 2. Differentiate among the various forms of cleft lip, including microform, minor form, incomplete, complete, and bilateral. 3. List the options for presurgical infant orthopedics. 4. Describe the techniques and the expected outcomes of primary cleft lip and nasal repair. Summary: The authors describe cleft lip and its surgical management. They address the anatomy, forms, techniques, and outcomes of this complex and challenging deformity.  (Plast. Reconstr. Surg. 132: 1040e, 2013.)

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o study the evolution of surgical techniques described to repair the cleft lip deformity is to witness a catalogue of the plastic surgeon’s struggle to conquer what may rightfully be considered the ultimate challenge in the discipline: to combine reconstructive principles with a keen aesthetic sense to restore both form and function, all while anticipating the seemingly uncontrollable influence of cicatrix and growth. The history of cleft lip repair is also a remarkable success story of the plastic surgeon’s quest to restore that which Millard termed the “ideal beautiful normal.” Improved techniques have begotten better outcomes, and no longer must the cleft-affected child fear a life of stigmatization. Still, significant challenges remain, and cleft surgeons continue to refine their methods. The final chapter has yet to be written. More than in any other area in plastic surgery, cleft care is truly team care. There is no place for surgical vainglory in the care of the cleft-affected child; the cleft surgeon must assume his or her rightful position as but one member of an interdisciplinary health care team to restore that which nature has taken away. However, for the cleft surgeon, the discipline has rewards that are beyond compare: to know the satisfaction and the privilege of following the physical and emotional growth and development of his or her patients—from birth through young adulthood—and, in the most literal sense, to build hope while building smiles. From Children’s Hospital of Pittsburgh, University of Pittsburgh; and Nationwide Children’s Hospital, The Ohio State University. Received for publication March 16, 2012; accepted July 30, 2012. Copyright © 2013 by the American Society of Plastic Surgeons DOI: 10.1097/PRS.0b013e3182a808e6

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This article addresses the subject of cleft lip and its surgical management. As in other areas of plastic surgery, ours is but one approach to a complex challenge. It is our hope that the reader will take away some fundamental truths while reading with a critical eye, asking questions while learning some answers and keeping an open mind to envision that which may be the contents of this story’s final chapter.

BACKGROUND Cleft lip with or without cleft palate is the most common facial birth defect in the world, with an incidence ranging from 0.2 to 2.3 per 1000 births. The incidence of cleft lip demonstrates variation across race and sex, with an incidence as high as one in 450 among Asian and Native American populations, one in 1000 among Caucasian populations, and one in 2000 among African American populations. Boys are twice as likely to be affected as girls. Unilateral cleft lip occurs twice as frequently on the left side than on the right and is nine times more common than bilateral cleft lip. Of newborns born with cleft lip and palate, 29 percent present with associated visceral anomalies or external malformations.1–4 Van der Disclosure: The authors have no financial interest to declare in relation to the content of this article. Related Video content is available for this article. The videos can be found under the “Related Videos” section of the full-text article, or, for Ovid users, using the URL citations published in the article.

www.PRSJournal.com

Volume 132, Number 6 • Primary Repair of Cleft Lip and Nose Woude syndrome, an autosomal dominant disorder associated with congenital lip pits, is the syndrome most frequently associated with cleft lip, accounting for 2 percent of all cases. The disorder has been mapped to a mutation in the interferon regulatory factor 6 (IRF6) gene.5–7 Other genes that have shown linkage to cleft lip and palate include MSX1, located on chromosome 4p16.1, and TBX22, located on Xq12-q21.8–11 Many risk factors have been associated with the development of nonsyndromic cleft lip, the most common of which is family history. The risk of cleft lip with or without cleft palate in first-degree relatives is approximately 4 percent.12–14 Several maternal factors have been associated with an increased incidence in orofacial clefting, with the most well substantiated of these being the anticonvulsant phenytoin and maternal smoking.15–19

EMBRYOLOGY Formation of the upper lip begins during the fourth week of gestation and is completed by the seventh week. The nostrils form first when the medial nasal processes fuse with the lateral nasal processes. The large nostril openings become smaller and more medial and vertically oriented as the maxillary prominences grow and push forward. The upper lip is finally formed when the maxillary prominences fuse with the medial nasal prominences. The medial nasal prominences form the intermaxillary segment when they fuse in the midline. From this intermaxillary segment, the nasal tip, columella, philtrum, labial tubercle, frenulum, and primary palate are derived. The Cupid’s bow and philtrum of the upper lip do not form until much later, in the third to fourth month of gestation.13,20,21

