eMedicine Specialties > Plastic Surgery > Craniofacial

Craniofacial, Unilateral Cleft Lip Repair

Pravin K Patel, MD, Associate Professor of Surgery, Division of Plastic Surgery, Northwestern University School of Medicine; Chief of Plastic and Craniofacial Surgery, Shriners Hospitals for Children; Head of Craniofacial Surgery, Children's Memorial Hospital
Raja Ramaswamy, MS, The Chicago Medical School; Mitchell F Grasseschi, MD, Assistant Professor, Department of Plastic Surgery, Feinberg School of Medicine, Northwestern University; Private Practice, Plastic and Reconstructive Surgery; David E Morris, MD, Assistant Professor of Surgery, Division of Plastic, Reconstructive, and Cosmetic Surgery, University of Illinois at Chicago College of Medicine; Staff Surgeon, Shriner's Hospital for Children

Updated: Jun 19, 2009

Introduction

The presence of unilateral cleft lip is one of the most common congenital deformities. A broad spectrum of variations in clinical presentation exists. Unilateral cleft lip involves deformity of the lip in addition to the alveolus and nose. Patients with this deformity require short-term care and long-term care and follow-up from practitioners in multiple specialties.¹ Patients may need multiple surgical interventions, from infancy to adulthood, in order to achieve necessary function and aesthetic quality. 
 
No universal agreement has been reached as to the timing and technique of repair. Several methods are used with comparable long-term results, which serves as an indication that more than one treatment option exists for definitive repair. Treatment goals include the restoration of facial appearance and oral function, improvement of dental skeletal and occlusal relationships, improvement of speech, and the psychosocial state.

History of the Procedure

In 1843, closure of the unilateral cleft lip with local flaps was described by Malgaigne. The following year, Mirault modified Malgaigne’s technique by using the lateral lip flap to fill the medial defect. All future methods of cleft lip closure are based on Mirault’s technique. LeMesurier and Tennyson modified this technique with a quadrilateral and triangular flap, respectively. In 1976, Millard published his definitive repair in which the lateral flap advancement into the upper portion of lip was combined with downward rotation of medial lip.² Other modifications have been published by Noordhoff, Mohler, and Onizuka.^3,4 Fisher has described an anatomical subunit approximation for definitive cleft lip repair. Millard’s methods, including variations, remain among the most popular method for unilateral cleft lip closure.⁴

Cleft lip surgery has evolved from a simple adhesion of paired margins of the cleft to an understanding of the various malpositioned elements of the lip to a more complicated geometric reconstruction using transposition, rotation, and advancement flaps.⁵

Problem

The cleft affects the facial form as an anatomic deformity and has functional consequences. These include the child's ability to eat, speak, hear, and breathe. Consequently, rehabilitation of a child born with a facial cleft must involve a multidisciplinary approach and staged appropriately with the child's development, balancing the timing of intervention against its effect on subsequent normal growth.

Frequency

The overall occurrence of cleft lip with or without cleft palate is approximately 1 in 750-1000 live births. Racial differences exist, with the incidence in Asians (1:500) greater than in Caucasians (1:750) greater than in African Americans (1:2000). The incidence of cleft lip/palate is more common in males.

The most common presentation is cleft lip and palate (approximately 45%), followed by cleft palate alone (35%) and cleft lip alone (approximately 20%). Unilateral cleft lips are more common than bilateral cleft lips and occur more commonly on the left side (left cleft lip:right cleft lip:bilateral cleft lip = 6:3:1).

The risk of a newborn having a cleft lip increases if a first-degree relative also has a cleft. If one child already has a cleft lip, the chance of a second child being born with the deformity is 4%. If a parent has a cleft lip, the chance of a newborn having a cleft is 7%. If both a parent and a sibling have a cleft lip, the newborn's risk rises to 15%.

Etiology

Clefting has a multifactorial basis, with both genetic and environmental causes cited. The observation of clustered cases of facial clefts in a particular family indicates a genetic basis. Clefting of the lip and/or palate is associated with more than 150 syndromes. The overall incidence of associated anomalies (eg, cardiac) is approximately 30% (more common with isolated cleft palate).

Environmental causes, such as viral infection (eg, rubella) and teratogens (eg, steroids, anticonvulsants), during the first trimester have been linked to facial clefts. The risk also increases with parental age, especially when older than 30 years, with the father's age appearing to be a more significant factor than the mother's age. Nevertheless, most presentations are of isolated patients within the family without an obvious etiology.

Midfacial development involves several sets of genes, including those involved in cell patterning, proliferation, and signaling. Mutations in any of these genes can change the developmental process and contribute to cleft development. Some of these genes include the DIX gene, sonic hedgehog (SHH) gene, transforming growth factor (TGF) alpha/beta, and interferon regulatory factor (IRF6).

