Cleft lip, cleft palate, and velopharyngeal insufficiency

This article provides an introduction to the anatomical and clinical features of the primary deformities associated with unilateral cleft lip-cleft palate, bilateral cleft lip-cleft palate, and cleft palate. The diagnosis and management of secondary velopharyngeal insufficiency are discussed. The accompanying videos demonstrate the features of the cleft lip nasal deformities and reliable surgical techniques for unilateral cleft lip repair, bilateral cleft lip repair, and radical intravelar veloplasty.     

Binderoid complete cleft lip/palate

A small subset of infants with complete cleft lip/palate look different because they have nasolabiomaxillary hypoplasia and orbital hypotelorism. The authors' purpose was to define the clinical and radiographic features of these patients and to comment on operative management, classification, and terminology. The authors reviewed 695 patients with all forms of incomplete and complete cleft lip/palate and identified 15 patients with nasolabiomaxillary hypoplasia and orbital hypotelorism. All 15 patients had complete labial clefting (5 percent of 320 patients with complete cleft lip/palate), equally divided between bilateral and unilateral forms. The female-to-male ratio was 2:1. Of the seven infants with unilateral complete cleft lip/palate, one had an intact secondary palate and all had a hypoplastic septum, small alar cartilages, narrow basilar columella, underdeveloped contralateral philtral ridge, ill-defined Cupid's bow, thin vermilion-mucosa on both sides of the cleft, and a diminutive premaxilla. Of the eight infants with bilateral complete cleft lip, one had an intact secondary palate. The features were the same as in patients with unilateral cleft, but with a more severely hypoplastic nasal tip, conical columella, tiny prolabium, underdeveloped lateral labial elements, and small/mobile premaxilla. Central midfacial hypoplasia and hypotelorism did not change during childhood and adolescence. Intermedial canthal measurements remained 1.5 SD below normal age-matched controls. Skeletal analysis (mean age, 10 years; range, 4 months to 19 years) documented maxillary retrusion (mean sagittal maxillomandibular discrepancy, 13.7 mm; range, 3 to 17 mm), absent anterior nasal spine, and a class III relationship. The mean sella nasion A point (S-N-A) angle of 74 degrees (range, 65 to 79 degrees) and sella nasion B point (S-N-B) angle of 81 degrees (range, 71 to 90 degrees) were significantly different from age-matched norms ( = 0.0007 and = 0.004, respectively). The ipsilateral central and lateral incisors were absent in all children with unilateral cleft, whereas a single-toothed premaxilla was typically found in the bilateral patients. Several modifications were necessary during primary nasolabial repair because of the diminutive bony and soft-tissue elements. All adolescent patients had Le Fort I maxillary advancement and construction of an adult nasal framework with costochondral or cranial graft. Other often-used procedures were bony augmentation of the anterior maxilla; cartilage grafts to the nasal tip and columella; and dermal grafting to the median tubercle, philtral ridge, and basal columella. Infants with complete unilateral or bilateral cleft lip/palate in association with nasolabiomaxillary hypoplasia and orbital hypotelorism do not belong on the holoprosencephalic spectrum because they have normal head circumference, stature, and intelligence, nor should they be referred to as having Binder anomaly. The authors propose the term cleft lip/palate for these children. Early recognition of this entity is important for counseling parents and because alterations in standard operative methods and orthodontic protocols are necessary.     

Principles and techniques of bilateral complete cleft lip repair

Important principles for repair of bilateral complete cleft lip are symmetry, primary orbicularis continuity, proper prolabial size and shape, median tubercle and mucocutaneous ridge formation from lateral lip tissue, and early construction of nasal tip and columella with anatomic placement of the alar cartilages. A two-stage repair employing techniques based on these concepts is described. At the initial procedure, the lateral crura are positioned and a tiny biconcave prolabium is shaped in anticipation of the changes with growth. The second stage (nasal correction) includes apposition of the alar genua, medial crural relocation, and intranasal transposition of banked forked flaps without disjunction of the columella-labial angle. The complete bilateral cleft lip is a four-dimensional problem.     

Primary repair of bilateral cleft lip and nasal deformity.