ANATOMY The orbicularis oris is the main intrinsic muscle of the mouth. Within the upper lip, the paired halves of the orbicularis oris arise from the modioli. In the normal lip, superficial fibers of the orbicularis oris decussate at the midline and insert into the skin in the region of the philtrum, whereas deeper fibers anchor the muscle to the underlying anterior nasal spine. The orbicularis oris comprises a marginal division (pars marginalis) that courses deep to the vermilion and acts to evert the lip margin, and a peripheral division (pars peripheralis).22–24 The blood supply to the upper lip is derived from the facial artery by means of the superior labial artery. The facial artery passes superficial to the buccinator and deep to the zygomaticus major, and then gives rise to the inferior and superior labial arteries

before it continues along the nasolabial groove as the angular artery. The superior labial artery runs within the substance of the orbicularis oris and gives rise to the paired columellar arteries.25 The philtrum of the upper lip has several distinctive anatomical features. It comprises the paired lateral philtral ridges and the central midline dimple. The philtral ridges are formed primarily by the insertion of the orbicularis oris fibers into the skin in this region, with some contribution from insertions of the musculus nasalis and levator labii superioris. The philtral dimple is believed to be formed by the underlying decussation of the orbicularis oris.22,26 The unilateral cleft lip deformity extends beyond the lip itself to affect the nose and the underlying maxilla in proportion to the degree of the cleft. If the cleft includes the underlying alveolus and palate, there is an outward rotation of the medial element and a posterolateral displacement of the lesser segment lateral element. The anterior nasal septum is displaced out of the vomerine groove into the noncleft nostril. The columella is short on the cleft side. The alar cartilage is malformed and hypoplastic, with a shortened medial crus and an elongated, S-shaped lateral crus that is displaced inferiorly and posteriorly. The nasal tip is widened and asymmetric; the cleft nostril is retropositioned and oriented horizontally. The orbicularis oris on the noncleft side attaches in abnormal fashion to the anterior nasal spine, the footplates of the medial crura, and the anterior nasal septum. Its unopposed pull allows the medial element to rotate anteriorly, and deviates the nasal septum and columella to the noncleft side. On the cleft side, the orbicularis oris inserts abnormally along the nostril sill and the periosteum of the piriform aperture. This pulls the alar base laterally and causes the nostril to be more horizontally oriented. In contrast, the pars marginalis, located in the vermilion, is displaced minimally, does not form abnormal attachments, and terminates in the mucosa (Reference 29, Level of Evidence: Therapeutic, IV).27–32 In the complete bilateral cleft lip, the premaxilla is not attached to the lateral palatal shelves. Because of septal cartilage growth and fetal tongue movements, the premaxilla is unrestrained in its forward growth. The anterior nasal spine can be small or absent, and the lateral piriform apertures are displaced posteriorly.33,34 The lower lateral cartilages are displaced laterally and posteriorly, and the domes of the lower lateral cartilages are widely splayed.35–37 The prolabium does not contain muscle and often has an indistinct white roll.30

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Plastic and Reconstructive Surgery • December 2013 CLASSIFICATION Both unilateral and bilateral cleft lip deformities demonstrate variable expression, with microform, minor, incomplete, and complete forms. Microform cleft lip is the proper term for the anomaly previously classified as “occult,” “minor,” or “forme fruste.” It represents the most minor of the cleft lip deformities and manifests as a vermilion notch that is less than 3 mm in height compared with the Cupid’s bow of the noncleft side, without nasal deformity.38 A minor cleft lip is characterized by a vermilion notch of greater than 3 mm, a vertical depression above the notch into the nasal sill, a variable degree of nasal deformity, and possibly a cleft of the alveolus.38 An incomplete cleft lip has a variable degree of separation but by definition has an intact nasal sill, commonly referred to as a Simonart band. A complete cleft lip extends through the nasal sill and floor and may or may not extend to the underlying alveolus and palate. A bilateral cleft lip can present as a combination of any of the above.39

alveolar segments. The most advanced method of presurgical infant orthopedics is a combination of a palatal appliance with nasal molding. In the method described by Liou et al., nasal molding is carried out simultaneously with reapproximation of the alveolar segments (Reference 41, Level of Evidence: Therapeutic, IV).41,42 The method used by Grayson et al. approximates the alveolar segments before initiating nasal molding.43–45 Both methods require close follow-up, with adjustments performed every 1 to 2 weeks by a dedicated pediatric dentist or orthodontist46 (Figs. 1 through 3). The burden of care may render nasoalveolar molding impractical for many infants with complete clefts. In such cases, surgical lip adhesion may be a useful alternative. In 1965, Randall published his preferred method using broad triangular flaps, and several modifications have

