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Semin Plast Surg. 2005 November; 19(4): 313–328.
PMCID: PMC2884747
Cleft Lip Repair: Trends and Techniques
Guest Editor Joseph K. Williams M.D., F.A.C.S., F.A.A.P.

Unilateral Cleft Lip—Approach and Technique

ABSTRACT

This article presents the philosophy, technique, and personal and team approach for treating children with unilateral cleft lip-nose deformities based on the senior author's 36 years of experience. The treatment of unilateral cleft lip almost without exception must involve correction of the nasal deformity. To obtain excellent results, the surgeon must integrate technique, teamwork, and timing based on multidisciplinary protocols developed over the years. Technique must involve broad dissection of the nasal and lip elements off the abnormal skeletal base and delicate but wide dissection of the nasal cartilage to achieve contour and tip projection. The team must include and assimilate surgical, orthodontic, speech, and when necessary orthognathic viewpoints to achieve the optimal result. The final goal is to bring the child to normal facial appearance at conversational distance. It is important to realize that the treatment of the unilateral cleft is rarely one procedure at one time but rather a culmination of several interventions precisely timed in the growing phase of the child from infancy to adulthood.

Keywords: Unilateral cleft lip, cleft lip, cleft lip and nose deformity, cleft orthognathics, cleft orthodontics

The term unilateral cleft lip is almost a misnomer because nearly always the nose is an integral part of the problem that must be addressed to obtain an improved result. Based on more than three decades of experience, this article presents the philosophy and the global approach to the treatment of the child with the unilateral cleft lip. There are several key elements that must be considered to obtain the desired result. First, the nasal involvement is almost ubiquitous and therefore must be addressed primarily at the time of lip surgery. Second, the treatment should always be multidisciplinary, involving the cleft surgeon, orthodontist, and speech pathologist when the palate is involved. Third, the problem confronted is dynamic over the time of the child's development, and therefore protocols spanning a time period (Tables 1 and and2)2) rather than one-time “home run” solutions are the rule rather than the exception.

Table 1
Surgical Protocol
Table 2
Orthodontic Protocol

The long-term protocols have evolved gradually over the years and consistently produce good to excellent results. Passive perisurgical orthopedics is applied at 2 weeks of age. Other than the use of the passive device, the abnormal skeletal base is mainly ignored at this stage and emphasis is put on the soft tissue repair of the cleft lip/nose complex. This is the most important surgical stage in primary cleft lip/nose repair. When the palate is involved, two-flap palatoplasty is performed at 8 months of age. Approximately 35% of the authors' patients need minor secondary correction of the lip and/or nose, generally performed at preschool age, around 5 years old. Definitive rhinoplasty is performed in most cases at or after completion of growth. Palatal expansion is performed at 5.5 years of age, followed by cancellous iliac bone grafting when one third to two thirds of the tooth's root has developed but prior to its eruption into the cleft void. In our experience, bone grafting at this stage provides enough bone for the orthodontist in 95% of the cases to achieve orthodontic restoration. An open airway promotes normal facial growth; therefore, limited septoplasty and turbinectomy are performed as needed from the early age of 4 to 5 throughout the completion of the case. Between the ages of 5 and 15, if sagittal growth of the maxilla is delayed because of the cleft dysmorphogenesis, we perform one of two procedures. In cases where there is up to 4 mm retrusion of the maxilla at the occlusal plane, a Delaire face mask traction is used (Great Lakes Orthodontics, Tonawanda, New York). With a retrusion of 12 mm or more we perform distraction.

After orthodontic alignment and leveling of the teeth upon completion of growth, orthognathic surgery is performed in about 35% of the patients to achieve optimal facial balance and aesthetics. A key tenet in removing cleft stigmata is creating a full, convex, projecting facial skeleton.

The senior author has developed and refined these protocols over 36 years of practice treating these difficult facial deformities. Early nasal reconstruction is key for enhancing the patients self-esteem from an early age. This has become the senior author's standard of care in the treatment of patients with unilateral cleft lip-nose and palate. Despite reluctance of some surgeons to perform early nasal surgery, simultaneous lip-nose reconstruction avoids the need for subsequent major nasal surgery with more severe nasal deformity and less pliable cartilaginous framework. This technique can be learned by any dedicated cleft surgeon. It is important to remember that while developing treatment protocols over time, they should be based on ongoing experience, continuous critique, and an adoptive attitude to improvements.