LEARNING OBJECTIVES: After studying this article, the participant should be able to: 1. List five principles that guide synchronous repair of bilateral complete cleft lip and nasal deformity. 2. Explain how different growth rates for the principal nasolabial features are applied during primary repair. 3. Describe two approaches for positioning the alar cartilages to form the columella. 4. Discuss the influences on referral patterns for a newborn with bilateral cleft lip. --Traditional repair of bilateral cleft lip focused on labial closure but accentuated the nasal deformities, which were addressed later. By the end of the past century, single-staged labial closure had replaced the old multistaged procedures and the technical emphasis had begun to shift from secondary to primary nasal correction. Now, presurgical maxillary orthopedics sets the bony foundation for synchronous nasolabial repair and for closure of the alveolar clefts. The study of normal nasolabial growth and the typical stigmata of the conventional methods provides the necessary foreknowledge to guide surgical sculpture in three dimensions and to anticipate the fourth dimension. The convergence of several forces are changing referral lines for children born with bilateral cleft lip. These include affirmation of centers of excellence, surgeons' self-regulation, prenatal diagnosis, economics of health-care delivery, and increasing parental sophistication. These pressures are not necessarily in conflict. Care by a subspecialized plastic surgeon and experienced team is in the best interests of the child and the third-party payer.     

A comparison of the effects of the Latham-Millard procedure with those of a conservative treatment approach for dental occlusion and facial aesthetics in unilateral and bilateral complete cleft lip and palate: part I. Dental occlusion

The purpose of this study was to compare the effect of the Latham-Millard presurgical orthopedics, gingivoperiosteoplasty, and lip adhesion protocol with conservative treatment (nonpresurgical orthopedics without gingivoperiosteoplasty) for palatal and dental occlusion in complete bilateral and complete unilateral cleft lip and palate. All patients were from the South Florida Cleft Palate Clinic. A retrospective dental occlusal study was conducted using serial dental casts that had been taken of patients from birth to 12 years of age. All surgical procedures, except for the secondary alveolar bone grafts in the conservative, nonpresurgical orthopedics group, were performed by D. Ralph Millard, Jr. Ralph Latham supervised the presurgical orthopedics cases. Samuel Berkowitz collected and analyzed all the serial records from 1960 to 1996. Among the patients with complete unilateral cleft lip and palate, 30 patients were treated with presurgical orthopedics, gingivoperiosteoplasty, and lip adhesion (the Latham-Millard protocol) and 51 patients were treated conservatively (i.e., nonpresurgical orthopedics without gingivoperiosteoplasty). Among the patients with complete bilateral cleft lip and palate, 21 patients were treated with the Latham-Millard protocol and 49 patients were treated conservatively. Conservative treatment was performed between 1960 and 1980. In patients with bilateral cleft lip and palate, a head bonnet with an elastic strip was used to ventroflex the protruding premaxilla. In all patients (unilateral and bilateral cleft), lip adhesion was performed at 3 months followed by definitive lip surgery at 6 to 8 months and palatal cleft closure between 18 and 24 months of age, in most cases. The Latham-Millard procedure was performed from 1980 to 1996; in bilateral cleft patients, it involved the use of a fixed palatal orthopedic appliance to bodily retract the protruding premaxilla and align it within the alveolar segments soon after birth. In all patients (unilateral and bilateral cleft), palatal alignment was also followed by gingivoperiosteoplasty and lip adhesion. Definitive lip surgery was performed between 6 and 8 months of age, and palatal closure was performed between 8 and 24 months of age using the von Langenbeck procedure with a modified vomer flap. All of the study participants had cleft lips and palates of either the unilateral or bilateral type; the unilateral and bilateral groups were further subdivided based on whether they had received the Latham-Millard protocol or the conservative treatment. It was then determined how many in each of these four basic groups had either anterior or buccal crossbites at four different age levels, when they were approximately 3, 6, 9, and 12 years of age. Although several children entered the study at or just before age 6, every patient in the 9-year-old and 12-year-old sample groups had been in the 6-year-old group and all of the 12-year-olds had been included in the immediate preceding age sample. Two-by-two chi-square tests were carried out within each cleft type (unilateral or bilateral) at each of the four age levels separately, to test whether the treatment groups (protocol versus conservative) differed in the frequency of cases with a given kind of crossbite (rather than not having that kind of crossbite). At every age level, a greater percentage of patients treated with the Latham-Millard protocol developed crossbites than did those treated more conservatively. This difference existed for both the anterior and buccal crossbites and for both unilateral and bilateral clefts. Chi-square tests of the treatment differences in crossbite frequency showed that in three quarters of the Latham-Millard protocol versus conservative treatment comparisons (12 out of 16), a significantly greater frequency of crossbite cases occurred after the Latham-Millard protocol treatment as compared with after the conservative procedure. The chi-square values for the differences in outcome between the two kinds of treatment procedures were greater for the anterior crossbites than for the buccal crossbites, suggesting that the Latham-Millard protocol, relative to the conservative method, was more likely to have an adverse effect on the anterior crossbites than on the buccal crossbites. For those patients born with a bilateral cleft, the differences in crossbite frequency between the protocol and the conservative treatment were statistically significant for patients with an anterior crossbite but not for patients with a buccal crossbite. The analysis shows that in complete bilateral and unilateral cleft lip and palate, the frequency of the anterior crossbite and (except for ages 3 and 12) the buccal crossbite is significantly higher with the Latham-Millard presurgical orthopedics, gingivoperiosteoplasty, and lip adhesion protocol compared with the conservative, nonpresurgical orthopedics without gingivoperiosteoplasty treatment. The exception in the bilateral buccal case may be attributed to the small experimental sample size, which brings down the confidence level.     

