Bilateral Cleft Lip and Nose Repair
Philip Kuo-Ting Chen, M. Samuel Noordhoff
○ The prolabial width must be kept narrow without compromising the blood supply.
○ The prolabial columellar complex should be advanced superiorly to allow muscle reconstruction in front of the premaxilla.
○ Limited dissection should be performed on the maxilla above the periosteum.
○ The nasal floor can be reconstructed with mucosal flaps.
○ The prolabial buccal sulcus can be reconstructed with prolabial mucosal flaps.
○ Maintain the continuity of orbicularis muscle and attach it to the anterior nasal spine.
○ Cupid’s bow is reconstructed with tissue from the lateral lips.
○ The height between the central and lateral lip should be balanced without creating incisions around the ala.
○ The presurgical nasolabial angle should be maintained when narrowing the nasal width.
○ The columella should be preserved and elongated, and the lower lateral cartilages repositioned, with overcorrection of the columellar height and nasal width.
The key deformities of a bilateral cleft lip are (1) a protruding or deviated premaxilla with relatively retropositioned lateral maxillary segments, (2) wide or asymmetrical alveolar gaps, and (3) a short columella with increased nasal width (Fig. 46-1).
Approximating the orbicularis muscles in front of the protruding premaxilla not only increases the risk of wound dehiscence but also places excessive tension on the premaxilla and maxilla, which will eventually cause maxillary retrusion or twisting of the premaxilla. The typical nasal shape after primary lip repair in a patient with bilateral cleft lip without nasoalveolar molding (NAM) is a wide, flat nose with inverted nostril axes and a broad nasal tip caused by the short columella and skeletal imbalance between the premaxilla and lateral maxillary segments (Fig. 46-2).
Fig. 46-1 The typical appearance of a bilateral complete cleft lip and palate. A, The frontal view shows the small prolabium and short columella. B, The worm’s eye view shows the protruding and twisting premaxilla and wide and asymmetrical alveolar gap.
Fig. 46-2 The typical nasal appearance of a repaired bilateral cleft lip without primary nasal reconstruction, with a persistent short columella and wide and flat nose with inverted nostril axes.
Because presurgical NAM is not available in most parts of the world, the more common approach was traditionally a two-staged operation with reconstruction of the lip in early childhood followed by secondary nasal reconstruction, mainly focused on elongation of the columella, at various older ages.1-4 With the help of presurgical NAM to balance the skeletal base and elongate the columella, more recent approaches aim to achieve good results in both the lip and nose in one stage. Mulliken,5,6 Trott and Mohan,7 and Cutting et al8 reported different techniques to achieve a more satisfactory one-stage repair. Most of the recent one-stage repair techniques rely on presurgical NAM to stretch the columella before the operation. The technique used in the Chang Gung Craniofacial Center has undergone a continuous evolution during the past 30 years.9–12 The most recent method used at the Center is an integrated approach with presurgical management, surgical refinements, and postsurgical maintenance.
GENERAL PLAN OF MANAGEMENT
In the Chang Gung Craniofacial Center, the initial visit is made as soon as possible after birth. An orthodontist and a plastic surgeon examine the infant and record tissue deficiencies and tissue distortion. The double-Y numbered classification system, created by Noordhoff 13 in 1990, is used to classify the cleft (Fig. 46-3). This classification provides a numeric means of defining clefts, from the most minor ones to the most severe. Baseline records, including casts and standard-view photographs, are also performed at this initial evaluation.
Presurgical NAM is initiated at the first visit. This process takes 3 to 4 months to achieve an optimal outcome, at which time lip repair is performed. The timing of the lip repair is also dependent on the general nutrition and growth of the infant. At approximately 12 months of age the palate is repaired and, if indicated, grommet tubes are inserted. Speech is assessed beginning at 2.5 years of age. If required, speech therapy is started at 3.5 years of age. If velopharyngeal insufficiency is diagnosed, it is confirmed by nasoendoscopy at 4 years of age and corrected before the child begins school. Residual alveolar clefts are closed before canine teeth erupt, usually around 9 to 11 years of age. If the patient experiences any psychological distress related to residual lip or nasal deformity, a revision is performed as needed.
