Bilateral Cleft Lip Repair Treatment & Management

Updated: Oct 18, 2016
  • Author: Pravin K Patel, MD; Chief Editor: Jorge I de la Torre, MD, FACS  more...
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Treatment

Surgical Therapy

Children born with a facial cleft benefit from a multidisciplinary approach in a team-based setting so that all aspects of their care can be coordinated efficiently.

Beyond lip repair are hearing, speech, dental, and psychosocial integration issues. [12] These should be addressed throughout the child's growth and development from birth through adolescence. These issues are as important as the anatomic reconstruction. Ultimately, the functional outcome of the reconstruction depends on cooperation between the surgeon, orthodontist, speech pathologist, and psychologist.

Each specialty involved must evaluate the child individually, formulate a treatment plan, and integrate it with those of the other specialties based on the child's needs. The Parameters of Care Guidelines established by the American Cleft Palate-Craniofacial Association should be followed. [13] However, rather than strictly adhering to one protocol, physicians should assess each child individually and formulate the treatment plan based on the team's experience, its overall philosophy of treatment, and available resources.

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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 can be 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 stress the importance of long-term and consistent follow-up care from birth through adolescence cannot be overemphasized.

No agreement is found in the literature regarding the ideal timing of lip repair. Some have advocated surgery in the early neonatal period, with a theoretical benefit in 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 level of 10 g, weight of 10 lb, and is aged 10 weeks. In general, most centers prefer to perform the lip reconstruction when the patient 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.

A presurgical, fabricated, passive, intraoral orthodontic palatal appliance can be used to maintain the arch width to prevent the nearly inevitable collapse that occurs with lip repair. Rarely is there an indication for active expansion of the maxillary segment. This passive plating and gentle traction is an integral component of surgical cleft lip repair. The repair reestablishes the soft tissue and muscular forces on the easily moldable maxillary arch segments. Recently, the palatal appliance has been modified to include a nasal extension to help improve the nasal tip form. The orthodontist takes impressions, and the custom appliance is fitted as soon as possible after birth and well before the lip repair.

The appliance also aids in the child's oral feeding, helping to decrease nasal regurgitation and assisting oral suction. Some centers have chosen either no presurgical orthopedic intervention or an active pin-based appliance (eg, Latham) to align the maxillary arch segments. For bilateral clefts, external pressure is routinely used to help maintain the premaxillary component within the arch alignment. Soft elastic tape (eg, 3M Microfoam tape) across the premaxilla, a head cap with elastic traction, or lip adhesion can be used prior to a definitive lip repair once the arch segments have approximated.

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Intraoperative Details

The ideal lip repair results in symmetrically shaped nostrils, nasal sill, and alar bases; adequate columellar length; a well-defined philtral dimple and columns; a natural-appearing cupid's bow with a pout to the vermilion tubercle; and an adequate labial sulcus. In addition, lip scars are used to approximate the natural landmarks. The ideal repair results in functional muscle repair that with animation that mimics a normal lip.

Intraoperative technique. (A) The anatomic landmar Intraoperative technique. (A) The anatomic landmarks are tattooed and the planned incisions are marked. (B) The orbicularis is dissected from the overlying skin and divided into bundles to allow interdigitation with its opposing element. Inferiorly, an element of the muscle is left attached with the triangular vermilion flap used to create a Cupid's bow. (C) The prolabial flap is developed. The lateral lip elements of the prolabium are discarded and the mucosal flaps are turned over to create a labial sulcus. (D) The lower lateral cartilages are freed from the overlying nasal skin from the base of the ala and columella. The nasal domes are approximated to each other and the cartilages are suspended from the upper lateral cartilages. (E) The series of interdigitating bundles of the orbicularis muscle are approximated to each other. (F) The skin is inset with a series of fine nylon sutures, which are removed 5-7 days postoperatively if a skin adhesive is not used. Xeroform gauze bolsters are placed as a temporary nasal stent.

A number of surgical procedures with many variations for the repair of bilateral cleft lip are well described. Among these are the repairs of Veau, Tennison, Manchester, Millard, and others. The Veau III operation is a straight-line closure without elevation of the prolabial skin and correspondingly without any attempt at restoring the continuity of the orbicularis oris. The central cupid's bow and tubercle is constructed from the vermilion of the lateral lip elements. In contrast, Millard's repair involved complete elevation of the prolabium and reconstitution of the orbicularis across the premaxilla. In addition, Millard banked lateral segments of the prolabium as "forked flaps" that were meant to add columellar height at a later stage. As with Veau, the central vermilion is recreated from the lateral lip elements.

