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The modern technique of presurgical orthopedics and nasoalveolar molding produces a better skeletal foundation and nasal shape for the repair of the bilateral cleft lip-nasal deformity. The general principles are as follows: (1) preserve the presurgical columellar length; (2) keep the width of the central lip segment narrow without compromising the blood supply; (3) advance the columella prolabium complex superiorly to allow reconstruction of the orbicularis oris muscle behind the prolabium; (4) release the alar cartilage attachment from the pyriform rim and provide additional coverage of this soft tissue deficiency with the use of inferior turbinate flaps; (5) release and reposition the lower lateral cartilage; (6) adequately dissect above the maxillary periosteum; (7) reconstruct the nasal floor by local mucosal flaps; (8) reconstruct the prolabial buccal sulcus with tissue from the prolabium; (9) reconstruct the orbicularis muscle sphincter and attach it to the anterior nasal spine; (10) reconstruct a new Cupid's bow, central vermilion, and lip tubercle with tissue from the lateral lips; (11) balance the height of both lateral lips without any incision around the ala; and (12) maintain the presurgical nasolabial angle. The residual nasal deformity remains a problem that needs further improvement. The long-term result in Chang Gung Craniofacial Center suggests overcorrection of columella height before, during, and after lip repair.
The objective for surgical correction of the bilateral cleft lip is to reconstruct a symmetrically balanced lip and nose with good columellar length. The most common approach is a two-stage correction with columella elongation as a secondary procedure at the age of 1 to 5 years.1,2,3,4,5,6,7,8,9,10 Noordhoff,11 in 1989, reported a one-stage reconstruction with microscopic dissection of the prolabium as an island pedicle flap and interdigitation of the two-forked flap between the columella and prolabium for primary elongation. It was abandoned because it was technically too complicated. Mulliken12,13 trimmed the forked flap and reconstructed the nose with intranasal and nasal tip incisions that allowed approximation of the splayed lower lateral cartilages for accentuation of the columella. Trott and Mohan14 advocated an open rhinoplasty approach raising the nasal tip with the prolabial flap for approximation of the alar domes. Cutting et al15 used presurgical nasoalveolar molding to stretch the columella to achieve a more satisfactory one-stage repair. Millard et al5 advocated aggressive, active presurgical orthopedics, gingivoperiosteoplasty, and lip adhesion along with a forked flap elongation of the columella. Most of the recent techniques that achieve a better result use the technique of presurgical orthopedics or nasoalveolar molding to stretch the columella presurgically and try to maintain or further lengthen it surgically.
There are many possible variations of the bilateral cleft lip. The morphology can vary from being complete on both sides to asymmetric with a complete cleft on one side and incomplete on the other. Kernahan's “striped Y” method16 cannot fully illustrate the range and diversity of the asymmetric cleft. The double-Y numbered classification, reported by Noordhoff in 1990,17 is a more accurate method for recording as well as a more suitable system for computer database documentation (Fig. 1). For patients with a complete cleft of primary and secondary palate on one side and a complete cleft of the secondary palate on the other side, this classification can record the pathology in a more accurate way than the single striped Y classification.
There is a wide variation in the quality and amount of tissue in the prolabium, premaxilla, nasal cartilages, vomer, and lateral lip elements.18,19,20,21,22 All bilateral clefts have some amount of asymmetry in their horizontal or vertical dimensions.23 All cleft patients have a certain amount of tissue deficiencies. These deficits are most severe in bilateral medial facial dysplasia patients24 (Fig. 2), who, therefore, have a less than optimal outcome after lip and nose repair. They always have a significant growth disturbance and require orthognathic surgery when they reach skeletal maturity. It is important to document these deformities or any preexisting asymmetry, or both, prior to surgery to assess the postoperative results more accurately.
Presurgical orthopedics, nasoalveolar molding, is started on the first visit. The aim of this molding process is to centralize the premaxilla, narrow the alveolar gaps, match the alar cartilages, and elongate the columella. This process usually takes 3 to 4 months to achieve an optimal outcome. The initial surgery is usually performed at 3 to 4 months of age, depending on the result of the molding process. The palate is repaired at about 12 months of age together with the insertion of grommet tubes. Speech assessment is started at 2.5 years. If the patient requires speech therapy, it starts at 3.5 years. Velopharyngeal insufficiency is diagnosed by nasoendoscopy at 4 years old and corrective surgery for velopharyngeal insufficiency is performed as soon as the diagnosis is made. Residual alveolar clefts are closed before the eruption of canine teeth, usually when the child is 9 to 11 years old. If the patient has any psychological problems related to any residual lip or nasal deformity, a revision surgery is usually done before the child enters primary school.17
The purpose of presurgical orthopedics or nasoalveolar molding is to restore a more normal nasal shape and a balanced skeletal base. The following techniques have all been used in Chang Gung Craniofacial Center for the past 20 years.