PREOPERATIVE ASSESSMENT The cleft-affected infant should be assessed as soon as possible after birth so that the family can be counseled, the infant’s feeding and growth can be assessed, and upcoming surgical procedures can be planned for. This evaluation should involve a multidisciplinary approach including the plastic surgeon, speech pathologist, feeding specialist, otolaryngologist, social worker, geneticist, and pediatrician. The initial evaluation should aim to identify any associated syndromes and possible comorbidities. At the first visit, the anatomy of the cleft should be assessed; photographs obtained; and a determination made of whether the parents will begin taping, whether presurgical molding will be initiated, or whether the child will require a lip adhesion before formal cleft lip repair.

Fig. 1. Bilateral cleft lip before nasoalveolar molding.

PRESURGICAL INFANT ORTHOPEDICS Many different approaches exist for the presurgical care of the cleft-affected child, with the selected approach depending on the surgeon’s preference and the medical and family resources available. The simplest method of presurgical infant orthopedics is lip taping, which may be started soon after birth. The goal of taping is to bring the alveolar segments into closer approximation.40 External taping can also be combined with the use of a palatal plate, which may be sequentially modified to guide movement of the

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Fig. 2. Bilateral cleft lip before nasoalveolar molding.

Volume 132, Number 6 • Primary Repair of Cleft Lip and Nose A bilateral lip adhesion is performed in a similar manner. All of the markings for a definitive repair are performed. Rectangular mucosal flaps are raised high on both lateral lip elements and on the prolabium. Bilateral buccal sulcus incisions are carried around the piriform aperture, and the lateral lip elements are freed from the maxilla so that they may be readily brought into approximation with the prolabial flaps. A permanent retention suture is placed, encircling the orbicularis oris of both lateral elements and crossing the prolabium (Fig. 5). Placement of silicone nostril conformers at the time of unilateral or bilateral lip adhesion serves to lengthen the columella and to reshape the lower lateral cartilages before definitive lip repair.48

UNILATERAL CLEFT LIP AND NOSE REPAIR

Fig. 3. (Above) Same patient following nasoalveolar molding. (Below) Same patient following nasoalveolar molding.

since been described.29,47 When performing a lip adhesion, the anticipated markings for the definitive cleft repair are first placed on the lip to ensure that all incisions for the adhesion are kept within the tissues that will be discarded at the time of repair. Rectangular mirror-image flaps are designed on the medial and lateral lip segments just beneath the nasal sill. On both the lateral and the medial lip elements, the incisions are made with an ophthalmic blade, and the flaps are elevated off of the underlying orbicularis oris. An upper buccal sulcus incision is made on the lateral element and carried around the piriform rim to the inferior turbinate. The ala and cheek are released from the underlying maxilla and piriform rim in the supraperiosteal plane to allow for tension-free adhesion of the lip elements. After full mobilization, a permanent retention suture is placed, encircling the orbicularis oris on each side of the cleft. The muscle and mucosal layers are then approximated with absorbable suture48 (Fig. 4).

Although descriptions of cleft lip, and cleft lip repair, can be found in ancient medical texts, the modern concept of cleft lip repair began with the straight-line repairs of Rose in 1891 and Thompson in 1912.49 Recognition that straight-line techniques often failed to adequately level the Cupid’s bow led to the development of techniques designed to introduce tissue from the lateral element into the foreshortened edge of the medial element. These repairs included the Randall-Tennison triangular flap repair and the quadrangular flap repair of Le Mesurier and Hagedorn.50–52 Triangular flap repairs predominated into the 1960s, and variations, such as the Fisher repair, remain popular today.53 The early repairs, however, suffered from the disadvantage of violating the philtral segment with unnatural scars and, in the case of the quadrangular flap, of occasionally producing a long lip. Moreover, these techniques offered little intrinsic correction of the cleft nasal deformity. In 1957, Millard described his rotationadvancement technique in which the Cupid’s bow was rotated down into position, with the high rotation gap being filled by a flap of skin advanced from the lateral element. The technique addresses the vertical height discrepancy of the lip and preserves the anatomy of the Cupid’s bow and philtrum. This method also provides for correction of the laterally displaced alar base. Although several modifications to the rotation advancement technique have been described, Millard’s basic principles provide the foundation for the majority of cleft lip repairs performed today, and a modified version of the rotation-advancement repair is the authors’ preferred method.54,55

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Fig. 4. (Above, left) Markings for a unilateral lip adhesion. (Below, left) Intraoperative unilateral lip adhesion demonstrating approximation of deep tissues. (Right) Intraoperative unilateral lip adhesion demonstrating skin closure.