Rhinoplasty: creating an aesthetic tip. A preliminary report

A new approach for creating an anatomically aesthetic nasal tip is presented. It is based on extensive cadaver dissections which demonstrate that a convex domal segment plus a sharp domal segment-lateral crural drop-off are key determinants of a refined tip. This configuration can be achieved with sutures in a manner similar to creating the anthelical curl in an otoplasty. Two operative variations are presented. One achieves tip refinement with a limited increase in projection, while the other provides maximum projection. Currently, the technique is of value in bilateral cleft lip noses, posttraumatic deformities, certain secondary cases, and very selected primary aesthetic cases where tip refinement and projection are limited.     

Rectangular flap for horizontal upper vermilion tightness in secondary cleft lip deformity

Radical paring of the cleft edge during a primary cleft operation or repeated secondary surgeries can result in tightness of the upper lip. The degree of the resulting side-to-side tension can vary, from mild cases for which improvement is sought through realignment of the misplaced oral sphincter muscle in secondary revision, to severe cases for which the possibility of a lip switch flap must be considered. When the lip tightness accompanies more than three-quarters loss of the Cupid's bow, an Abbé flap is an alternative. However, the lip switch flap is far from ideal, in both artistic and functional perspectives, and should be avoided if at all possible in mild to moderate degrees of lip tightness. This study presents a method of correcting horizontal cleft upper lip tightness, especially of the vermilion. The method involves local transfer of an inferiorly based rectangular flap from the relatively redundant upper two-thirds to the lower one-third of the upper lip and vermilion. Primary indications for the technique include vermilion tightness with half to three-quarters loss of Cupid's bow. The method has the advantage of supplementing the horizontal lip dimension on the cleft side and restoring a natural Cupid's bow, thereby repositioning the shifted philtral column and adding fullness to the lower one-third of the upper lip. Incorporation of the upper lip scar in the rectangular flap removes ugly scars and spares the lower lip from surgical violation. The orbicularis sphincter function, as seen in facial animation, was well regained. Twenty unilateral and three bilateral cases with a maximal follow-up period of 4.5 years are presented.     

Radical correction of secondary nasal deformity in unilateral cleft lip patients presenting late