Classification

Kernahan developed a classification scheme in which the defect can be classified onto a Y-shaped symbol. In this diagram, the incisive foramen is represented as the focal point. This system has been applied to both cleft lip and palate.

Millard modification of Kernahan striped-Y classification for cleft lip and palate. The small circle indicates the incisive foramen; the triangles indicate the nasal tip and nasal floor.

Pathophysiology

While the normal embryologic development of the face is detailed in Head and Neck Embryology, a brief outline relevant to the formation of facial clefts follows.

In short, the branchial arches are responsible for the formation of several areas, including the mouth and lip. Mesenchymal migration and fusion occurs during weeks 4-7 of gestation. The first branchial arch is responsible for the formation of the maxillary and mandibular processes. The maxillary and mandibular prominences form the lateral borders of the primitive mouth or stomodeum.

Mesenchymal migration and fusion of the primitive somite-derived facial elements (central frontonasal, 2 lateral maxillary, mandibular processes), at 4-7 weeks gestation, is necessary for the normal development of embryonic facial structures. When migration and fusion are interrupted for any reason, a facial cleft develops along embryonic fusion lines. The embryonic development of the primary palate (lip and palate anterior to the incisive foramen) differs from the secondary palate (palate posterior to the incisive foramen).

The developing processes of the medial nasal prominence, lateral nasal prominence, and maxillary prominences form the primary palate. Fusion occurs, followed by "streaming" of mesodermal elements derived from the neural crest. In contrast, the secondary palate is formed by the fusion of palatal processes of the maxillary prominence alone. The difference in embryonic development suggests the possibility of differing degrees of susceptibility to genetic and environmental influences and accounts for the observed variation in incidences.

In summary, unilateral cleft lip results from failure of fusion of the medial nasal prominence with the maxillary prominence.

Presentation

For treatment purposes, unilateral cleft lip can be placed into one of three categories: microform/forme fruste, incomplete, or complete cleft lip. 

• Microform cleft (forme fruste): This defect is characterized by a "light" furrow along the vertical length of the lip with a small vermilion notch and minor imperfections in the white roll. A small component of vertical lip length deficiency and associated nasal deformity may be present.
• Incomplete cleft lip: This defect is characterized by the varying degree of vertical lip separation. By definition, it has an intact nasal sill, commonly termed the Simonart band.
• Complete cleft lip: This involves the full-thickness defect of the lip and alveolus (primary palate), extends into the base of the nose (no Simonart band exists), and is often accompanied by a palatal cleft (secondary palate). The premaxilla is typically rotated outward and projects anteriorly in relation to a relatively retropositioned lateral maxillary alveolar element.

Indications

Patients born with a cleft lip should undergo surgical repair unless otherwise contraindicated. The goal of reconstruction is to establish normal morphologic facial form and function in order to provide the optimal conditions for the development of dentition, mastication, hearing, speech, and breathing, and psychosocial status.

Relevant Anatomy

Normal lip and nasal anatomy is essential for an understanding of the distortion caused by a facial cleft. The elements of the normal lip are composed of the central philtrum, demarcated laterally by the philtral columns and inferiorly by the Cupid's bow and tubercle. Just above the junction of the vermilion-cutaneous border is a mucocutaneous ridge frequently referred to as the white roll. Within the red vermilion of the lip is a noticeable junction demarcating the dry and wet vermilion, the increased keratinized portion of the lip that is exposed to air from the moist environment of the labial mucosa.

The primary muscle of the lip is the orbicularis oris, and it has two well-defined components: the deep (internal) and the superficial (external) components. The deep (internal) fibers run horizontally or circumferentially from commissure (modiolus) to commissure (modiolus) and functions as the primary sphincteric action for oral feeding. The superficial (external) fibers run obliquely, interdigitating with the other muscles of facial expression to terminate in the dermis. They provide subtle shades of expression and precise movements of the lip for speech.

The superficial fibers of the orbicularis decussate in the midline and insert into the skin lateral to the opposite philtral groove forming the philtral columns. The resulting philtral dimple centrally is depressed as there are no muscle fibers that directly insert into the dermis in the midline. The tubercle of the lip is shaped by the pars marginalis, the portion of the orbicularis along the vermilion forming the tubercle of the lip with eversion of the muscle.

In the upper lip, the levator labii superioris contributes to the form of the lip. Its fibers, arising from the medial aspect of the infraorbital rim, sweep down to insert near the vermilion cutaneous junction. The medial-most fibers of the levator labii superioris sweep down to insert near the corner of the ipsilateral philtral column and vermilion-cutaneous junction, helping to define the lower philtral column and the peak of the Cupid's bow.