Fig. 46-3 The double-Y numbered classification. The numbers 1 to 9 represent a right-side cleft; the numbers 11 to 19 represent a left-side cleft. The number 10 represents a submucous cleft palate.
The patient is reexamined a few days preoperatively. Tissue deficiency, distortion, disproportion, and asymmetry are carefully recorded for surgical planning and to help in assessing postoperative results.
MANAGEMENT ALGORITHM: INTEGRATED APPROACH FOR LIP AND NOSE REPAIR
The integrated approach for lip and nose repair consists of presurgical management, surgical refinements, and postsurgical maintenance. The purpose of the NAM is to lengthen the columella, restore the normal shape of the cartilaginous framework, and balance the skeletal base. The surgical technique includes reconstruction of the nasal floor with various mucosal flaps, anatomic restoration of the orbicularis muscle, and reconstruction of the Cupid’s bow with a narrow central lip and tissue from the lateral lips. The initial surgery also focuses on primary nasal correction with alar base repositioning, cartilage repositioning, lengthening of the columella, and restoration of a good nasal height-to-width ratio through overcorrection. Postsurgical maintenance includes micropore tape and silicone sheeting for lip scar care and silicone nasal conformers to maintain the overcorrected nasal reconstruction.
Several techniques have been used in the Chang Gung Craniofacial Center for presurgical management during the past 25 years, including retraction of the premaxilla with tapes or elastics, presurgical orthopedics with acrylic plates and lip taping, and three different NAM techniques ranging from very simple to very sophisticated.9,14–17 All of the techniques need to be initiated in early infancy (4 to 6 weeks after birth). Among the three techniques, modified Grayson’s and Liou’s techniques were more commonly used. Both molding techniques require follow-up adjustments every 1 to 2 weeks.
Modified Grayson’s Technique
In the modified Grayson’s technique, a passive type of orthopedic appliance is used together with lip taping for alveolar molding. Alveolar molding is initiated first to narrow the alveolar gaps and mold the premaxilla into a proper position. After the alveolar gaps are sufficiently narrowed to 5 mm and the arch is better aligned, a nasal molding device is added to the orthopedic appliance. The nasal molding device lengthens the columella and reshapes the alar domes. Tape across the upper lip also acts as a lip adhesion that decreases the nasal width (Fig. 46-4).
The device used for Liou’s technique is composed of a dental plate, nasal molding components, and several micropore tapes. The dental plate is held to the palate with dental adhesive. Micropore tape is placed across the cleft to minimize the alveolar cleft, retract the premaxilla, and pull both alar bases medially. The nasal components are projected forward in a sagittal direction from the dental plate. Columella lengthening is achieved by the combined forces of backward movement of the premaxilla and forward movement of the nasal tip (Fig. 46-5).
To decrease the burden of care for the caregivers, a new molding device using a spring mechanism was developed by modifying the Liou technique. The molding prongs are made from 0.032-inch beta titanium wire with a helix. This device requires only four visits before lip repair can be performed, and thus can significantly decrease the number of follow-up visits. This can be very important in less-developed regions or for patients who live far from the treatment center (Fig. 46-6).
Fig. 46-4 A modified Grayson’s device for presurgical nasoalveolar molding.
Fig. 46-5 A Liou’s device for presurgical nasoalveolar molding.
Fig. 46-6 NAM device with a spring mechanism.
The following surgical concepts are essential for optimal outcome:
- Keep the prolabial width narrow without compromising the blood supply.
- Advance the prolabial columellar complex superiorly to allow muscle reconstruction in front of the premaxilla.
- Perform limited dissection on the maxilla above the periosteum.
- Reconstruct the nasal floor with mucosal flaps.
- Reconstruct the prolabial buccal sulcus with prolabial mucosal flaps.
- Create continuity of the orbicularis muscle and attach it to the anterior nasal spine.
- Reconstruct the Cupid’s bow with tissue from the lateral lips.
- Balance the height among the central and lateral lips without incisions around the ala.
- Maintain the presurgical nasolabial angle, and narrow the nasal width.