UNILATERAL CLEFT LIP-NOSE REPAIR

The field of cleft surgery has seen major advances over the past 30 years.1,2,3 Normal function and normal to near-normal appearance are a realistic goal and can be achieved.4 To obtain excellent results, a dedicated team approach following a surgical–orthodontic–speech-oriented protocol based on long-term experience is essential.

The most important stage in treating cleft patients is the primary cleft lip-nose repair. It has become the standard of care in the United States and in several other countries to treat the nasal deformity concomitantly with the lip deformity. Many different surgeons have reported consistently good results when performing primary nose repair at the time of lip repair (H. Anderl, personal communications, 1986). 5,6,7,8,9,10 Despite the growing acceptance of the timing of primary nose repair, severe secondary deformities are not uncommonly seen (Fig. 1). The reason is experience. Because of the complexity of both primary and secondary cleft procedures, only the experienced or well-trained surgeon should perform these operations. The correlation of experience and results has been well depicted by the Clinical Standards and Advisory Group study.11 Even more emphasis should be put on meticulous follow-up and self-critical analysis of the results to obtain improved results over time.

Figure 1
(A, B) Frontal and lateral views of secondary deformity due to inadequate dissection and release of lip and nasal components referred to our institution for correction. Note the short lip and the left alar bucking. (C, D) ...

The technique for primary lip and nose repair has been extensively described by the senior author over the years. Based on close analysis of the patients followed over the years, certain modifications and improvements have been incorporated in the authors' original techniques to obtain more consistent results in terms of decreased scarring and improved symmetry, balance, and aesthetics. These modifications are described in this article.

NECESSARY OR NOT NECESSARY?

Nasal Deformity

Correction of the nasal cleft deformity is necessary during the primary surgery as discussed previously. The abnormal anatomy of the cleft nose includes several components. The alar base, the medial and lateral crus of the alar cartilage, the nasal dome, the columella, and the nasal septum—all are affected by the skeletal base, consisting of the alveolus, maxillary segments, and palate. The severity of the primary nasal deformity is intimately related to the degree of displacement, abnormality, and hypoplasia of the maxillary segments. Continued hypoplasia and displacement of the maxillary segments, particularly the lesser segment, result in varying degrees of maxillary deficiency in the unilateral cleft lip and nose deformity. Subsequent growth and the final degree of deformity and outcome depend on the cleft dysmorphogenesis and the selection of surgical procedures and sequencing. Rehabilitation must address the three-dimensionality and growth over time to obtain the desired result.

In the base of the primary deformity of the nose is the displacement of the lower lateral cartilage laterally and inferiorly on the cleft side. The nasal dome is flattened and slumped in a downward position. The alar cartilage on the cleft side is flat and gives it a false appearance of lengthening when compared with the noncleft side, which is abnormally displaced to the other side. The relationship of the lower lateral cartilage to the septum is normal, but the septum itself is tilted because of the cleft deformity, thereby tilting the base of the nose toward the noncleft side and the tip of the nose toward the cleft side. The question that arises in our experience is whether the septum should also be translocated or moved surgically. Anderl believes so; we feel it is not necessary. But the answer is in long-term analysis of results, which we are currently conducting. The key to correction of the cleft nasal deformity is dissecting free and translocating the alar cartilage with its attached vestibular lining into a normal position, thereby establishing the normal vault and shape of the cartilage.5,6,8 At this stage, the major deformity of the nose is corrected. When combined with complete freeing of the soft tissue envelope of the nose and correction of the alar bases and floor of the nose, consistently good results may be achieved at the time of primary correction.

Lip Adhesion

Lip adhesion may contribute to unnecessary additional scarring and abnormal tethering of the lip or nasal elements and therefore is an unnecessary procedure. The current author evaluated early cases of 50 patients in a double-blind randomized fashion comparing those performed with and those without lip adhesions; improved aesthetics in the group that did not undergo lip adhesion brought the author to abandon this technique. Still, many experienced cleft surgeons continue to use the lip adhesion12 with the purpose of treating the abnormal skeletal base, making it easier for the surgeon to close the lip at the expense of the overall aesthetic result for the lip and nose. Others have reported benefits in using nonsurgical lip adhesion with tape.13 Lip adhesion may actually cause fixation or scarring of the alar base or associated adjacent structures in an abnormal position, making it more difficult to obtain a definitive normal contour of the nose.