The nasal muscles are equally important. The levator superioris alaeque arises along the frontal process of the maxilla and courses inferiorly to insert on the mucosal surface of the lip and ala. The transverse nasalis arises along the nasal dorsum and sweeps around the ala to insert along the nasal sill from lateral to medial into the incisal crest and anterior nasal spine. These fibers join with the oblique fibers of the orbicularis and the depressor septi (nasalis), which arises from the alveolus between the central and lateral incisors to insert into the skin of the columellar to the nasal tip and the footplates of the medial crura.

A unilateral cleft thus disrupts the normal termination of the muscle fibers that cross the embryologic fault line of the maxillary and nasal processes, resulting in symmetric but abnormal muscular forces between the normal equilibrium that exists with the nasolabial and oral groups of muscles. With an unrestrained premaxilla, the deformity accentuates with differential growth of the various elements. The alar cartilages are splayed apart and rotate caudally, subluxed from the normal position. Consequently, the nasal tip broadens, the columellar is foreshortened, and the alar bases rotate outwardly cephalad.

Contraindications

• Malnutrition, anemia, or other pediatric conditions that result in the patient's inability to tolerate general anesthesia are contraindications to this procedure.
• Cardiac anomalies that may coexist must be addressed prior to the lip repair.

Workup

Laboratory Studies

• Perform a thorough physical examination, not limited to the head and neck region, to uncover associated anomalies in the infant presenting with a unilateral cleft lip with or without a palatal cleft. Additional workup is determined by physical findings that suggest involvement of other organ systems.
• The child's weight, oral intake, and growth and/or development are of primary concern and must be followed closely.
• Routine laboratory studies typically are not required, other than a hemoglobin study shortly before the planned lip repair.
• Routine imaging is not indicated in a healthy patient with isolated cleft lip.

Treatment

Surgical Therapy

Children born with a facial cleft benefit from multidisciplinary clinical care. This is a team-based approach allowing efficient coordination of all aspects of care. Beyond the lip repair are other issues such as hearing, speech, dental, and psychosocial integration. With the multidisciplinary approach, as the child grows, comprehensive care can be given from birth through adolescence. These associated issues are as important as the anatomic reconstruction, and ultimately the functional outcome of the reconstruction depends on addressing them.

Each specialty involved must evaluate the child individually and formulate a treatment plan, then the team forms a combined individual integrated protocol that follows the Parameters of Care Guidelines established by the American Cleft Palate Craniofacial Association. Rather than strictly adhering to any one protocol, each child is assessed based on the present need in his or her development, and a treatment plan is created based on the team's experience.

Preoperative Details

While the lip repair is the initial focus for many parents, treatment begins by assessing the child's nutritional status and assisting the parents with oral feeding techniques so that appropriate weight gain occurs.

Parents who suddenly are faced with caring for a child with a facial cleft are overwhelmed. The importance of spending sufficient time with them to allay their fears, to discuss staging and timing of reconstruction, to stress the need for involvement of other specialists, and to instruct them on the importance of long-term and consistent follow-up care from birth through adolescence cannot be overemphasized.

The optimal timing of the surgical repair is still somewhat controversial. Some centers have advocated surgery in the early neonatal period, with a theoretical benefit in the scar appearance and nasal cartilage adaptability, thus minimizing the nasal deformity. To minimize anesthetic risks, some still adhere to the rule of 10s: perform surgical repair of cleft lip when the child has a hemoglobin of 10 g, weight of 10 lb, and is aged 10 weeks. In general, however, most centers prefer to perform the unilateral lip repair when the infant is aged 2-4 months; anesthesia risks are lower, the child is better able to withstand the stress of surgery, and lip elements are larger and allow for a meticulous reconstruction.

Before the definitive lip surgery, cleft centers utilize lip taping, alone or in combination with a passive intraoral appliance or an active pin-based appliance (eg, Latham) to align the maxillary arch segments; or no presurgical orthopedic intervention at all. This choice depends on the center's protocol and resources.

A number of cleft centers prefer to use a passive intraoral orthodontic palatal appliance to maintain the arch width to prevent the nearly inevitable collapse that occurs with the lip surgery.⁶ The lip repair reestablishes the soft tissue and muscular forces on the easily moldable maxillary arch segments. Additionally, this appliance may include a nasal extension to help improve the nasal tip form. This nasal alveolar molding device is incorporated into the intraoral appliance. Several weeks of treatment prior to the surgery and regular adjustments are needed to mold the alar cartilages into a more favorable position, thus facilitating the surgical correction of the nasal deformity. Impressions are taken soon after birth so that the custom appliance can be applied as soon as possible before the lip repair. The appliance also assists in the child's oral feeding, helping to decrease nasal regurgitation and assisting oral suction.