The protruding premaxilla may be gradually pushed back by applying micropore tapes across the lip with or without traction rubber bands.25 It is suggested that the patient sleep in either the prone or side-lying position to increase pressure on the cheeks. The movement of the alveolar segments is controlled by an acrylic plate. This simple technique is effective in expanding the prolabial tissue and places the premaxilla in a better position.26
A silicone nasal conformer can be used as a tool for presurgical nasal molding when the patient has an incomplete cleft lip.27 The height of the conformer can be adjusted by gradually adding some soft resin or flat silicone sheets on the domes (Fig. 3).
A passive-type orthopedic appliance is used together with taping of the lip for premaxilla and alveolar molding. The protruding premaxilla is molded first into a proper position. When the alveolar gap is approximated and the arch is aligned, a nasal molding device is added to the orthopedic appliance to increase the columellar length as well as to reshape the alar dome. A nonsurgical lip adhesion is performed by placing tape across the upper lip. The tape aids in the closure of the clefts, decreases the width of the base of the nose, and helps to approximate the lip.28
Alveolar molding and nasal molding are performed simultaneously using an acrylic plate with rigid acrylic nasal extension. Rubber bands are connected to the acrylic plate for gentle retraction of the premaxilla backward. A soft resin ball attaching to the acrylic plate across the prolabium is sometimes used to maintain the nasolabial angle29 (Fig. 4).
The nasoalveolar molding device is composed of a dental plate, two nasal components for nasal molding, and several micropore tapes for premaxillary retraction. Denture adhesive (Poligrip, Australia) keeps the dental plate on the maxillary lateral segments. The nasal components are made up of 0.028-inch stainless steel wire projecting forward and upward bilaterally from the anterior part of the dental plate. The top portion contains a soft resin molding bulb that fits underneath the nasal cartilages for nasal molding. Micropore tapes are placed across the cleft lips and prolabium to minimize the alveolar cleft and retract the premaxilla. At the same time, they pull both alar bases medially. Retraction of the premaxilla and lengthening of the columella are performed at the same time. The columella is lengthened and stretched by pulling on the premaxilla backward. The nasal tip is kept at the same height while the premaxilla is pulled back. Rather than pushing forward, the soft resin molding bulbs basically support the nasal cartilages and nasal tip30 (Fig. 5).
The key point of nasal molding in bilateral clefts is to push the alar domes forward in a sagittal direction for columellar lengthening instead of pushing the domes upward in a cephalic direction into a turned-up nasal tip. Nasoalveolar molding techniques require regular patient follow-up with an interval of 1 to 2 weeks. Grayson's technique approximates the alveolar cleft before the nasal molding. Both Figueroa's and Liou's method achieve nasal and alveolar molding at the same time.
There are several surgical principles that need to be stressed. They are as follows: (1) preserve the presurgical columellar length; (2) keep the width of the central lip segment narrow without compromising the blood supply; (3) advance the columella prolabium complex superiorly to allow reconstruction of the orbicularis oris muscle behind the prolabium; (4) release the alar cartilage attachment from the pyriform rim and provide additional coverage of this soft tissue deficiency with the use of inferior turbinate flaps; (5) release and reposition the lower lateral cartilage; (6) adequately dissect above the maxillary periosteum; (7) reconstruct the nasal floor by local mucosal flaps; (8) reconstruct the prolabial buccal sulcus with tissue from the prolabium; (9) reconstruct the orbicularis muscle sphincter and attach it to the anterior nasal spine; (10) reconstruct a new Cupid's bow, central vermilion, and lip tubercle with tissue from lateral lips; (11) balance the height of both lateral lips without an incision around the ala; and (12) maintain the presurgical nasolabial angle.25,31
The landmarks of the lip are marked out on the prolabium and both lateral segments. The various vertical and horizontal measurements are evaluated for any asymmetry. The width between CPHL and CPHR is usually maintained at 5 to 6 mm. The central segment is gradually narrowed toward the columellar base and maintained at 4 mm in width at the level of the columellar base. Traction applied to the alae is usually needed to identify the nasolabial junction. The incision lines are kept straight, not curvilinear. The proposed peak of the Cupid's bow on the lateral lips (CPHR' and CPHL') is marked at the point where the vermilion first becomes widest and usually would be 13 to 15 mm from the commissure or 3 to 4 mm lateral to the converging junction of the red line and white skin roll (WSR)(Fig. 6).