THE ABNORMAL SKELETAL BASE AND PREOPERATIVE ORTHOPEDICS

Passive Perisurgical Orthopedics

Obtaining symmetry of the skeletal base is one of the main long-term goals for complete correction of the cleft deformity. The attempt to achieve skeletal symmetry at infancy by active perisurgical orthopedics in cases of unilateral deformities is misguided treatment in the senior author's opinion. Early periosteoplasty abnormally locks the segments, producing additional scarring, and does not consistently produce enough bone to support the teeth in the cleft defect to allow excellent orthodontic restoration. Close to half these patients need future bone grafting. In the senior author's opinion, it is key to ignore the skeletal abnormality at the early stage and reconstruct the soft tissue components consisting of skin, cartilage, and muscle.

For these reasons, the current authors' team approach for the past 29 years, for all complete clefts involving the alveolus and maxillary segments, is to use passive perisurgical orthopedics. In the first few days of the child's life an impression is made on which the orthopedic passive appliance is fabricated. This acrylic device initially prevents collapse of the maxillary segments and aids in feeding. The primary objective of the appliance is to control the segments once the lip is closed. It functions as a guide to the maxillary segments and locks them into position after cheiloplasty and before palatoplasty. They also aid or improve the nasal airway by providing a temporary midline closure and nasal septum after closure of the lip, thereby probably also contributing to more normal growth of the nasal airway.

There is no scientific proof that passive orthopedics provide improved results—it is our empirical approach based on team member interaction and influence. Based on clinical observation, the senior orthodontist believes that the appliance improves horizontal and vertical skeletal deficiency by stimulating bone production before and after lip closure; however this hypothesis remains to be proved.14 Cases in which the midline is shifted more than 2 mm require moving the entire maxilla using a Le Fort I maxillary osteotomy. It is easier to perform this in infants treated with a passive perisurgical orthopedic appliance for midline and maxillary deficiencies. The current authors believe that this contributes to better symmetry of the alar bases on the deficient cleft side and improves septal deviation by guiding the maxillary segments into a more normal anatomic relationship. No effort is directed at shifting the deviated septum or changing the skeletal base actively with this technique. The passive appliance allows better control of the maxillary segments before the time of palatoplasty and is worn from infancy to the time of the two-flap palatoplasty at the age of 8 months. After palatoplasty, this method may decrease the amount of maxillary collapse by locking in the maxillary segments.

Active Presurgical Orthopedics

Millard and Latham are credited with popularizing active presurgical orthopedics.15,16 This represents an opposite philosophy to the senior author's in that active orthopedics with active force is used to alter the skeletal base prior to cleft lip nasal repair. Some advocates of this philosophy have added a primary gingivoperiosteoplasty to close the cleft alveolus. One report states that 60% of these patients do not need bone grafting later.17 Essentially, this means that 40% of the patients eventually need a second procedure for bone grafting. In the senior author's hands, primary bone grafting yields a 96% success rate. Therefore, a procedure that yields only 60% success and necessitates a second procedure is both unnecessary and detrimental to midfacial growth and outcome.18 For these reasons, periosteoplasty has been abandoned by some centers.19 Performing early distraction and completing early orthodontic treatment in infants are, in the current authors' opinion, detrimental to growth and development.20 Anterior cross bite and anterior open bite are frequent; therefore, premature surgery due to insufficient amount of bone to support the teeth is detrimental.

As previously mentioned, the authors' approach of ignoring the abnormal skeletal base at the time of primary surgery has resulted in near-normal growth in nearly 70% of the patients. With this approach of passive orthopedics, palatal expansion at the average age of 5.5, and bone grafting of the cleft deficiency at the time of tooth development, these patients do not require orthognathic surgery. Bone grafting at the age of 7 to 9 at the time of cuspid or lateral incisor development using immobilization at the time of grafting produces a 96% success rate in the authors' experience.21,22

Presurgical Nasoalveolar Molding

Nasoalveolar molding has been used and advocated in both unilateral and bilateral cleft cases.23 Through expansion it probably creates more columellar tissue, which is needed in bilateral cleft deformities. In the most common cases of unilateral cleft lip the nasal and lip elements are present; therefore, the senior author does not believe nasoalveolar molding is needed in most unilateral deformities. In the rare Tessier facial cleft, there may be missing elements, in which cases tissue expansion is warranted. Nasoalveolar molding is quite labor intensive, requiring weekly adjustments by an experienced and dedicated orthodontist or surgeon. More so, full compliance is needed from the parents of the infant and there are multiple frequent visits. Most often this technique is used in combination with active presurgical orthopedics, which the senior author thinks is detrimental to growth. The unilateral cases presented here do not warrant this technique. Because of the complexity and labor intensiveness of this technique, it is not practical nor available in most places in the world. The New York group23 has provided this modality and has shown excellent results in bilateral cases, but the use in most unilateral cases is in our opinion unnecessary.