Unlike Veau and Millard, Manchester preferred to maintain the prolabial vermilion to create the cupid's bow and tubercle, but similarly to Veau, Manchester's repair did not involve repairing the orbicularis, as he felt this would create an overly tight lip. In recent years, significant contributions by McComb, Mulliken, Nakajima, and Cutting have integrated the correction of the associated nasal deformity with simultaneous lip repair that appears to achieve adequate primary columellar lengthening and nasal tip projection. [14, 15, 16, 17, 18, 19] More recently, McComb's experience led him to stage the repair. [14] The initial stage involves approximating and repositioning the splayed alar cartilages through a V-Y nasal tip "gull-wing" incision that allows redraping the overlying skin with a simultaneous bilateral lip adhesion. A definitive lip repair follows at a second stage.

Mulliken's extensive experience has evolved from a median nasal tip incision for exposure to bilateral rim incisions that allow adequate access to correct the nasal cartilage deformity. [20] Nakajima and Cutting have introduced presurgical molding of the nasal tip and columellar with acrylic outriggers attached to a palatal appliance. [18, 19] For the specific details of each of these repairs and supportive treatment, the reader is referred to more detailed references of the individual surgeons or more comprehensive texts cited in the Bibliography.

A cursory description follows of an operative technique the authors have used.

  • Use general anesthesia with a noncuffed Oral 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), tattoo the anatomic landmarks with a methylene blue dye and mark the proposed incisions.

  • Two key elements are involved in the preoperative skin marking for the elevation of two crucial flaps (the prolabial flap and lateral lip advancement flaps). Make the prolabial flap in a "tie" shape, with the peak of the Cupid's bow between 2-2.5 mm on either side of the midline. The flap narrows superiorly to the columella base. On the lateral lip element, the peak of the Cupid's bow is determined where the dry vermilion is maximal in width before it tapers off superiorly. Mark the incision of the lateral lip from the vermilion-cutaneous junction to the alar base, with medial flaps of vermilion included for recreation of the central tubercle. The incision continues vertically intranasally along the mucocutaneous junction. The vermilion of the lateral lip flaps will fit into the inferior edge of the prolabial flap and to each other in the midline, forming a tubercle. The white roll should be included with the vermilion flaps.

  • Elevate the prolabial flap to the base of the columella. The lateral cutaneous elements are primarily discarded, except at the columella base, where it is tailored to reconstruct the nasal sill (not as banked fork flaps). The mucosa is then turned down to create the labial sulcus.

  • Elevate the lateral lip flaps by incising the marking just above the white roll through the full thickness of the lip. Identify the orbicularis oris muscle and separate it from the overlying skin and underlying labial mucosa. The muscle is divided inferiorly to allow it to accompany the vermilion flap. Make an upper buccal incision above the attached gingival to allow medial mobilization of the labial mucosa.

  • Free the alar bases from their attachments to the piriform region to allow medial and inferior mobilization of the ala and the corresponding transversus nasi muscles (to prevent alar flare).

  • The lower lateral alar cartilages are freed from the overlying nasal skin through an infracartilaginous incision laterally and medially. The domes are approximated to each other with intradomal sutures, and the complex is suspended from the upper lateral cartilages with temporary fixation sutures.

  • Reconstructing the lip begins with creating the labial sulcus by approximating the labial mucosa of the lateral lip elements to the turned-over central prolabial mucosa. To approximate the orbicularis oris muscle, it is divided into bundles and interdigitated with its opposing element with a series of sutures. The alar bases are then set into place (inferior and medial) to the nasal spine. Approximate the prolabial skin flap and lateral lip flaps either with dermal sutures or in combination with an adhesive. The vermilion flaps are tailored to create a central tubercle. Tailor the flaps at the nasal sill and then close the alar and intranasal incisions.

  • Place the Xeroform bolsters and nasal stents. Apply a topical antibiotic ointment to the lip.