Intraoperative Details

The ideal lip repair results in symmetrically shaped nostrils, nasal sill, and alar bases; a well-defined philtral dimple and columns; and a natural appearing Cupid's bow with a pout to the vermilion tubercle. In addition, it results in a functional muscle repair that with animation mimics a normal lip. While ideally the lip scars approximate natural landmarks, ultimately the eye first focuses on symmetry and then normal contours of the lip at rest and in animation.

A number of surgical procedures for the repair of a unilateral cleft lip are well described, with a multitude of variations, including the LeMesurier quadrilateral flap repair, Randall-Tennison triangular flap repair, Millard rotation-advancement repair,⁹ and Skoog and Kernahan-Bauer upper and lower lip Z-plasty repairs. Many other variations exist; of particular note are the repairs by Delaire and by Poole.

The Rose-Thompson repair involves curved or angled paring of the cleft margins to lengthen the lip as a straight-line closure.

Hagedorn-LeMesurier repair. The medial lip element is lengthened by introducing a quadrilateral flap developed from the lateral lip element.

Tennison-Randall repair. The medial lip element is lengthened by introducing a triangular flap from the inferior portion of the lateral lip element.

Skoog repair. The medial lip element is lengthened by introducing two small triangular flaps developed from the lateral lip element.

While none of the repairs is ideal, each has advantages and disadvantages, and each results in an excellent repair in experienced hands, underscoring the fact that more than a single acceptable technique, rather than a single ideal repair, is available. However, because of the limitations of this article, the authors choose to focus on the repair Millard first described in 1955, as today it is perhaps the most commonly adapted repair of cleft lip.

The rotation-advancement method of Millard advances a mucocutaneous flap from the lateral lip element into the gap of the upper portion of the lip resulting from the inferior downward rotation of the medial lip element.⁹ The repair attempts to place the lip scars along anatomic lines of the philtral column and nasal sill. Conceptually, Millard's approach is elegant but it is not always technically easy to accomplish without some modifications to deal with the wide variation in clefts. As with any other repair, consistency in achieving a good result is operator-dependent.

A cursory description of a modified Millard operative technique used by the authors is as follows:

• Use general anesthesia with a noncuffed oro-Rae endotracheal tube positioned midline. Typically the otolaryngologist then examines the ears; if needed, myringotomy and pressure equalizing tubes are placed.
• Prior to infiltration with a local anesthetic (0.5% lidocaine with 1:200,000 epinephrine), mark the anatomic landmarks and tattoo them with a methylene blue dye.
  
  

  

  Figure illustrates important anatomic landmarks used in all cleft lip repairs. Measurements of various distances are used to guide the surgeon in creating a symmetric lip.

  
  
• Two key elements are involved in the markings: the placement of the final position of the new Cupid's bow peak and the vertical length of the philtral column to be created on the cleft side. Referring to the diagram, Point 3 is determined as the mirror image of Point 2 based on the distance from the midpoint to the peak of the Cupid's bow on the noncleft side. The peak on the cleft side, Point 4, is not determined as easily but typically is placed level with Point 2, where the dry vermilion is widest and the white roll above is well developed. The white roll and dry vermilion taper off medial to this point. It is unreliable to determine the peak on the cleft side using the distance between the peak of the Cupid's bow from the commissure on the noncleft side because of unequal tension of the underlying orbicularis muscle.
• Once the anatomic points are marked, draw incision lines that define the 5 flaps involved in the lip reconstruction. These are the inferior rotation flap (R) of the medial lip element, the medial advancement flap (A) of the lateral lip element, the columellar base flap (C) of the medial lip element, and the two pared mucosal flaps of the medial (m) and lateral (l) lip elements. Two additional flaps that refine the repair often are used: a white roll flap and a vermilion triangular flap to allow for a smoother transition at the vermilion cutaneous junction and at the vermilion contour.
• The essential marking is the line that determines the border between the R and C flaps. This line becomes the new philtral column on the cleft side. For the vertical lengths of the philtrum on the cleft side and noncleft side to be symmetric, the length of the rotation advancement flap (y) should equal the vertical length of the philtral column (x) on the noncleft side (distance between alar base and Cupid's bow peak). For the two lengths, x and y, to be equal, the path of y must be curved as illustrated. In marking the curve, take care to avoid a high arching curve that comes too high at the columellar base to create a generous philtrum, as this significantly diminishes the size of the C flap.
• While all flaps are marked, the authors typically refine the design of the A flap after the R and C flaps are repositioned appropriately so that it more accurately is tailored to fill the gap left by the inferior rotation of the R flap and the final placement of the C flap.
• Pare the margins of the cleft and develop the m and l flaps. The l flap can be used to inset into the nasal vestibule lining, and the m flap can be used as part of the orolabial vestibule lining as needed. Alternatively, both flaps can be used to reconstruct the nasal and orovestibular lining of the nasal floor depending on the situation. The pars marginalis of the orbicularis typically is tethered by its abnormal insertion and further is pared, allowing the constricted muscle to expand.
• In the region of the vermilion-cutaneous junction, incise the muscle for approximately 2-3 mm on either side of the cleft paralleling the vermilion border to allow development of vermilion-cutaneous muscular flaps for final alignment.
• Develop the R and C flaps by incising the line (x) between the flaps to allow inferior rotation of the R flap so that it lies horizontally tension free with Point 3, level with Point 2. For this to occur, release must be at all levels (skin, subcutaneous tissue, muscle, fibrous attachments to the anterior nasal spine, labial mucosa). Occasionally an additional 1- to 2-mm back cut just medial to the noncleft philtral column is required along with a mucosal back cut to allow for adequate inferior rotation of the R flap. The back cut occasionally can be limited to the subdermal portion to avoid lengthening the cutaneous scar.
  