A double hook is used to retract the columella up, and a small single hook is used to stretch the prolabium. The central segment is developed by laying a number 11 blade on the incision line of the prolabium to give a straight cut. The two forked flaps are developed with lateral incisions on the skin-vermilion junction extending behind the columella up into the membranous septum and continuing up along the skin-mucosa junction to the dome area, then along the lower border of the lower lateral cartilages (LLCs) as a gull wing open rhinoplasty incision or outside the alar rim as a Trott incision (Fig. 6, insert). The central segment, the forked flap, and the columella are raised as a unit to expose the cartilaginous framework. The central part of the vermilion and mucosa of the prolabium is used for the lining of the raw surface on the premaxilla. The lateral parts of the prolabial mucosa flaps (PM flaps) are used for nasal floor reconstruction (Figs. 7 and and88).
The incision is made from the proposed peak of Cupid's bow along the cleft edge to the edge of the alveolar cleft. The incision is right above the WSR to develop a WSR–vermilion–free border flap. This flap will be used for reconstruction of the central Cupid's bow. An L-mucosal flap is raised along the cleft edge. The incision is then turned upward along the pyriform rim and then around the inferior turbinate to be incorporated with the inferior turbinate flap. The dissection is carried above the periosteum on the maxilla. The abnormal muscle insertion on the lateral segment is released adequately until the lateral segment can be brought medially to touch the medial segment without tension. The cleft edge is then opened to develop the WSR–vermilion–free border flap. The dissection on the mucosal side is limited to 2 mm, and the dissection on the skin side is quite extensive to separate the abnormal muscle insertion from the skin. The dissection is carried below the alar base to release the abnormal muscle component that inserts to the alar base (Fig. 7).
The inferior turbinate flap is used to fill in the defect on the pyriform area after the LLCs are advanced. The turbinate and L flaps are sutured together, brought across the cleft, and sutured to the septal incision to reconstruct the nasal floor. Special attention must be focused on the width of the nostril. The PM flap is sutured below the L-flap for lining. The orbicularis muscles are approximated with 4-0 polyglactin sutures with the upper edge sutured to the anterior nasal spine (Figs. 9 and and1010).
The separated LLCs are approximated by absorbable sutures, 5-0 polydioxanone, or nonabsorbable sutures, 5-0 polypropylene, depending on the surgeon's preference (Fig. 8). The fibrofatty tissue on the nasal tip is brought to the top of the approximated nasal tip. The skin flap of the central segment is then sutured to the lateral lip. Through-and-through alar transfixion sutures are placed on the alar-facial groove to provide further support to the LLCs (Fig. 11, insert). The excessive tissue on the nasal floor is adequately trimmed and the floor is closed. The full-thickness WSR, vermilion, and free border flap are brought together below the central segment to reconstruct the central lip. Excessive orbicularis marginalis muscle on the tip of the WSR–vermilion–free border flaps is preserved for augmentation of the lip tubercle (Fig. 11).
The wounds on lip and nose are covered by antibiotic ointment without any dressing. The sutures are removed 5 to 7 days after surgery at the outpatient clinic. The lip scar is supported by micropore tapes as well as silicone sheets for 6 months. A silicone nasal splint is needed for 6 to 9 months. Throughout this period, the height of the splint is gradually increased by adding silicone sheets to the domes of the splint. The central prolabial portion of the lip will gradually widen and lengthen by the age of 3 years. The nasal width will also increase, similar to the central prolabial portion of the lip width. The columella length will shorten slightly after the primary lip repair and then remain stable without further growth, while the rest of the nose will grow significantly in both height and width. This results in a relative relapse appearance of the columella.30 Figure Figure1212 shows a series of photographs demonstrating the effect of presurgical nasoalveolar molding and the postoperative changes of the nasal shape.