It is universally acknowledged that correction of a cleft lip nasal deformity continues to be a difficult problem. In developing countries, it is common for patients with cleft lip deformities to present in their early or late teens for correction of severe secondary lip and nasal deformities retained after the initial repairs were carried out in infancy or early childhood. Such patients have never had the benefit of primary nasal correction, orthodontic management, or alveolar bone grafting at an appropriate age. Along with a severe nasal deformity, they present with alveolar arch malalignments and anterior fistulae. In the study presented here, a strategy involving a complete single-stage correction of the nasal and secondary lip deformity was used.In this study, 26 patients (nine male and 17 female) ranging in age from 13 to 24 years presented for the first time between June of 1996 and December of 1999 with unilateral cleft lip nasal deformity. Eight patients had an anterior fistula (diameter, 2 to 4 mm) and 12 patients had a secondary lip deformity. An external rhinoplasty approach was used for all patients. The corrective procedures carried out in a single stage in these patients included lip revision; columellar lengthening; repair of anterior fistula; augmentation along the pyriform margin, nasal floor, and alveolus by bone grafts; submucous resection of the nasal septum; repositioning of lower lateral cartilages; fixation of the alar cartilage complex to the septum and the upper lateral cartilages; augmentation of nasal dorsum by bone graft; and alar base wedge resections. Medial and lateral nasal osteotomies were performed only if absolutely indicated. The median follow-up period was 11 months, although it ranged from 5 to 25 months. Overall results have been extremely pleasing, satisfactory, and stable.In this age group (13 years of age or older), it is not fruitful to use a technique for nasal correction that corrects only one facet of the deformity, because no result of nasal correction can be satisfactory until septal deviations and maxillary deficiencies are addressed along with any alar repositioning. The results of complete remodeling of the nasal pyramid are also stable in these patients because the patients' growth was nearly complete, and all the deformities could be corrected at the same time, leaving no active deforming vector. These results would indicate that aesthetically good results are achievable even if no primary nasal correction or orthodontic management had been previously attempted.     

Premaxillary excision: reasons and effects

Case reports of five bilateral cleft lip and palate patients subjected to premaxillectomy at ages from 5 to 11 years are presented and the reasons for the procedure noted in each instance. Followed into adolescence, an abnormal peaked configuration of the maxillary arches termed "church steeple" defect developed in four of the five patients. Midfacial growth arrest with pseudoprognathism did not occur in any. Details of the surgical technique used are specified. Features of prosthetic and soft-tissue reconstruction are noted. The history and rationale of primary and secondary premaxillectomy are reviewed. Secondary excision of the premaxilla can be a valid treatment option in a few specifically selected cases.     

Reconstruction of the superior labial sulcus in secondary bilateral cleft lip deformities: an inverted U-shaped flap.

A shallow buccal sulcus deformity following bilateral cleft lip repair is not rare. A variety of techniques are described for the secondary reconstruction of a deficient sulcus. Most of these are associated with a variable amount of contraction with subsequent obliteration of the sulcus. In this article, an inverted U-shaped flap is described for the secondary reconstruction of the deficient sublabial sulcus. In these patients, mobility of the upper lip was severely restricted, so orthodontic treatment was not possible. This technique was used in nine patients whose primary cleft lip repairs were performed in different institutions. The amount of re-adhesion or contraction was negligible, because a bare surface was not left behind and skin or mucosal grafts were not used. By advancing the lateral segments of the lip medially, projection of the upper lip was increased. The procedure resulted in adequate upper lip mobility for all patients, and sufficient sulcus was maintained during 1 to 6 years of follow-up. The patients experienced no difficulty with orthodontic appliances after this reconstruction.     

Assessment of bilateral cleft lip nose deformity: a comparison of results as judged by cleft surgeons and laypersons

Reconstruction of bilateral cleft lip nose deformity is difficult and the outcome is inconsistent. This study was conducted to evaluate the gross outcome and the difference in the assessment of nasal appearance as judged by two groups of raters, cleft surgeons and laypersons. Sixty-four patients with bilateral cleft lip were selected for review. The patients' ages ranged from 5 to 30 years. All patients had undergone primary cleft lip repair and secondary nasal reconstruction, and had been followed for at least 6 months. One image for each patient, which included a digitized frontal, lateral, and worm's-eye view, was projected for evaluation by the raters. The raters included five cleft surgeons and five laypersons. A rating scheme was used in which a score of 3 was given for a good, close to normal nasal appearance, 2 for an average result that needed minor revision, and 1 for a poor result that needed major reconstruction. The scores were averaged for each patient in each group and for each group as a whole. The final outcome was judged as good, fair, or poor on the basis of the mean score for each patient. Statistical analysis was performed. The mean score for all patients was 2.08 as assessed by the laypersons and 2.18 as assessed by the cleft surgeon group. There was no statistically significant difference between the two groups. Comparisons on rating scores among different raters revealed a fair agreement on the ratings within each of the two groups. The results were found to be good in 29.7 percent, fair in 64.1 percent, and poor in 6.3 percent of patients when evaluated by the surgeons. When rated by the laypersons, the nasal appearance was found to be good in 26.6 percent, fair in 60.9 percent, and poor in 12.5 percent of patients. This difference in distribution between the two groups was not statistically significant. When comparing the results given by the two groups of assessors, there was agreement on the nasal appearance in 65.6 percent of patients, and a difference in grading in the rest. For the patients who received different grading, the surgeons rated them one grade higher in 63.6 percent and one grade lower in 36.4 percent. There was no difference in grading between any of the evaluators that reflected a two-grade discrepancy in evaluation of results. This study shows that the surgical outcome of bilateral cleft lip nose deformity repair, at the authors' institution, is less than optimal. When assessing bilateral cleft lip nose appearance, the judgment of results by cleft surgeons was similar to that of the laypersons. However, different rating of results existed within each of the two groups, supporting the importance of clearly assessing patient/parent expectations and defining realistic surgical goals.     