  

  

  Millard repair. With maximal rotation of the R flap, any residual lip length discrepancy can be corrected with an inferior Z-plasty or a triangular flap. In a secondary correction, further rotation of the R flap can be considered.

  
  
• Correspondingly free the C flap with the medial crus of the alar cartilage and allow it to be repositioned, creating a large gap to be filled by the A flap.
• Develop the A flap from the lateral lip element for advancement into the gap between the R and C flaps. In developing the A flap, keep the incision along the alar base at a minimum; it rarely is required to extend much beyond the medial-most aspect of the alar base. The key to allowing adequate mobilization of the A flap is the subcutaneous release of the fibrous attachments of the alar base to the piriform margin of the maxilla and not necessarily a continued cutaneous incision along the alar margin. Other surgeons have chosen to mobilize the ala at the subperiosteal level.
  
  

  

  Millard repair. The medial lip element [R] is rotated inferiorly and the lateral lip element [A] is advanced into the resulting upper lip defect. The columellar flap [C] is then used to create the nasal sill (see text for details).

  
  
• A lateral labial mucosal vestibular release also is required to mobilize the A flap medially and to avoid a tight-appearing postoperative upper lip deformity. Do not forget that the maxillary alveolar arches typically are at different heights in the coronal plane, and the ala must be released completely and mobilized superior medially to achieve symmetry, although ultimately its maxillary support is inadequate until arch alignment and bone grafting can be accomplished.
• As part of the mobilization of the ala, make an incision along the nasal skin-mucosal vestibular junction (infracartilaginous) where the previously developed l flap may be interposed if needed. Currently, the trend is toward more aggressive mobilization and repositioning of the lower lateral cartilages as an integral part of the cleft lip repair.
• Widely undermine the nasal tip between the cartilage and the overlying skin approaching laterally from the alar base and medially from the columellar base.
• While the A flap can be inserted as a mucocutaneous flap incorporating the orbicularis, the authors repair the muscle separately to allow for differential re-orientation of its vectors. Dissect the muscle from the overlying skin and the underlying mucosa to accomplish this and divide it into bundles that can be repositioned and interposed appropriately.
• Once all the flaps are developed and the medial and lateral lip elements are well mobilized, begin reconstruction. Typically, this begins with creating the labial vestibular lining from superior to inferior and then proceeding to the junction of the wet-dry vermilion with completion of the remainder of the vermilion after the cutaneous portion of the lip is completed.
• At this point, the labial mucosa can be advanced as needed, with additional lengthening and a back cut to allow for adequate eversion of the lip and to avoid a tight-appearing lip postoperatively.
• Direct attention to approximating the muscle bundles. Appropriately reorient the nasolabial group of muscles toward the nasal spine. Follow this by approximating the orbicularis, interdigitated with its opposing element along the full length of the vertical lip. Inset the C flap to create a symmetric columellar length and flare at its base. Millard originally described the C flap to cross the nasal sill to insert into the lateral lip element as a lateral rotation-advancement flap. Millard later refined the C flap as a medial superior rotation flap to insert into the medial lip element, augmenting the columellar height and creating a more natural flare at the base of the medial footplate. The latter method occasionally results in a nexus of scars at the base of the columellar with unfavorable healing if the flaps are not well planned. However, the authors and others continue to use the C flap in either position as needed.
  
  

  

  Millard repair. Two of the most common variations described with utilization of the C Flap to correct the hemi-columellar deficiency (Millard II] and the nasal sill alar base region [Millard I]

  
  
• Set the ala base in place. As the C and A flaps and the ala are inset, take care to leave an appropriate width to the nasal sill to avoid a constricted-appearing nostril, which is nearly impossible to correct as a secondary deformity.
• Approximate the vermilion-cutaneous junction and inset the vermilion mucocutaneous triangular flap. If the lip appears to be vertically short at this point, the authors inset a small, 2- to 3-mm triangular flap into the medial lip just above the vermilion.
• Use dermal sutures to approximate the skin edges. Final approximation is with either rapidly absorbing sutures or nylon sutures, ideally removed at 5 days. If the cutaneous edges are well approximated with dermal sutures alone, the authors occasionally use a cyanoacrylate-type adhesive. Reposition the cleft alar cartilage with suspension/transfixion sutures and a stent. Further shape the ala with through-and-through absorbable sutures as needed.