PROTRACTION AND DISTRACTION OSTEOGENESIS

The proportional effect of surgical scarring versus the severity of the primary cleft dysmorphology on abnormal growth seen in patients with cleft deformities is not completely clear. Although there is literature stating that in some third world countries certain untreated cleft cases have shown near-normal facial development,24 we think even those untreated cases do not have normal development of the small segment, especially in the severe cases. There is no conclusive evidence that scarring from surgery is the sole reason for abnormal facial growth, although we consider it a major factor. We believe that the degree of tissue absence and cleft dysmorphogenesis most probably correlates with the degree of facial growth abnormality when careful surgery is performed with minimal scarring. Compensation for this abnormal growth may be performed in certain patients in the age group of 5 to 15 years by either facial protraction with a mask or distraction osteogenesis, depending on the amount of retrusion.

Facial Protraction

In cases with maxillary retrusion of 4 mm or less, the Delaire facial protraction mask (Great Lake Orthodontics, Tonawanda, NY) is used. At the senior author's center, new bone production has been demonstrated with advancement of the anterior nasal spine using protraction in cleft patients. The posterior nasal spine is retracted in our palatal flaps, probably due to the use of posterior vomer flaps.25

Distraction Osteogenesis

After 13 years of evaluating distraction in about 250 patients, it is our opinion that distraction, in its current state of art, should be used in major deformities or retrusion of the maxilla of 12 mm or more. Distraction offers balancing of the facial skeleton during growth, allowing improvement in appearance, speech, and occlusion—all of which improve self-esteem.

Although a majority of these patients require additional definitive skeletal surgery, distraction provides ongoing normalization of jaw relationships during growth and development, offering a major advance in cleft care. It is important to note that definitive orthodontic surgery is more difficult after distraction. Osteotomies should avoid any teeth or tooth buds. We need more data to evolve a more definitive protocol for distraction.

COMMONLY SEEN SURGICAL ERRORS AND PITFALLS

Achieving good consistent results in primary repair of cleft patients depends on in-depth understanding of the cleft lip-nose deformity. Suboptimal outcome or secondary deformities may result from poor operative planning, operative error, or postoperative scar contraction. Most secondary deformities that are encountered by the senior author are from inadequate understanding of the biology of the cleft deformity or of the described technique. This probably results from inadequate technical appreciation of how to release, reshape, and reconstruct the lip and nose adequately while minimizing a detrimental scar.

One of the most common mistakes seen in secondary cases is inadequate release of the abnormally attached lower lateral cartilage to the pyriform rim in unilateral and bilateral cases. Release above the inferior turbinate is crucial for advancement of the abnormally displaced alar cartilage to achieve tip projection or nasal symmetry. An inadequate release of the abnormally tethered alar base is often encountered in these secondary cases. Another common and related mistake is inadequate release and mobilization of the nasal lining, achieved by extending the incision cephalad to the inferior turbinate to allow proper mobilization of the alar cartilage and lining. Many authors incorrectly think, in the senior author's opinion, that this mobilization necessitates the addition of tissue as a mucosa or turbinate flap. The additional scarring formed by this method may actually tether the alar cartilage instead of enabling mobilization of all the nasal elements. Creating cartilage lining flaps is unnecessary. The sentinel concept is sufficient dissection for adequate mobilization of the cartilage.

The basic tenet of tension-free closure applies without exception to the lip closure. If lip closure is performed under tension, more scarring will result.

Similarly, careful technique is important to minimize scarring associated with surgery and a probable contributor to abnormal growth.26 The senior author believes that raising mucoperiosteal flaps in cases of cleft palate repair does not in itself cause significant sagittal growth abnormalities; it does cause alveolar collapse. Any excessive dissection in the space of Ernst may cause severe scarring in the pterygomaxillary region, causing potential growth restriction.