Primary correction of the unilateral cleft nasal deformity

An 18-year experience with the management of the unilateral cleft nasal deformity in 1200 patients is presented. A primary cleft nasal correction was performed at the time of lip repair in infancy; a secondary rhinoplasty was done in adolescence after nasal growth was complete. The technical details of the authors' primary cleft nasal correction are described. Exposure was obtained through the incisions of the rotation-advancement design. The cartilaginous framework was widely undermined from the skin envelope. The nasal lining was released from the piriform aperture, and a new maxillary platform was created on the cleft side by rotating a "muscular roll" underneath the cleft nasal ala. The alar web was then managed by using a mattress suture running from the web cartilage to the facial musculature. In 60 percent of cases, these maneuvers were sufficient to produce symmetrical dome projection and nostril symmetry. In the other 40 percent, characterized by more severe hypoplasia of the cleft lower lateral cartilage, an inverted U infracartilaginous incision and an alar dome supporting suture (Tajima) to the contralateral upper cartilage were used. Residual dorsal hooding of the lower lateral cartilage was most effectively managed with this suture. This primary approach to the cleft nasal deformity permits more balanced growth and development of the ala and domal complex. Some of the psychological trauma of the early school years may be avoided. Also, because of the early repositioning of the cleft nasal cartilages, the deformity addressed at the time of the adult rhinoplasty is less severe and more amenable to an optimal final result.     

A long-term retrospective outcome assessment of facial growth,secondary surgical need,and maxillary lateral incisor status in a surgical-orthodontic protocol for complete clefts

In 1965, the cleft palate team at Children's Memorial Hospital embarked on a new surgical-orthodontic protocol in the habilitation of newborn complete cleft lip and palate cases. It brought the orthodontic effort into focus at birth and in planned sequence to correspond with the surgical procedures of lip closure, maxillary alveolar stabilization by means of an autogenous graft of the authors' design, and complete palate closure, all within the first year of life. The purpose of this investigation is threefold: first, to review the authors' previous publications and assess growth, secondary surgical need, and lateral incisor status of teeth adjacent to the cleft in a series of patients who have all followed a precise, early surgical/orthodontic protocol; second, to compare these cases with other collaborative studies wherein this protocol was not used; and third, to report on an additional 82 cases with regard to secondary surgical need and the status of teeth adjacent to the cleft. Methods of assessment have included cephalometric radiography, periapical and occlusal dental radiography, computer-assisted tomography, plaster cast analysis, and intraoral and extraoral photography. The authors have demonstrated, along with other collaborative studies, that there is growth as good as other similar samples wherein there was no primary osteoplasty. In addition, the authors found their incidence of orthognathic surgery to be 18.29 percent; pharyngoplasty, 3.65 percent; and oronasal fistulas requiring surgical closure, 29.27 percent. In the case of unilateral complete clefts, 53.13 percent of those lateral incisors present adjacent to the cleft area were usable, and in bilateral cases, 57.77 percent were usable. The authors remain convinced after more than 35 years of following this successful protocol that early maxillary orthopedics and their technique of primary osteoplasty in planned sequence with lip and palate closure can produce a more favorable alignment of maxillary growth potential and, with comprehensive orthodontic treatment, can lead to teeth in a better overall occlusion than if these procedures had not been undertaken.     