Postoperative Details

• Oral feedings: For the child who is breastfed, the authors encourage uninterrupted breastfeeding after surgery. Bottle-fed children can resume feedings immediately following surgery with the same crosscut nipple used before surgery. Some centers still advocate having the child use a soft catheter-tip syringe for 10 days and then resuming normal nipple bottle feeding, but the authors have found this degree of caution to be unnecessary.
• Activities: The authors instruct the parents to avoid giving the child pacifiers or toys with sharp edges for 2 weeks after surgery. No other particular restrictions on activity are necessary. Some centers do advocate the use of Velcro elbow immobilizers on the patient for 10 days to minimize the risk of inadvertent injury to the lip repair. These are periodically removed several times a day under supervision.
• Lip care: The exposed suture line at the base of the nose and red lip can be cleaned using cotton swabs with diluted hydrogen peroxide, and topical antibiotic ointment can be applied several times a day. The authors then remove the permanent sutures on postoperative day 5-7. If cyanoacrylate adhesive is used, no additional care is required in the immediate postoperative period until the adhesive film comes off. The authors tell the parents to expect noticeable scar contracture, erythema, and firmness 4-6 weeks postsurgery, and that this gradually begins to improve 6-12 months after the procedure. Typically, the authors instruct parents to massage the upper lip during this phase and to avoid placing the child in direct sunlight until the scar matures.

Follow-up

Following cleft lip repair, patients are evaluated periodically by the various cleft team members. Oral hygiene and dental care must be promoted, hearing and speech must be assessed, and psychosocial evaluation and treatment should be made available.

Despite technical advances and simultaneous correction of the nasal deformity performed at the time of lip repair, a significant number of patients still require a secondary procedure to restore nasal symmetry and improve function.¹⁰ Such procedures should be individualized. The alar base symmetry is unlikely to be improved until the alveolar alignment is corrected and grafted with bone. The remaining components of cleft care are addressed in other articles, including the following:

• Cleft Lip and Palate
• Cleft Palate
• Craniofacial, Bilateral Cleft Lip Repair
• Craniofacial, Bilateral Cleft Nasal Repair
• Craniofacial, Unilateral Cleft Nasal Repair
• Cleft Lip Nasal Deformity

Complications

Several common mistakes are made in the rotation-advancement method of unilateral cleft lip repair. These include insufficient rotation of the R flap, vermilion-cutaneous mismatch, vermilion notching and a tight-appearing lateral lip element, a lateral muscle bulge, a laterally displaced ala, and a constricted-appearing nostril.

Aside from unsatisfactory appearance of the surgical result, possible complications include dehiscence of the repair (more common if the repair is delayed until the child is learning to walk and falls) and excessive scar formation and/or contracture of lip scars. If dehiscence occurs, postpone re-operation until the induration has subsided completely. With lip scars that appear red, thick, and contracted, the authors use an occlusive tape dressing and if needed, Kenalog-10 (triamcinolone acetonide) injection and/or flurandrenolide tape. For most repairs, the observed contracture is part of the normal healing process and improves with time. Postpone revisional surgery until the scar matures. Intervention should be guided by the severity of the residual deformity. Keep revisions to a minimum.

Outcome and Prognosis

Careful preoperative assessment of the cleft lip deformity and attention to detail in the reconstruction typically results in an excellent repair that achieves many characteristics of the natural lip. Realistically, many variables are involved beyond the technical aspects of a particular repair. Ultimately, the outcome depends on the natural course of uncomplicated healing of the initial repair, alignment of the skeletal framework on which the lip rests, and the differential effect of normal growth and development on the operated lip.

While a poor initial result is unlikely to improve with time, do not assume that an excellent initial result will not require some revisional procedure because of uncontrolled variables. Moreover, while the lip repair may be acceptable, additional procedures required to achieve nasal symmetry are not uncommon, despite the initial primary nasal surgery incorporated as an integral part of lip repair.

Future and Controversies

Cleft lip surgery has evolved from a geometrically defined "cookie-cutter" type approach to a more adaptable repair using the principles outlined by Millard's elegant rotation advancement technique.^9,11 Skin flap design has led to a better understanding of the underlying musculature that is disrupted by the cleft and the importance of realignment of the individual bundles to create a functional repair. With a better understanding of the underlying anatomy, cleft surgery currently results in an excellent lip repair but is marred by a residual cleft nasal deformity.