The team approach is important. The use of a multidisciplinary team, particularly integration of the surgical and orthodontic care, is key for achieving the best results. Primary surgery without close and continuous orthodontic follow-up and intervention during growth will consistently produce poor results. Mission surgery without developing a local team is a flawed concept for delivery of the best possible result. Optimal results cannot be expected during and after completion of growth by surgery alone. Multidisciplinary management of the cleft deformity is vital in all patients who have complete cleft of the lip and palate.1

SURGICAL TECHNIQUE

As noted before, the final result is a product of two factors. The first, which we have no control over, is the degree of primary dysmorphology. The second, which is scarring, is at least partially dependent on respect for the tissue and technique. The following procedure descriptions are the latest modifications the senior author has added to his technique in the repair of unilateral cleft lip and nose deformity, in the quest to achieve consistent symmetry and balance of the nose and lip at the time of the primary repair.

A balanced face that is attractive reflecting “no” deformity is the goal. This can now be achieved in most cases in our experience.

Lip

There is a large variety of ways to obtain lip closure with excellent results, each one with advantages and disadvantages. Many techniques today put an emphasis on exact preoperative markings on the skin, which commit the surgeon to some extent to incisions that may not always be optimal after full dissection of the muscle and release of the lip and nose from the abnormal skeletal base. After identification of the peak of the Cupid's bow on the cleft side of the median segment, a near-vertical incision the length of the noncleft philtral column is made attempting to mirror the noncleft philtral column. A transverse incision on the lateral lip segment is performed through and through on the vermilion-cutaneous junction laterally until encountering a normal white roll, thus creating a vermilion flap. There is little resemblance to the initial Millard procedure.2 The method the senior author uses is fluid and allows improvisation and artistry by the surgeon as well as good access to the nose during the primary repair. The final skin design is decided after the muscle and alar symmetry is obtained.

Muscle

Alignment of the muscle is an important basis for lip reconstruction.27 Medial and especially lateral preperiosteal dissection releases the abnormally positioned muscle from the skeletal base, which is key to accomplishing symmetry of the alar bases. When releasing the muscle within the lip, a small sliver of muscle should be left attached to the vermilion to provide an orbicularis marginalis. Dissection of the muscle from the lip should not be extended for more than 4 to 5 mm laterally and should avoid crossing the midline of the philtrum medially to avoid effacing the natural philtral pit. Those who believe that a major dissection subcutaneously or subperiosteally is necessary to achieve a good result are probably mistaken. Unnecessary dissection causes unnecessary scarring, which is detrimental to growth. In the midline, muscle fibers may be slightly bunched together to provide fullness to the midlip.

Combined Correction of the Lip and Nose

The peak of the Cupid's bow on the cleft side is marked on the vermilion cutaneous border (the white roll) at an equal distance from the midline to that of the noncleft side (Fig. 2). To facilitate the symmetric design of the philtrum, a single arm skin hook may be placed in the middle of the prolabium, retracting the prolabium to the midline and then marking. Another important factor is preincision marking of the wet line on the vermilion of each side of the lip, which is critical for a good color match of both sides of the lip and improved aesthetic result as observed by Noordhoff.28 Also, when performing the transverse incision on the lateral lip a sliver of orbicularis should be left on the new vermilion to provide a full vermilion with orbicularis marginalis. As seen in Figure Figure2,2, the incision on the lateral segment follows the dotted line cephalically above the inferior turbinate. This allows full access to the lower lateral cartilage to perform its release from the skin envelope and partially from the nasal lining. If more access is needed, the incision may be extended further within the nose. Despite the extensive freeing of the cartilage, the cartilage itself is not exposed. The incision around the base of the ala on the lateral rotation advancement flap is no longer performed. This eliminates the scarring around the alar base but still allows appropriate release of the alar cartilage. The rotation incision on the medial lip may extend along the base of the columella to provide additional length, but no back cut or extension into the columella is performed, as used earlier by the senior author while developing the technique29 (Fig. 3).

Figure 2
Design of lip repair with the authors' modification of the rotation-advancement procedure. The peak of the Cupid's bow on the noncleft side is marked at an equal distance from the midline of the columella-lip junction. The incision lines on the cleft ...
Figure 3
The incision on the medial lip segment is performed with a number 67 Beaver blade. The incision on the lateral lip segment is carried through and through with a number 65 Beaver blade on the vermilion-cutaneous junction, dissecting a vermilion flap. ...