The different techniques of alveolar or nasoalveolar molding are used in Chang Gung Craniofacial Center. Grayson's technique, with emphasis on approximating the alveolar clefts before nasal molding, achieves the best preoperative nasal shape symmetry and skeletal base balance. However, it is also the most expensive and time-consuming method. Figueroa's and Liou's techniques of performing alveolar and nasal molding at the same time are simpler and less expensive methods. A study32 comparing the three techniques in unilateral clefts showed that the latter two techniques tend to result in a larger diameter in the cleft side nostril postoperatively.
Millard, Mulliken, and Cutting5,12,13,15,33 all advocated the importance of primary gingivoperiosteoplasty. The long-term result from Cutting and Grayson's report34 showed that 60% of the patients who received primary gingivoperiosteoplasty do not need alveolar bone grafting later on. However, it is very difficult to perform a primary gingivoperiosteoplasty unless the alveolar gap is around 1 to 2 mm. Figueroa's and Liou's techniques tends to leave the alveolar gap larger, 3 to 4 mm, which limits the possibility of a primary gingivoperiosteoplasty.
There is a significant difference in the outcome of the shape of the central lip in the bilateral cheiloplasty with or without muscle approximation. In the technique without muscle approximation, the central lip tends to become wider and remains short. With muscle approximation, the central lip segment has less widening but more lengthening. Mulliken12,13 advocated narrowing the central lip width down to 2 to 3 mm for a better long-term result. Noordhoff,11 in attempting a primary elongation of the columella by interdigitating the forked flaps into a transverse incision in the columella, found two vessels running from the columella to the prolabium. A central segment that is 2 mm wide at the columellar base may injure the vessels. A 4-mm-wide base of the prolabium includes both columellar vessels, providing a good blood supply to the prolabium. The long-term result shows a tendency of widening as well as lengthening of the central segment. A wide central segment in primary lip repair, although maintaining a good blood supply, may result in an unnaturally wide Cupid's bow.
Regarding the vertical height of the central lip, Lee35 advocated that the vertical height of the central lip in a 3-month-old baby should be around 7 mm. However, the height is somewhat predetermined by the size of the prolabium. A prolabium shorter than 7 mm should not be lengthened on the operating table, as it will always lengthen vertically with muscle approximation and have a satisfactory appearance. A relatively longer prolabium can provide additional tissue for the columella and make it easier to achieve a better nose. The critical problem in determining the vertical height of the lip occurs when there is a marked discrepancy between the vertical height of the central lip and the lateral lips. In this situation, the surgeon should vertically shorten the lateral lip to match the vertical height of the central prolabial portion of the lip. Otherwise, the nasal tip will be pulled downward because of the tension in the central segment. Even a short vertical length of 4 to 5 mm will elongate adequately with muscle repositioning.
Millard suggested preserving the prolabial tissue lateral to the central segment as forked flaps that are banked on the nasal floor. These banked forked flaps are used for columellar lengthening in secondary revisions. The experience in Chang Gung Craniofacial Center does not support his concept in Oriental patients. Pigott36 studied the ratio between dome component and columellar component in Caucasians of varying ages. The dome columellar ratio is much greater in Orientals than in their Caucasian peers. A nose with a disproportionately long columella often results after a columellar elongation procedure using the banked forked flaps. The banked forked flaps also end up with unsightly scarring on the nasal floor. The authors do not bank these forked flaps. They are trimmed to an adequate size and sutured backward to the septum to improve the nasolabial angle. The report from Nakajima et al suggested a similar approach.37
Cutting et al15 raised the central segment tissue behind the medial crura of LLCs and reported that it has a safer blood supply to the prolabium. Trott and Mohan14 used a technique of raising the central segment in front of the LLCs. The Chang Gung experience comparing the two techniques shows that there is no difference in terms of blood supply to the central prolabium between these two techniques. Cutting and Noordhoff believe that the medial crura need to be elevated superiorly on the septal cartilage, and Trott and Mulliken leave the LLCs attached to the septum. In the authors' experience, the two techniques offer a similar early result. Technically, the retrograde dissection and approximation as advocated by Cutting is more difficult compared with the technique of approximation of cartilages under direct vision as advocated by Trott.