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Postoperative Details

See the list below:

  • Oral feedings: For a child who is breastfed, the authors encourage uninterrupted breastfeeding after surgery. Bottle-fed children can resume feedings immediately following surgery with a crosscut nipple. Some centers still advocate having the child use a soft catheter tip syringe for 10 days following surgery, followed by resumption of normal nipple bottle feeding. 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 pointed edges for 2 weeks after surgery. No other restrictions on activity are necessary. Some centers advocate the use of Velcro elbow immobilizers on the patient for 10 days following surgery to minimize the risk of inadvertent injury to the lip repair. These are periodically removed during the day under supervision.

  • Lip care: Any exposed suture line, at the base of the nose and lip, should be cleaned using cotton swabs with diluted hydrogen peroxide several times a day, followed by the liberal application of topical antibiotic ointment. The authors then remove any permanent sutures 5-7 days after surgery. If cyanoacrylate adhesive is used, no additional care is required in the immediate postoperative period until the adhesive film comes off. The parents are told to expect noticeable scar contracture, erythema, and firmness for about 4-6 weeks postsurgery and that this gradually begins to improve 3-12 months after the procedure. The authors typically instruct parents to massage the upper lip during this phase and to avoid placing the child in direct sunlight until the scar matures.

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Follow-up

Following cleft lip repair, patients are evaluated periodically by the various cleft team members. Oral hygiene and proper dental care need to be emphasized. Psychosocial evaluation and treatment should be made available. Follow-up appointments with speech pathologists should be continued until normal or near normal speech is achieved. Close cooperation among the members of the cleft team is necessary for optimal outcomes.

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Complications

Aside from an unsatisfactory appearance of the surgical result, possible complications include dehiscence of the repair, hypertrophic scar formation, or contracture of the lip scars. If dehiscence occurs, re-operation is postponed until the induration has subsided completely. In the intervening period, control of the premaxilla with orthodontic devices to prevent rotation caused by asymmetric forces may be needed. With lip scars that remain red, thick, and contracted, the authors use an occlusive tape dressing and, occasionally, Kenalog-10 (triamcinolone acetonide) injection and/or flurandrenolide tape. For most repairs, the observed contracture is part of normal healing and improves with time. Wait to perform intervention until the lip scar matures (generally 1 y), and the intervention should be guided by the severity of the residual deformity with the goal of minimizing the number of revisions.

A number of secondary deformities of a less than ideal outcome are well characterized. These primarily include the whistle deformity of the lip, a vertically deficient upper lip, a constricted lip, and muscular diastasis. Correction of these residual deformities must be specifically tailored and range from minor revisions to fully re-creating the defect and reconstructing each of the elements of the lip to discarding the prolabial element and replacing it with a lower lip Abbe reconstruction.

Preoperative and postoperative images of a child b Preoperative and postoperative images of a child born with a complete bilateral cleft lip and palate. Note that the prolabial width increases because of the tension. Ideally, the initial width should have been set narrower.
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Outcome and Prognosis

Although symmetry is perhaps more achievable in the bilateral cleft lip repair, the outcome can be less satisfying than with unilateral clefts. Careful preoperative assessment of the cleft lip deformity and attention to appropriate presurgical management and detail in the reconstruction typically results in an acceptable repair that achieves some of the characteristics of the natural lip and nose. 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, an excellent initial result may require some revision because of uncontrolled variables. Moreover, while the lip repair may be acceptable, additional procedures to achieve nasal symmetry are commonly required, despite the initial primary nasal surgery incorporated as an integral part of lip repair. Realistically, one must realize that, despite physicians' best attempts, the stigmata of a bilateral cleft deformity remains in many children. [21]

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Future and Controversies

Bilateral cleft lip surgery has evolved from discarding the premaxillary and prolabial elements in a simple approximation of the cleft margins to, currently, a definitive single-stage lip and primary cleft nasal repair that incorporates the underlying musculature. Accompanying the evolution of the surgical repair is the increasingly important involvement of early presurgical alveolar and nasal molding to possibly improve surgical outcome.

The basics of cleft surgery are to achieve a good philtrum size, shape, and positioning of the cartilages, and muscular continuity. Although the basics are the same, the development of presurgical techniques continue to evolve and provide an exciting scaffold to the management of bilateral cleft lip.

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