The medial lip incision toward the columella is performed with a number 67 Beaver blade. The lateral lip element is incised at the vermilion-cutaneous junction with a number 65 Beaver blade with attention to including a piece of orbicularis muscle in the vermilion flap to create the orbicularis marginalis. Now the lateral dermal skin element is undermined and dissected off the muscle for a distance of 2 to 4 mm with careful hemostasis (Fig. 4). At this stage the medial lip element is rotated downward with a hook, comparing the height to the height of the noncleft lip. If the cleft lip height is still short, extension of the incision through the skin, muscle, or underlying mucosa is performed to achieve adequate length (Fig. 5). Undermining of the skin medially in general should not pass the midphiltrum if the philtral dimple is to be preserved. The incision from the alar base is extended intranasally above the inferior turbinate (Fig. 6). The extension of this incision is determined by the degree of nasal deformity. Through this incision the skin envelope may be dissected and the underlying nasal cartilage released, thus allowing repositioning of the alar cartilage into a more symmetric and correct anatomical position. The alar cartilage is at the same time dissected from the lining, leaving the cartilage attached at the dome near the genu (Fig. 7). The abnormally attached foot plate of the medial crus is released using tenotomy scissors. Through this incision, the abnormally attached muscles of the lip and nose are freed, and access to the nasal dome is gained (Fig. 8).

Figure 4
The orbicularis oris muscle is pulled with pick-ups, and the skin of the lateral lip segment is undermined from the underlying muscle at a distance of 2 to 4 mm. The medial lip segment is rotated downward and pulled with a hook until its height matches ...
Figure 5
The medial lip segment is pulled down to match the length of the lip segment on the cleft side. The skin on the medial lip segment is undermined from the orbicularis oris muscle.
Figure 6
The intranasal incision from the base of the ala is extended above the inferior turbinate. The extent of the incision is determined by the degree of nasal deformity. Through this incision, the soft tissue is dissected from the underlying abnormal skeletal ...
Figure 7
The scissors are inserted through the incision at the base of the ala on the cleft side to dissect the skin from the lateral crus of the lower lateral cartilage. The nasal mucosa is dissected from the lateral cartilage, except for the area at the dome. ...
Figure 8
Dissection of the skin over the lateral cartilage on the noncleft side.

The importance of meticulous dissection and freeing of all elements of the lip and nose cannot be overemphasized. This must be completed prior to suturing the muscle if symmetry is the goal. If needed, further dissection should be performed. Dissection over the alar dome is carried to the noncleft side to reposition the abnormally positioned alar cartilage on both the cleft and the noncleft side. Once all elements are free, two straight Keith needles (Richard Allen Scientific, Chicago, IL) on Prolene sutures driven through Dacron pledgets are used to transpose the alar cartilage and shape it. The goal is correct cartilage placement and relationship with the adjacent skin and vestibular lining. It may be necessary to reinsert the suture until optimal position is achieved. This allows recruiting of the alar cartilage to the dome at the genu to achieve projection of the nasal tip (Fig. 9). The stent stitch is pulled upward without tying it down while the second key suture is placed in the orbicularis oris muscle of the lip to achieve lip and nasal symmetry. Sometimes the domal stitch may need to be reinserted after the muscle suture is done due to further shift in tissue. In most cases, symmetry is achieved.

Figure 9
Straight Keith needles with Dacron pledgets are inserted intranasally through the nasal mucosa, lower lateral cartilage, and skin in the dome area. When tied, this suture shifts the alar dome to project the nasal tip.

Often, a buried alar base stitch from the cleft side to the noncleft side helps achieve more nasal base symmetry. Once the muscle closure is completed, an additional lateral alar stent suture is placed to adapt the nasal lining and the overlying skin with the cartilage at the alar base. Alternatively, a buried stitch may be used, but the senior author believes that in his hands better cartilage molding and contour can be obtained with a stent stitch. No tissue is excised in the floor of the nose; any excess skin or lining is sutured and allowed to fall in the nasal floor deficiency.

The transverse lateral element incision is brought into the floor of the nose to create the sill (M.S. Noordhoff, personal communication, 1992).10 The incision length is according to the position of soft tissue elements of the lip and alar base (Fig. 10). To get the best vermilion match, the vermilion is matched both at the cutaneous-vermilion junction and at the wet-dry mucosa line (Fig. 11). Rarely, a small triangular flap above the white role is performed to achieve better positioning of the vermilion. The senior author prefers to delete this procedure if possible. How the skin and lining are used in closure is case dependent and depends on how they lie when the nose is positioned into the proper projection. The skin of the columella C flap is closed according to how it wants to lie, without tension in the nostril or nose when the ala is pulled up with the stent sutures. The subdermis is closed with interrupted 6-0 PDS (Ethicon, Somerset, NJ). Mucosal closure is performed with a 4-0 chromic, and a 6-0 nylon is used for skin. This procedure produces a consistently good result in the hands of an experienced surgeon, who may make modifications or alterations in each step of the operation as needed.