- Preserve and elongate the columella by repositioning the lower lateral cartilages (LLCs) with overcorrection of the columellar height and nasal width.
Marking and Measurement of the Central Lip
The landmarks of the lip are marked out on the prolabium and both lateral segments, and various measurements are taken and evaluated for possible asymmetry. On the prolabium, the width between the proposed peaks of the Cupid’s bow (cleft philtrum horizontal right [point cphr] and cleft philtrum horizontal left [point cphl]) is designed at 4 mm. The vertical limbs are straight lines on the prolabium from points cphl and cphr, narrowed to a 3 mm width at the base of the columella and never extended lateral to the columella. The height of the central segment is determined by the original height of the prolabium.
The central lip tends to become horizontally wide with time, even with good muscle approximation. The key factor to achieving a normal-looking Cupid’s bow is to keep the central lip narrow. Two columellar arteries branch from the superior labial artery and travel to the nasal tip. A width of 3 mm at the columellar base will include both columellar vessels and thus provide a good blood supply to the philtrum. A width of 4 mm between points cphr and cphl will give a properly proportioned central lip philtral segment. A philtral width greater than 4 mm results in an unnaturally wide Cupid’s bow.
Two forked flaps are designed on the sides of the base of the columella. The lower incisions of the forked flaps are perpendicular to the vertical limbs of the prolabial flap and extend laterally to the skin-mucosal junction on the premaxilla. This incision then turns upward along the skin-mucosal junction behind the columella and extends up to the alar dome. These forked flaps are much smaller compared with the forked flaps originally advocated by Millard.18 The prolabial tissue, lateral to the vertical limbs of the philtrum and inferior to the forked flaps, can be left attached to the prolabium and deepithelialized, increasing the tissue bulk lateral to the vertical limbs and enhancing the philtral column as advocated by Mulliken.5,6
Millard18 suggested banking the forked flaps in the nasal floor. However, using banked forked flaps for secondary nasal revision will often create unsightly scarring on the nasal floor and the nasolabial junction. We prefer to design a smaller forked flap and suture the tips backward toward the septum to restore the nasolabial angle.
The lower margin of the philtral incision is placed above the indistinct white skin roll (WSR) with the points cphr and cphl higher than the point labiale superius (LS) for the shape of the Cupid’s bow. The vermilion and mucosa below this incision will be designed as an inferiorly based mucosal flap for deepening of the central sulcus, and two laterally based prolabial mucosal (PM) flaps will be created for reconstruction of the nasal floor.
Marking and Measurement of the Lateral Lips
The proposed base of the lateral philtral column on the lateral lips (points cphr’ and cphl’) are marked at the point where the vermilion first becomes widest and is usually 3 to 4 mm lateral to the converging junction of the red line and WSR.19 The points should be placed on the upper margin of the WSR. The horizontal length from this point to the commissure is usually 13 to 15 mm. The vertical and horizontal lengths of the lips on both sides are measured to identify any asymmetry in the vertical and horizontal dimensions. The incision line on the lateral lips is extended along the free margin of the lip, at the upper margin of the WSR, forming a WSR-free border flap. Approximately 4 to 5 mm of WSR, medial to point cphr’ and cphl’, is retained and will be used for Cupid’s bow reconstruction (Fig. 46-7).
Fig. 46-7 Preoperative markings. Point LS (1) is the central point of the Cupid’s bow. The lateral points of the Cupid’s bow (cleft philtrum horizontal right [point cphr; 2] and cleft philtrum horizontal left [point cphl; 3]) are designed to keep the total width of the Cupid’s bow at a distance of 4 mm. The vertical limbs extend to the base of the columella with the base narrowed to 3 mm wide. Points cphr’ (2′) and cphl’ (3′) are the anatomic points for the base of the philtral column on the lateral lip. These points are approximately 3 to 4 mm lateral to the converging red line (the junction of dry vermilion and wet mucosa) and the white skin roll (WSR). This point is usually where the vermilion first becomes widest. The prolabial mucosal flap (PM) is used for reconstruction of the nostril floor. The central part of the prolabial mucosa is used for lining the premaxilla. (LS, Labiale superius.)