Adjunct treatment. including early presurgical alveolar and nasal molding with a palatal appliance. may improve the long-term outcome, with the ultimate intent to remove the accompanying cleft nasal deformity, which is the most common stigmata of a facial cleft.

Only close long-term follow-up care and an honest assessment of the results can establish these improvements in outcome.¹ Advances in the treatment of children with clefts will come only from a team-based approach in which close cooperation of multiple disciplines can address all the child's needs. Such children deserve to be cared for at major centers where an interdisciplinary approach is possible and substantial experience is available.

Multimedia

Media file 1: Figure illustrates important anatomic landmarks used in all cleft lip repairs. Measurements of various distances are used to guide the surgeon in creating a symmetric lip.

Media file 2: The Rose-Thompson repair involves curved or angled paring of the cleft margins to lengthen the lip as a straight-line closure.

Media file 3: Hagedorn-LeMesurier repair. The medial lip element is lengthened by introducing a quadrilateral flap developed from the lateral lip element.

Media file 4: Tennison-Randall repair. The medial lip element is lengthened by introducing a triangular flap from the inferior portion of the lateral lip element.

Media file 5: Skoog repair. The medial lip element is lengthened by introducing two small triangular flaps developed from the lateral lip element.

Media file 6: Millard repair. The medial lip element [R] is rotated inferiorly and the lateral lip element [A] is advanced into the resulting upper lip defect. The columellar flap [C] is then used to create the nasal sill (see text for details).

Media file 7: Millard repair. Two of the most common variations described with utilization of the C Flap to correct the hemi-columellar deficiency (Millard II] and the nasal sill alar base region [Millard I]

Media file 8: Millard repair. With maximal rotation of the R flap, any residual lip length discrepancy can be corrected with an inferior Z-plasty or a triangular flap. In a secondary correction, further rotation of the R flap can be considered.

Media file 9: Millard modification of Kernahan striped-Y classification for cleft lip and palate. The small circle indicates the incisive foramen; the triangles indicate the nasal tip and nasal floor.

References

1. Salyer KE, Genecov ER, Genecov DG. Unilateral cleft lip-nose repair--long-term outcome. Clin Plast Surg. Apr 2004;31(2):191-208. [Medline].

2. Millard DR Jr. Cleft Craft. Vol 1, 2, 3. Boston, Mass: Little Brown & Company; 1976.

3. Noordhoff MS. Bilateral cleft lip reconstruction. Plast Reconstr Surg. Jul 1986;78(1):45-54. [Medline].

4. Sitzman TJ, Girotto JA, Marcus JR. Current surgical practices in cleft care: unilateral cleft lip repair. Plast Reconstr Surg. May 2008;121(5):261e-270e. [Medline].

5. Sykes JM, Tollefson TT. Management of the cleft lip deformity. Facial Plast Surg Clin North Am. Feb 2005;13(1):157-67. [Medline].

6. Da Silveira AC, Oliveira N, Gonzalez S. Modified nasal alveolar molding appliance for management of cleft lip defect. Journal of Craniofacial Surgery. 2003;14(5):700-3. [Medline].

7. 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. Sep 15 2004;114(4):858-64. [Medline].

8. Grayson BH, Santiago PE, Brecht LE, Cutting CB. Presurgical nasoalveolar molding in infants with cleft lip and palate. Cleft Palate Craniofac J. Nov 1999;36(6):486-98. [Medline].

9. Millard DR. Extensions of the rotation-advancement principle for wide unilateral cleft lips. Plast Reconstr Surg. Dec 1968;42(6):535-44. [Medline].

10. Cohen M, Smith BE, Daw JL. Secondary unilateral cleft lip nasal deformity: functional and esthetic reconstruction. Journal of Craniofacial Surgery. 2003;14(4):584-93. [Medline].

11. Trier WC. Repair of unilateral cleft lip: the rotation-advancement operation. Clin Plast Surg. Oct 1985;12(4):573-94. [Medline].

12. American Cleft Palate-Craniofacial Association. Parameters for Evaluation and Treatment of Patients with Cleft Lip/Palate or Other Craniofacial Anomalies. Vol 30. Pittsburgh, Pa: American Cleft Palate-Craniofacial Association; 1993.

13. Bardach J. Salyer & Bardach's Atlas of Craniofacial & Cleft Surgery: Cleft Lip and Palate Surgery. Vol 2. Lippincott-Raven Publishers; 1999.

14. Bardach J, Salyer KE. Surgical Techniques in Cleft Lip and Palate. 2^nd ed. Chicago: Mosby Yearbook; 1991.

15. Berkowitz S, ed. Cleft Lip and Palate. San Diego, Calif: Singular Publishing Group Inc;1996.

16. Clifford RH, Pool R. The analysis of the anatomy and geometry of the unilateral cleft lip. Plast Reconstr Surg. 1959;24:311.