Figure 10
The nasal mucosa and orbicularis oris muscles are sutured. A stent suture with two pledgets—one placed inside the nasal cavity in the dome area and one in the skin—is pulled upward and medially to correct the position of the lower lateral ...
Figure 11
Stent sutures are in place, positioning the lower lateral cartilage on the cleft side symmetric to the one on the opposite side. The floor of the nose, lip, and vermilion are repaired. In most cases, this procedure results in a well-balanced lip and ...

Finally, it is probably better to delay primary nasal surgery if the technique consistently causes scarring, vestibular stenosis, a small nostril, or any other deformity.

RESULTS

Results are depicted in Figures Figures1212 and and1313.

Figure 12
(A, C, E) Frontal preoperative views at age 2 months and postoperative views at ages 5 and 14. (B, D, F) Submental vertex views respectively.
Figure 13
(A) Preoperative frontal view at age 3 months. (B) Postoperative frontal view at age 5. (C) Postoperative frontal view at age 16. (D, E) Postoperative left and right lateral views at age ...

DISCUSSION

At the time of inception of this technique, early nasal surgical intervention was considered detrimental to the growth of the young infant's nose. The results and techniques at that time gave poor results. The prevalent belief was to allow uninterrupted completion of growth prior to surgical intervention. After 36 years of performing this procedure on over 750 patients with long-term follow-up, the senior author believes that most cleft lip-nose deformities can and should be repaired at the primary operation. The alar repositioning, sill reconstruction, and tip projection can be achieved at the time of the primary procedure. Of course, in many cases changes occur with growth that necessitate further secondary revisions. These changes are usually related to the septum and skeletal base. Using the described treatment protocol, the results the senior author achieves at the primary repair of the soft tissue remain consistent with subsequent growth. Failure to achieve satisfactory results should be apparent to the surgeon at the time of completion of the primary repair.

Alignment of the skeletal base is not necessary at the time of primary repair. Passive devices may initially direct the segments toward each other after cheiloplasty and subsequently help prevent palatal collapse after palatoplasty. Active preoperative surgical orthopedics is currently popular and assists the surgeon in closure of the soft tissue by approximating the skeleton, but the authors believe that this causes abnormal locking position of the maxillary segments, causing damage to the growth of the face, resulting in a more severe deformity when growth is completed. Late results have demonstrated this assumption.30 The use of periosteal flaps to close the bony cleft early results in 40% of the patients needing additional bone grafting.

The authors' technique attempts to eliminate the early stigmata of primary cleft lip-nose in young children. It eliminates the alar buckling, a consistent finding of cleft nasal deformities prior to primary repair. When performed by an experienced surgeon, the primary procedure may create a near-normal external nose. When surgery is done, continued multidisciplinary treatment is continued based on a long-standing protocol, and secondary procedures are performed, the result can be a normal, attractive child with minimally noticed deformity. This technique allows mobilization and repositioning of the alar cartilage and creation of a near-normal nasal tip projection and alar base symmetry. If the desired lip and nose shapes are not achieved, minor secondary procedures can be done, either before school age or when final surgical correction is performed. It must be understood that subsequent treatment is needed in all the patients.

Over the years, with gained experience, this procedure has been changed to give consistently improved and more predictable results. When the senior author published his first 15 years of experience with this technique, it showed that buckling of the alar cartilage and flattening of the nose were consistently eliminated on the cleft side as opposed to patients who had only lip repair without treating the nose. Thus far, in the senior author's series to date, there are no severe residual cleft nasal deformities. However, the perfect nose without deformity is unusual. Patients who have excessive scarring of the nose and lip frequently have poor results. Patients with compliant parents generally have good nasal contour throughout their growth after primary surgery and may require only minimal surgery at a later stage. With a critical eye on the results, the senior author reports that about 35% of the patients require early minor secondary procedures, generally performed before the child begins school. Definitive repair after completion of growth is considered more of an aesthetic correction to obtain optimal facial balance and harmony rather than a major deformity correction. Almost all unilateral cases need septoplasty and turbinectomy during the course of care. The authors' goal for these children is to achieve facial harmony and balance, normal speech, full dentition with normal occlusion, and a beautiful smile, resulting in an attractive face with no stigmata of clefting at a conversational distance.

