Unilateral Cleft Lip Repair Treatment & Management
- Author: Pravin K Patel, MD; Chief Editor: Jorge I de la Torre, MD, FACS more...
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.
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. 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.
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,[11, 12] 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. See the images below.
Each of these techniques ultimately has the common goal of achieving symmetry and restoring the continuity of the underlying orbicularis muscle. All attempt to lengthen the foreshortened philtrum on the cleft side by interposing tissue from the lateral lip element into the medial lip element through various combinations of rotation, advancement, and transposition flaps.
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. 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. See the image below.
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. See the image below.
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. See the image below.
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. See the image below.
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.
In a study of 62 patients who underwent unilateral cleft-lip nasal repair, Sherif demonstrated some improvement in alar contour and symmetry using a modification of the Millard flap. This modification involved lifting a small subcutaneous alar base flap from the nasolabial region, which was then turned over to bulk up the maxilla and raise the depressed ala.
See the list below:
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.
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. 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:
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.
In a study, Bonanthaya et al concluded that in repair of complete unilateral cleft lip, the greater the size of the alveolar defect, the greater the amount of vermilion asymmetry. The study included 20 patients, aged 6-18 months.
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.[11, 16] 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. However, new approaches and modifications are always on the horizon.
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. 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.
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