The primary goals of cleft lip repair are to produce functional continuity of the orbicularis oris muscle, recreate symmetry, reconstitute the Cupid’s bow, produce a slight pout of the tubercle, balance the vermilion, and achieve nasal symmetry. The benefits of the rotation-advancement repair are that it preserves the Cupid’s bow and does not violate the philtral segment. It can be difficult to perform well, however, in clefts with a severe vertical height discrepancy. Early scar contraction may shorten the lip, although this may settle out with maturation of the scar in some cases. Markings are placed on the lip with methylene blue. First, the nadir of the Cupid’s bow is marked; then, the height of the bow on the cleft and noncleft sides of the medial element is marked, and the distance of each from the nadir

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point is checked for symmetry. The Cupid’s bow peak on the lateral lip element is marked next. This point should be placed at a point where the vermilion is fullest and there is a well-developed white roll. The distance between the alar base and the Cupid’s bow point selected on the lateral element should equal that between the alar base and the Cupid’s bow peak on the noncleft side of the medial element to ensure equal lip length. The advancement flap from the lateral lip element is then drawn along the edge of the vermilion, then along the nasal sill, without going beyond the alar base. Limiting the length of the sill incision assists in correction of alar flare and limits scarring. The rotation incision is then marked on the medial element from the Cupid’s bow peak on the cleft side, along what will be the cleft-side philtral column, up to the base of the columella, then curves

Volume 132, Number 6 • Primary Repair of Cleft Lip and Nose

Fig. 5. (Above) Markings for a bilateral lip adhesion. (Below) Intraoperative bilateral lip adhesion demonstrating close approximation of the segments.

gently along the columella-lip junction, extending slightly downward just medial to the philtral column on the noncleft side. The amount of back-cut required can be determined later and should be limited to the smallest measure necessary to level the Cupid’s bow. Care should be taken to ensure that the length of the rotation incision is at least as long as the leading edge of the advancement flap. If the latter is too long, the excess can be trimmed at the time of flap inset. A small triangular flap of skin may be taken from the lateral element and inset into a back-cut above the white roll of the medial element. This provides for a little extra length if required to balance the Cupid’s bow. The C flap is marked, and the cardinal points are tattooed once all markings have been checked for proper placement (Fig. 6). The lip and nasal tip are then infiltrated with local anesthetic containing epinephrine.56 Once the vasoconstrictive effect of the epinephrine is complete, the skin is incised with an ophthalmic blade, and the muscle is dissected carefully from the underlying mucosa and from the overlying skin for a short distance (Fig. 7). Care is taken to avoid dissecting the muscle past the midline of the philtrum, to preserve the philtral dimple. High on the medial element, the muscle is dissected free from its abnormal attachments to the septum, nose, and maxilla until it can be rotated freely. The back-cut may be extended at this time if, after full mobilization, the peaks of the Cupid’s bow are not level on a horizontal plane. The C flap is dissected free, taking care to leave an adequate base. The caudal nasal septum

Fig. 6. (Left) Diagram of unilateral cleft lip markings. (Right) Intraoperative markings of unilateral cleft lip repair (method of J.E.L.).

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Fig. 7. (Left) Diagram of unilateral cleft lip skin and muscle incisions. (Right) Diagram of unilateral cleft lip muscle dissection.

is then exposed and can be released from the anterior nasal spine and medialized. Following dissection of the advancement flap, an incision is carried along the upper buccal sulcus of the lateral element and along the piriform aperture on top of the inferior turbinate. The abnormal muscle attachments along the piriform aperture and alar base are released, and the ala-cheek-lip complex is elevated in the supraperiosteal plane. The piriform attachments of the lower lateral cartilage are released, and dissection continues until the entire ala-cheek-lip complex can be repositioned easily. From this pocket, the lateral crus may be dissected free from the overlying skin. Following repair of the mucosa, the pars peripheralis of the orbicularis oris is approximated with a series of interrupted horizontal mattress sutures, taking care to evert the muscle edges to recreate the philtral column (Fig. 8). The pars marginalis is repaired separately with simple sutures to avoid notching of the

Fig. 8. Diagram of the preferred method of muscle suturing.