Modified technique of presurgical infant maxillary orthopedics for complete bilateral cleft lip and palate

This article introduces technical modifications to the conventional presurgical infant maxillary orthopedics device for newborns with complete bilateral cleft lip and palate, providing procedural simplicity and efficiency as well as therapeutic efficacy. The modifications incorporate a wax block-out on the stone model prior to device fabrication in a manner that the need for periodic acrylic addition and removal is not required, and thus eliminates the risk of natural maxillary growth restriction during infant maxillary orthopedics treatment. The premaxilla is completely excluded from the acrylic palatal plate and is repositioned primarily by the bilateral labial tape alone. In addition, nasal stent wires are installed on the same day of the palatal plate delivery to establish a tripod-like retention mechanism for the intraoral device to be able to replace the conventional mechanical lock-type retention methods. Applying these modifications, infant maxillary orthopedics treatment objectives for bilateral cleft lip and palate can be successfully achieved within 8 weeks of treatment, and the definitive primary cleft lip repair can be performed within 3-4 months of infant maxillary orthopedics treatment at our Center.     

Preoperative positioning of the protruding premaxilla in the bilateral cleft lip patient

The rationale and technique for preoperative retraction of a protrusive premaxilla in the bilateral complete cleft lip and palate patient are presented. Two types of pinned intraoral appliances are presented that can expand the palatal shelves while retracting the premaxillary segment. Findings from lateral cephalometric x-ray studies of eight appliance patients and six control patients with bilateral clefts but no appliance treatment are presented at age 15. The data indicate that the cephalometric values at age 15 are within the normal range for most patients. Incisor angulation was quite varied among the subjects.     

Correction of secondary cleft lip deformities

Learning Objectives: After studying this article, the practitioner should be able to (1) describe the common secondary deformities of the cleft lip, (2) determine the appropriate timing for surgical intervention to correct the deformities, and (3) determine the best method of addressing each of the individual secondary deformities of the cleft lip. Secondary deformities are common in children born with a cleft lip and palate. Patients with cleft lip deformity will undergo multiple surgical procedures early in life, so it is imperative to prioritize treatment of their secondary deformities and minimize the number of interventions needed. Of the many approaches used to correct these problems, surprisingly few work well consistently. As with all plastic surgery, the timing and procedure should be predicated on the severity of the deformity.     

Primary definitive nasal correction in patients presenting for late unilateral cleft lip repair

Almost 25 percent of unilateral cleft lip and palate patients present with their deformity in their teens or later years in the developing world. Because more than 80 percent of the world population lives in the developing world, the established protocol for repair of these deformities is not applicable to these patients. Despite the magnitude, there are no significant reports in the literature that deal with this problem. Several issues need to be addressed, but the author limits himself here to the correction of the nasal deformity. The patients at this age are very much concerned with the aesthetic outcome. Procedures described hitherto for primary nasal correction in infants are not successful in restoring nasal shape and symmetry at this late age of presentation. Our experience with radical correction of secondary nasal deformity in unilateral cleft lip patients presenting late prompted us to extend the concept by undertaking a definitive primary correction of the nasal deformity in cleft patients presenting late. Twenty-two patients with unilateral cleft lip deformity (nine male patients and 13 female patients) with ages ranging from 13 to 22 years, presenting between August of 1997 and December of 2000, are included in this study. Of these, 11 patients had a cleft of the lip alone, eight also had a cleft of the alveolus, and three had a cleft of the palate continuous with the cleft lip. All patients showed some maxillary hypoplasia. An external rhinoplasty with lip repair was carried out in all patients. The corrective procedures on the nose included columellar lengthening; augmentation along the pyriform margin, nasal floor, and alveolus using bone grafts; submucous resection of the nasal septum; repositioning of lower lateral cartilages; and augmentation of nasal dorsum by bone graft. Clinical follow-up ranged from 4 to 24 months, and the median follow-up period was 13 months. Results have been very good, and much better than results seen earlier with other primary rhinoplasty techniques. While repairing unilateral cleft lip in adolescents, the author thinks it would be most appropriate to address the entire gamut of the deformity in a single stage, provide complete vector reorientation, and augment the hypoplastic elements by autologous tissue. It is not just the fear of poor follow-up, but that merely correcting the lip deformity in these patients without attempting definitive rhinoplasty, in the author's opinion, would be insufficient surgical intervention.     