In his anatomical dissection around the nasolabial angle, Wu38 showed that the angle is maintained by a ligament from the subcutaneous tissue to the anterior nasal spine. Whenever the columella-prolabium complex is raised, the nasolabial angle tends to be flattened after operation. However, this procedure is definitely necessary as the separated orbicularis muscles need to be approximated under the prolabium to achieve an anatomical repair. The technique with the placement of the incision behind the medial crura tends to maintain a better nasolabial angle postoperatively than the technique with the incision located in front of the medial crura. Restoration of the ligament by a tuck-down suture from skin to anterior nasal spine may jeopardize the blood supply to the prolabium. Suturing the tips of the forked flaps backward to the septum may be more helpful in maintaining the nasolabial angle.37
The approximation of the LLCs can be achieved through either an open or closed rhinoplasty. The authors' experience shows a similar result with the two techniques. Technically, a closed rhinoplasty with two rim incisions or bilateral Tajima39 incisions is simpler than the open rhinoplasty through a gull wing or Trott incision. However, an open gull wing incision, approximating the LLCs through direct vision with nonabsorbable sutures, is the author's preferred method. This allows the surgeon better visualization for accurate approximation of the LLCs. The open technique also provides a better approach for redraping or redistributing the central segment tissue.
Friede et al40 used a postoperative acrylic molding splint to improve nasal configuration. Other reports used a similar concept for postoperative maintenance.27,41,42,43,44,45 In Chang Gung Craniofacial Center, a silicone conformer is routinely used after surgery and proved its efficacy in maintaining the postoperative nasal shape in unilateral clefts46 as well as in bilateral clefts. It is necessary to use the splint for at least 6 months postoperatively while waiting for scar maturation.
Delaire47 suggested wide subperiosteal dissection on the maxilla to achieve a functional closure. There is still controversy about whether a subperiosteal or supraperiosteal muscle dissection is better in terms of function or subsequent facial growth. There are no scientific data supporting the concept that a subperiosteal dissection results in less scarring or better facial growth. A muscle dissection above the periosteum seems to offer a better release of the abnormal muscle insertion around the alar base from both the skin side and periosteal side. The technique presented here keeps the extent of muscle dissection as minimal as possible but still adequate for muscle approximation at the center. This should create minimal scarring or muscle tension in front of the maxilla.
Manchester48,49 felt that the orbicularis muscle should not be reconstructed as it would cause too much tension and growth disturbance. Nagase et al50 showed that there was no significant growth disturbance after muscle reconstruction. There is a definite difference in the appearance of the bilateral lip repair with or without muscle reconstruction. Muscle reconstruction produces a much better result both functionally and aesthetically.
The techniques leaving the prolabial WSR and vermilion on the prolabium to reconstruct the Cupid's bow result in a Cupid's bow with abnormal peaking, indistinct prolabial WSR, and irregular vermilion with a depressed scar at the central lip. The quality of the WSR from the lateral lips is much better compared with the WSR of the prolabium. The reconstruction of the central lip by advancing the WSR–vermilion–orbicularis marginalis flaps from the lateral lip beneath the prolabial segment gives a continuous WSR, underlying vermilion, parallel red , and a full central prolabial tubercle without notching.31,51,52
From the experience in unilateral cleft lip repair,53 the horizontal incision below the nasal floor is usually unnecessary. Nevertheless, the alar-facial groove has a better appearance if the skin is kept intact. The surgeon needs only to approximate the orbicularis muscles. However, in the presence of a vertical discrepancy between the central lip and lateral lips, a horizontal incision below the nasal floor may be needed. The lateral incision is used for shortening of the longer lateral lip.
The long-term results in nasal reconstruction usually give an impression of relapse of the nasal shape. However, studies of long-term results for both unilateral and bilateral clefts by photometric measurements with 1:1 photographs show that there is a tendency of increase of the nostril width even when the nostril height is maintained postoperatively. This condition gives an impression of relapse.30,54 These studies show the importance of overcorrection of the nostril height and nostril width during surgery and the need to maintain the nostril shape in an overcorrected position postoperatively. There are no scientific data suggesting the amount of overcorrection during surgery. The postoperative maintenance can be achieved by gradually adding silicone sheets or soft resin to the nasal splint to increase the nostril height.
Presurgical orthopedics and nasoalveolar molding provide the surgeon with a much better skeletal foundation for reconstruction of the bilateral cleft lip. The goal is to restore all the displaced tissue, reconstruct the dynamic sphincter, reconstruct the Cupid's bow with tissue from the lateral lips, overcorrect the nasal width, maintain and further elongate the columella during surgery, and maintain the columellar height postoperatively. Reconstruction of the bilateral cleft lip-nasal deformity remains a challenging task. The results vary with any technique as there is a wide range of tissue deficiencies that are also factors in achieving a successful result.