17. Grabb WC, Rosenstein SW, Bzoch KR, eds. Cleft Lip and Palate. Boston, Mass: Little Brown & Company; 1971.

18. McComb H. Treatment of the unilateral cleft lip nose. Plast Reconstr Surg. May 1975;55(5):596-601. [Medline].

19. McComb HK, Coghlan BA. Primary repair of the unilateral cleft lip nose: completion of a longitudinal study. Cleft Palate Craniofac J. Jan 1996;33(1):23-30; discussion 30-1. [Medline].

20. Menard RM, Schendel SA. Rotation-Advancement Repair of the Unilateral Cleft Lip: Current Status and Future Horizons. St. Louis, Mo: Mosby-Yearbook; 1997:247-78.

21. Muller W. Differentiated reconstruction of the orbicularis oris muscle in unilateral labioplasty. J Craniomaxillofac Surg. Dec 1989;17 Suppl 1:11-3. [Medline].

22. Salyer KE, Genecov ER, Genecov DG. Unilateral cleft lip-nose repair--long term outcome. Clin Plast Surg. 2004;Apr;31(2):191-208. [Medline].

Keywords

cleft lip, cleft lip repair, cleft palate, cleft palate repair, unilateral cleft lip repair, unilateral cleft lip, cleft lip and palate, facial cleft, cleft, lip, palate, congenital deformities, Kernahan, Kernahan classification, Millard technique, Millard repair, Millard cleft repair, face embryology, facial development, facial defect, embryonic development

Contributor Information and Disclosures

Author

Pravin K Patel, MD, Associate Professor of Surgery, Division of Plastic Surgery, Northwestern University School of Medicine; Chief of Plastic and Craniofacial Surgery, Shriners Hospitals for Children; Head of Craniofacial Surgery, Children's Memorial Hospital
Disclosure: Nothing to disclose.

Coauthor(s)

Raja Ramaswamy, MS, The Chicago Medical School
Raja Ramaswamy, MS is a member of the following medical societies: Alpha Omega Alpha
Disclosure: Nothing to disclose.

Mitchell F Grasseschi, MD, Assistant Professor, Department of Plastic Surgery, Feinberg School of Medicine, Northwestern University; Private Practice, Plastic and Reconstructive Surgery
Disclosure: Nothing to disclose.

David E Morris, MD, Assistant Professor of Surgery, Division of Plastic, Reconstructive, and Cosmetic Surgery, University of Illinois at Chicago College of Medicine; Staff Surgeon, Shriner's Hospital for Children
David E Morris, MD is a member of the following medical societies: Chicago Medical Society and Illinois State Medical Society
Disclosure: Nothing to disclose.

Medical Editor

Larry Hollier, Jr, MD, Assistant Professor, Department of Plastic Surgery, Baylor University College of Medicine
Larry Hollier, Jr, MD is a member of the following medical societies: Alpha Omega Alpha, American Cleft Palate/Craniofacial Association, American College of Surgeons, American Medical Association, American Society of Maxillofacial Surgeons, AO Foundation, and Phi Beta Kappa
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

R Edward Newsome, MD, Program Director and Chief of Plastic Surgery, Henderson Chair in Surgery, Assistant Dean for Graduate Medical Education, Tulane University School of Medicine
R Edward Newsome, MD is a member of the following medical societies: American College of Surgeons, American Medical Association, American Society of Plastic and Reconstructive Surgery, American Society of Plastic Surgeons, and Louisiana State Medical Society
Disclosure: Nothing to disclose.

CME Editor

Nicolas (Nick) G Slenkovich, MD, Director, Colorado Plastic Surgery Center
Nicolas (Nick) G Slenkovich, MD is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, American College of Surgeons, American Medical Association, American Society of Aesthetic Plastic Surgery, American Society of Plastic Surgeons, and Colorado Medical Society
Disclosure: Nothing to disclose.

Chief Editor

Jorge I de la Torre, MD, FACS, Professor of Surgery and Physical Medicine and Rehabilitation, Residency Program Director, Division of Plastic Surgery, University of Alabama at Birmingham; Director, Center for Advanced Surgical Aesthetics
Jorge I de la Torre, MD, FACS is a member of the following medical societies: American Association of Plastic Surgeons, American Burn Association, American College of Surgeons, American Medical Association, American Society for Laser Medicine and Surgery, American Society for Reconstructive Microsurgery, American Society of Maxillofacial Surgeons, American Society of Plastic Surgeons, Association for Academic Surgery, and Medical Association of the State of Alabama
Disclosure: Nothing to disclose.

The authors and editors of eMedicine gratefully acknowledge the contributions of previous authors Kara K Criswell MD, Bryan K Criswell MD, and Mimis Cohen, MD, FACS, FAAP to the development and writing of this article.

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