Improved primary surgical and dental treatment of clefts

The improved combination of surgical and dental teamwork in the primary treatment of clefts presented here is consistent with principles. In fact, this is a staged design for correction of classic clefts of the lip and palate that, based on biological principles, facilitates the continuance of the failed embryonic "migrations" toward a normal end point. Positioning of the alveolar segments, dissection of mucoperiosteum out of the cleft, and union of mucoperiosteum across the alveolar and anterior hard palate cleft make it possible to create a periosteal tunnel across the bony gap and set up a condition conducive to bone formation and eventual tooth eruption in the cleft area. Lip closure by adhesion reduces the tension of the primary lip closure and allows gentle molding until solidification of the arch occurs. Thus a complete cleft has been rendered an incomplete cleft. With a balanced, stabilized maxillary platform, the definitive lip and nose corrections can be carried to completion early (by 2 to 4 years of age). These planned actions bypass a persistent cleft, fistulas, raw areas, malposition of alveolar segments, and probably the necessity for later bone grafting. The only question not totally answered is the effect of this approach on final growth. Although most reports seem to indicate that growth has and will proceed within normal limits, another 10 years of careful follow-up is indicated and, in fact, is in progress.     

Simultaneous reconstruction of the secondary bilateral cleft lip and nasal deformity: Abbé flap revisited

The purpose of this retrospective study was to review the method of using the Abbé flap for correction of secondary bilateral cleft lip deformity in selected patients with tight upper lip, short prolabium, lack of acceptable philtral column and Cupid's bow definition, central vermilion deficiency, irregular lip scars, and associated nasal deformity. A total of 39 patients with the bilateral cleft lip nasal deformity received Abbé flap and simultaneous nasal reconstruction during a period of 6 years. Mean patient age at the time of the operation was 19.1 years, and ranged from 6.6 to 38.5 years. The average follow-up period was 1.8 years. Fourteen patients had prior orthognathic operations. The Abbé flap was designed 13 to 14 mm in length and 8 to 9 mm in width and contained full-thickness tissue from the central lower lip, with a slightly narrow reverse-V caudal end. The prolabium, including the scars and central vermilion, was excised. Lengthening procedures of the upper lip segments were performed if vertical deficiency existed. Part of the prolabial skin was preserved and mobilized for columellar elongation, if indicated. Open rhinoplasty was carried out with or without cartilage graft for columella and nasal tip reconstruction. Reduction of the alar width and nostrils was achieved by a Z-plasty or excision of scar tissue at the nostril floor. The Abbé flap was then transposed cephalad, insetting into the median defect and sutured in layers. The results demonstrated no flap problems or perioperative complications. Seven patients needed further minor revisions on the nose and/or lip. Laser treatment was used to improve the lip scars in three patients. The patients were satisfied with the final outcome and found the lower lip scars acceptable. In conclusion, the described technique of Abbé flap and simultaneous rhinoplasty is an effective reconstructive method for select patients with bilateral cleft lip and nasal deformity.     

Distinct functions for Bmp signaling in lip and palate fusion in mice

Previous work suggested that cleft lip with or without cleft palate (CL/P) is genetically distinct from isolated cleft secondary palate (CP). Mutations in the Bmp target gene Msx1 in families with both forms of orofacial clefting has implicated Bmp signaling in both pathways. To dissect the function of Bmp signaling in orofacial clefting, we conditionally inactivated the type 1 Bmp receptor Bmpr1a in the facial primordia, using the Nestin cre transgenic line. Nestin cre; Bmpr1a mutants had completely penetrant, bilateral CL/P with arrested tooth formation. The cleft secondary palate of Nestin cre; Bmpr1a mutant embryos was associated with diminished cell proliferation in maxillary process mesenchyme and defective anterior posterior patterning. By contrast, we observed elevated apoptosis in the fusing region of the Nestin cre; Bmpr1a mutant medial nasal process. Moreover, conditional inactivation of the Bmp4 gene using the Nestin cre transgenic line resulted in isolated cleft lip. Our data uncover a Bmp4-Bmpr1a genetic pathway that functions in lip fusion, and reveal that Bmp signaling has distinct roles in lip and palate fusion.