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vermilion. At this point, the skin edges should be lying in close approximation, and adequate rotation of the Cupid’s bow should be achieved. The advancement flap is inset with buried sutures, proceeding superiorly from the Cupid’s bow. Any skin excess may be trimmed from the superior edge of the advancement flap at this time. Care is taken to precisely align the skin edges, obviating the need for sutures through the epidermis. The vermilion, often narrower beneath the Cupid’s bow on the cleft side, may be evened through inset of a triangular vermilion flap from the lateral element into a back-cut within the red line of the medial element. The C flap is then trimmed and sutured into place, lengthening the columella on the cleft side and reconstructing the medial nasal sill (Fig. 9, left). The nasal deformation may be corrected with a semiopen technique using the lip incisions or an alar rim incision. The overlying skin is dissected from the tip cartilages under direct vision. Excess fibrofatty tissue between the domes of the lower lateral cartilages is released and allowed to remain with the overlying skin. The lower lateral cartilage on the cleft side is repositioned, narrowing the angle of domal divergence using mattress sutures. If necessary, the lower lateral cartilage can be suspended to the contralateral upper lateral cartilage. The incisions are closed carefully with absorbable interrupted suture55–60 (Fig. 9, right). (See Video, Supplemental Digital ­ Content 1, which displays repair of a primary cleft lip, available in the “Related Videos” section of the fulltext article on PRSJournal.com or, for Ovid users, at http://links.lww.com/PRS/A914; and Video, Supplemental Digital Content 2, which displays

Volume 132, Number 6 • Primary Repair of Cleft Lip and Nose

Fig. 9. (Left) Diagram of unilateral cleft lip skin closure. (Right) Intraoperative unilateral cleft lip repair (method of J.E.L.).

Video 1. See Videos, Supplemental Digital Content 1, which displays repair of a primary cleft lip, available in the “Related Videos” section of the full-text article on PRSJournal.com or, for Ovid users, available at http://links.lww.com/PRS/A914.

Video 2. Supplemental Digital Content 2, which displays continued repair of a primary cleft lip, available in the “Related Videos” section of the full-text article on PRSJournal.com or, for Ovid users, available at http://links.lww.com/PRS/A915.

continued repair of a primary cleft lip, available in the “Related Videos” section of the full-text article on PRSJournal.com or, for Ovid users, at http:// links.lww.com/PRS/A915.)

methylene blue on the prolabium and lateral elements and checked for symmetry. The width between the points of the Cupid’s bow is set at 4 to 5 mm, and the vertical limbs are drawn as curved lines that narrow to a width of 3 to 4 mm at the columellar base. The peaks of the Cupid’s bow on the lateral lip segments are marked at the point where the vermilion is fullest and the white roll is distinct (Fig. 10). The tubercle of the vermilion is reconstructed from vermilion flaps derived from the lateral elements, as the prolabial vermilion is nearly always deficient and its white roll absent or indistinct.61,62 The prolabium is incised along the standard markings with an ophthalmic blade, with care

BILATERAL CLEFT LIP AND NOSE REPAIR The primary objectives of bilateral cleft lip repair are to create a balanced Cupid’s bow, repair the orbicularis oris, create an upper labial sulcus, reduce alar flare and reconstruct the nasal sill, restore columellar length, correct the malposition of the lower lateral cartilages, and restore nasal tip projection. Landmarks are marked with

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Plastic and Reconstructive Surgery • December 2013

Fig. 10. (Left) Diagram of bilateral cleft lip markings. (Right) Bilateral markings (method of J.E.L.).

Fig. 11. Diagram of bilateral cleft lip incisions.

Fig. 13. Diagram of bilateral cleft lip muscle dissection.

Fig. 12. Diagram of bilateral cleft lip sulcus creation.

Fig. 14. Diagram of bilateral cleft lip muscle repair.