With the described technique, the senior author achieves good to excellent results after primary repair. No external scars or exposure of the cartilage is needed.31,32 Minor positioning and sculpting of the alar cartilages during the teenage years to achieve improved aesthetic balance is all that is necessary. Most of these patients have rhinorrhea and some degree of nasal obstruction. The authors have obtained good results by doing early inferior turbinectomy or limited submucosal resection at the age of 5 to 10 and definitive submucosal resection or inferior turbinectomy after growth is complete. Early elimination of nasal obstruction has improved the authors' results.

The key to achieving consistently good results with this early repair technique is total release and mobilization of all the elements, including the skin envelope, the nasal cartilage, the underlying musculature, the vestibular lining, and the oral mucosa. Once complete mobility is achieved, what remains is repositioning the lip and nasal components in the appropriate anatomical position to obtain normal contour and symmetry with the normal side. This procedure can be consistently performed in these patients and maintained using nasal stent sutures or sutures without stents. Early primary repair of the deformity contributes to more normal growth and development of the nose, and more important, it corrects the deformity early, promoting better psychosocial development and good self-image before adverse psychosocial effects of the deformity cause damage.

An emphasis on the intranasal incision is important. The incision is placed at the level of the inferior turbinate, extending into the nose far enough to achieve complete mobility of all nasal and lip elements without distortion when repositioned. By this approach, complete mobilization of the abnormally based alar base is performed. Bardach and Salyer1 previously emphasized no dissection over the maxilla. The senior author finds it necessary to perform extensive preperiosteal dissection over the maxilla to free the abnormally attached lip and nasal musculature. The authors do not believe this interferes with subsequent growth, which has been demonstrated in their own patients.33

The current authors do not fell it necessary to close the area of the incision along the inferior turbinate with the use of a turbinate mucosa flap or an L or M flap.2 In the senior author's opinion, such flaps may cause distortion of the tissue, interfering with obtaining the desired result. Total mobilization of all the displaced elements and proper repositioning are key for successful reconstruction. Whether the surgeon uses a triangular flap or rotational advancement flaps for the skin incision itself is of secondary importance. The alar base incision in the lateral advancement flap design is now eliminated, thus avoiding the scar in the nostril floor.

The senior author advocates the use of nasal stents postoperatively. The current authors insert these at the time of primary suture removal 1 week postoperatively. This technique splints the nostril with silicone conformers to limit the effects of scarring and wound contracture. The splints are worn for 3 months, and the authors believe they improve scarring and vestibular stenosis in primary cases. The splint used is a Koken stent (Silimed, Porex Surgical, Newnan, GA) in primary and secondary cases. The senior author believes that individually designed silicone nostril stents may further improve this technique. Preoperative nostril expansion using tissue expansion is unnecessary in patients with unilateral cleft lip-nose defects because it is time consuming and labor intensive.

The authors are evaluating speech results, facial balance, and dental occlusion in 50 random completed cases.34 Their findings revealed that velopharyngeal closure could be obtained in 92% of patients treated with two-flap palatoplasty at 8 months of age. Good to excellent facial aesthetic balance of the face, lip, and nose could be achieved in all compliant patients receiving complete treatment. Excellent dental occlusion was achieved with orthodontic treatment and orthognathic surgery when required.

SUMMARY

Good to excellent results have consistently been achieved by the authors in primary unilateral cleft lip-nose repair. Over the years, modification and improvements have led to improved symmetry and balance with less scarring. When used by experienced surgeons, this technique yields consistent, predictable, and reproducible results for all patients with unilateral cleft lip and nose. The goal is normal appearance and function at conversational distance. The achievement of excellent soft tissue and bone restoration while optimizing the patients' facial growth as they grow depends on a surgical-orthodontic-speech-oriented treatment plan. Cleft surgery is not a one-person, one-time reconstructive process. As evidenced in the last patient presented, to obtain excellent results using this primary technique, a multidisciplinary team approach based on a long-term treatment protocol is essential (Figs. 14–17).

Figure 14
(A) Preoperative frontal vertex view at age 1 month. (B) Postoperative frontal view at age 8 months. (C) Postoperative frontal view at age 3 years.
Figure 15
(A, C, E, G) Frontal views of the patient in Figure Figure1414 at the ages of 5, 9, 16, and 17. (B, D, F, H) Occlusion in same ages respectively (note orthodontic work over the years).
Figure 16
(A–E) Stacked lateral views at the ages of 2, 5, 9, 16, and 17. Note orthognathic surgery (Le Fort 1) performed between parts D and E
Figure 17
(A–C) Lateral cephalograms of the same patient (Figs. 16) at ages 9, 16, and 17.

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