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Volume 132, Number 6 • Primary Repair of Cleft Lip and Nose

Fig. 15. (Above, left) Bilateral cleft lip following muscle repair, before skin closure, showing markings for nasal repair (this is the same patient shown in Figs. 1 through 3) (method of J.E.L.). (Below, left) Bilateral cleft lip (base view). (Right) Bilateral cleft lip (side view).

taken to preserve the tattooed landmarks on the philtral segment (Fig. 11). Flaps derived from lateral and aspects of the prolabium are approximated to the alar base flaps to reconstruct the nasal sills. The prolabial mucosa is turned down to line the anterior surface of the premaxilla and to set the height of the upper labial sulcus (Fig. 12). The white roll and vermilion of the lateral lip elements are used to reconstruct the Cupid’s bow. Upper buccal sulcus incisions on each side of the cleft extend along the piriform rim to the inferior turbinate, allowing for release of the soft-tissue attachments to the maxilla. Dissection along the piriform rim releases the abnormal attachments of the lower lateral cartilage. Supraperiosteal dissection along the face of the maxilla allows for complete release of the ala-cheek-lip complexes. The orbicularis oris is dissected from the mucosa and from the dermis. Mucosal dissection is limited to the distance needed to advance

the mucosal flaps to the midline. On the dermal aspect, the dissection is carried laterally to the alar base to release all abnormal attachments to the alar base and piriform rim (Fig. 13). At this point, both lateral elements should be free from all abnormal attachments to the maxilla and should be easily mobilized to the midline for a tension-free repair. In wide clefts, several additional maneuvers can be used to provide closure. A T flap can be elevated from the inferior turbinate to provide nasal lining and to prevent cicatricial contracture. Following elevation, the flap is rotated 90 degrees and sutured along the vestibule. The maxillarybased L flap can then be inset at the base of the septum to reconstruct the nasal floor. If a T flap is used, the tip is sutured to the base of the L flap. The premaxilla flap is then brought across the cleft and sutured to the base of the L flap to complete the nasal floor coverage and bridge the anterior alveolar gap.

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Fig. 16. Same patient at the time of palate repair.

Fig. 17. Same patient at 3.5 years of age.

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Volume 132, Number 6 • Primary Repair of Cleft Lip and Nose

Fig. 18. (Left) Bilateral lip and nose repair before nasal stenting. (Right) Bilateral lip and nose repair at 2 weeks postoperatively following nasal stenting.

The alar base width may be reduced by use of an alar cinch suture. Care is taken to place the suture so that the base of the alar rim is reoriented from its abnormal vertical position to a more normal horizontal position. Repair of the orbicularis oris is performed using absorbable sutures (Fig. 14). After this, the lateral skin flaps should lie in close juxtaposition to the prolabial flap without undue tension. The flaps are approximated with fine intradermal sutures, and the lateral vermilion flaps are sutured in the midline to reconstruct the central tubercle.63–68 A semiopen approach to the nasal tip is performed either through the lip incisions or through bilateral rim incisions. The overlying skin is completely dissected from the surface of the lower lateral cartilages. The excess fibrofatty tissue between the domes is released and allowed to remain with the overlying skin. The lower lateral cartilages are repositioned, narrowing the angle of domal divergence using mattress sutures. Any excess tissue along the alar rim may be trimmed, and the incisions are closed carefully with absorbable interrupted sutures61,62,65,66 (Figs. 15 through 17). Nasal stents may be used postoperatively. One author (J.E.L.) prefers the use of stents for at least 6 weeks and ideally for 3 months. The patient is fitted with the appropriate size in the operating room. The stents should fit snugly without causing undue blanching of the nasal tip. These are secured with a sterile adhesive strip that has been cut to allow for patency of the nasal airways. The parents are instructed to remove the stents each day for cleaning and are taught how to replace

and tape them on postoperative day 1. The nasal stents are up-sized in the clinic at the first postoperative visit if possible as the swelling resolves. Significant improvement can be seen at 2 weeks postoperatively (Fig. 18). Joseph E. Losee M.D. Children’s Hospital of Pittsburgh One Children’s Hospital Drive 4401 Penn Avenue Faculty Pavilion, 7th Floor Pittsburgh, Pa. 15224 [email protected]

PATIENT CONSENT

Parents or guardians provided written consent for use of patients’ images. REFERENCES 1. Spritz RA. The genetics and epigenetics of orofacial clefts. Curr Opin Pediatr. 2001;13:556–560. 2. Al-Holou WN, Muraszko KM, Garton HJ, Buchman SR, Maher CO. The outcome of tethered cord release in secondary and multiple repeat tethered cord syndrome. J Neurosurg Pediatr. 2009;4:28–36. 3. Jones MC. Facial clefting: Etiology and developmental pathogenesis. Clin Plast Surg. 1993;20:599–606. 4. Derijcke A, Eerens A, Carels C. The incidence of oral clefts: A review. Br J Oral Maxillofac Surg. 1996;34:488–494. 5. Gatta V, Scarciolla O, Cupaioli M, Palka C, Chiesa PL, Stuppia L. A novel mutation of the IRF6 gene in an Italian family with Van der Woude syndrome. Mutat Res. 2004;547:49–53. 6. Scioletti AP, Brancati F, Gatta V, et al. Two novel mutations affecting splicing in the IRF6 gene associated with van der Woude syndrome. J Craniofac Surg. 2010;21:1654–1656. 7. Stuppia L, Capogreco M, Marzo G, et al. Genetics of syndromic and nonsyndromic cleft lip and palate. J Craniofac Surg. 2011;22:1722–1726.

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Plastic and Reconstructive Surgery • December 2013 8. Lidral AC, Murray JC, Buetow KH, et al. Studies of the candidate genes TGFB2, MSX1, TGFA, and TGFB3 in the etiology of cleft lip and palate in the Philippines. Cleft Palate Craniofac J. 1997;34:1–6. 9. Romitti PA, Lidral AC, Munger RG, Daack-Hirsch S, Burns TL, Murray JC. Candidate genes for nonsyndromic cleft lip and palate and maternal cigarette smoking and alcohol consumption: Evaluation of genotype-environment interactions from a population-based case-control study of orofacial clefts. Teratology 1999;59:39–50. 10. Suazo J, Santos JL, Carreño H, Jara L, Blanco R. Linkage disequilibrium between MSX1 and non-syndromic cleft lip/palate in the Chilean population. J Dent Res. 2004;83:782–785. 11. Murray JC, Nishimura DY, Buetow KH, et al. Linkage of an autosomal dominant clefting syndrome (Van der Woude) to loci on chromosome Iq. Am J Hum Genet. 1990;46:486–491. 12. Mossey PA, Little J, Munger RG, Dixon MJ, Shaw WC. Cleft lip and palate. Lancet 2009;374:1773–1785. 13. Bernheim N, Georges M, Malevez C, De Mey A, Mansbach A. Embryology and epidemiology of cleft lip and palate. B-ENT 2006;2(Suppl 4):11–19. 14. Bixler D, Fogh-Andersen P, Conneally PM. Incidence of cleft lip and palate in the offspring of cleft parents. Clin Genet. 1971;2:155–159. 15. Annegers JF, Elveback LR, Hauser WA, Kurland LT. Epilepsy anticonvulsants and malformations. Birth Defects Orig Artic Ser. 1975;11:157–160. 16. Annegers JF, Elveback LR, Hauser WA, Kurland LT. Do anticonvulsants have a teratogenic effect? Arch Neurol. 1974;31:364–373. 17. Biale Y, Lewenthal H, Aderet NB. Congenital malformations due to anticonvulsive drugs. Obstet Gynecol. 1975;45:439–442. 18. Czeizel A. Letter: Diazepam, phenytoin, and aetiology of cleft lip and/or cleft palate. Lancet 1976;1:810. 19. Honein MA, Rasmussen SA, Reefhuis J, et al. Maternal smoking and environmental tobacco smoke exposure and the risk of orofacial clefts. Epidemiology 2007;18:226–233. 20. Allan FD. Normal and abnormal development of the upper lip, palate, and associated structures. J Am Med Womens Assoc. 1966;21:899–903. 21. Ohbayashi N, Eto K. Relative contributions of the facial processes to facial development: A microsurgical assay. J Craniofac Genet Dev Biol Suppl. 1986;2:41–44. 22. Briedis J, Jackson IT. The anatomy of the philtrum: Observations made on dissections in the normal lip. Br J Plast Surg. 1981;34:128–132. 23. Hwang K, Kim DJ, Hwang SH. Musculature of the pars marginalis of the upper orbicularis oris muscle. J Craniofac Surg. 2007;18:151–154. 24. Zhang KQ. Gross anatomy and microstructure of the orbicularis muscle and philtral contour (in Chinese). Zhonghua Kou Qiang Yi Xue Za Zhi 1990;25:353–355, 383. 25. Zhang KQ. Artery supply of the lip and palate in normal and cleft patients (in Chinese). Zhonghua Kou Qiang Yi Xue Za Zhi 1994;29:30–33, 63. 26. Lee ST. A histological study of the philtrum. Ann Acad Med Singapore 1988;17:328–334. 27. McComb H. Primary correction of unilateral cleft lip nasal deformity: A 10-year review. Plast Reconstr Surg. 1985;75: 791–799. 28. McComb H. Treatment of the unilateral cleft lip nose. Plast Reconstr Surg. 1975;55:596–601. 29. Mulliken JB, Martínez-Pérez D. The principle of rotation advancement for repair of unilateral complete cleft lip and nasal deformity: Technical variations and analysis of results. Plast Reconstr Surg. 1999;104:1247–1260.

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30. Mulliken JB, Pensler JM, Kozakewich HP. The anatomy of Cupid’s bow in normal and cleft lip. Plast Reconstr Surg. 1993;92:395–403; discussion 404. 31. Latham RA. The pathogenesis of the skeletal deformity associated with unilateral cleft lip and palate. Cleft Palate J. 1969;6:404–414. 32. Latham RA, Bursto WR. The effect of unilateral cleft of the lip and palate on maxillary growth pattern. Br J Plast Surg. 1964;17:10–17. 33. Latham RA. Development and structure of the premaxillary deformity in bilateral cleft lip and palate. Br J Plast Surg. 1973;26:1–11. 34. King BF III, Workman CH III, Latham RA. An anatomical study of the columella and the protruding premaxillae in a bilateral cleft lip and palate infant. Cleft Palate J. 1979;16:223–229. 35. McComb H. Primary repair of the bilateral cleft lip nose: A 15-year review and a new treatment plan. Plast Reconstr Surg. 1990;86:882–889; discussion 890. 36. McComb H. Primary repair of the bilateral cleft lip nose. Br J Plast Surg. 1975;28:262–267. 37. McComb HK. Primary repair of the bilateral cleft lip nose: A long-term follow-up. Plast Reconstr Surg. 2009;124:1610–1615. 38. Mulliken JB. Microform cleft lip. In: Losee JE, Kirschner RE, eds. Comprehensive Cleft Care. New York: McGraw-Hill; 2009:273–284. 39. Mulliken JB. Double unilimb Z-plastic repair of microform cleft lip. Plast Reconstr Surg. 2005;116:1623–1632. 40. Pool R, Farnworth TK. Preoperative lip taping in the cleft lip. Ann Plast Surg. 1994;32:243–249. 41. Liou EJ, Subramanian M, Chen PK. Progressive changes of columella length and nasal growth after nasoalveolar molding in bilateral cleft patients: A 3-year follow-up study. Plast Reconstr Surg. 2007;119:642–648. 42. Liou EJ, Subramanian M, Chen PK, Huang CS. The progressive changes of nasal symmetry and growth after nasoalveolar molding: A three-year follow-up study. Plast Reconstr Surg. 2004;114:858–864. 43. Grayson BH, Cutting CB. Presurgical nasoalveolar orthopedic molding in primary correction of the nose, lip, and alveolus of infants born with unilateral and bilateral clefts. Cleft Palate Craniofac J. 2001;38:193–198. 44. Grayson BH, Maull D. Nasoalveolar molding for infants born with clefts of the lip, alveolus, and palate. Clin Plast Surg. 2004;31:149–158, vii. 45. Grayson BH, Santiago PE, Brecht LE, Cutting CB. Presurgical nasoalveolar molding in infants with cleft lip and palate. Cleft Palate Craniofac J. 1999;36:486–498. 46. Grayson BH, Garfinkle JS. Nasoalveolar molding and columella elongation in preparation for the primary repair of unilateral and bilateral cleft lip and palate. In: Losee JE, Kirschner RE, eds. Comprehensive Cleft Care. New York: McGraw-Hill; 2009:701–720. 47. Randall P. A lip adhesion operation in cleft lip surgery. Plast Reconstr Surg. 1965;35:371–376. 48. Kirschner RE, Losee JE. Lip adhesion In: Losee JE, Kirschner RE, eds. Comprehensive Cleft Care. New York: McGraw-Hill; 2009:267–272. 49. Millard DR Jr. Cleft Craft. Boston: Little, Brown; 1976. 50. Randall P. A triangular flap operation for the primary repair of unilateral clefts of the lip. Plast Reconstr Surg Transplant Bull. 1959;23:331–347. 51. Le Mesurier AB. The quadrilateral Mirault flap operation for hare-lip. Plast Reconstr Surg (1946) 1955;16:422–433. 52. Straith CL, Pilling MA, Lewis JR. Repair of single cleft lip by the Hagedorn-Le Mesurier technic. J Int Coll Surg. 1950;13:394–401.

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Primary repair of cleft lip and nasal deformity.

After studying this article, the participant should be able to: 1. Describe the relevant anatomy and embryology that contribute to the cleft lip and n...
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