Analysis of the lengthening effect of the muscle repair in functional cleft lip repair

In 14 patients undergoing functional cleft lip repair, changes in the lengths of the key lip segments were measured preoperatively, after the muscle layer was repaired, and after the skin was repaired using pieces of wire bent to follow the curves of the lip in three dimensions. The cleft side of the lip was shorter than the normal side in the vertical and horizontal dimensions. Freeing the muscle from its dermal insertions, splitting it, and advancing it into the medial side of the cleft lengthened the cleft side of the lip vertically and horizontally. The Z-plasty skin repair further lengthened the cleft side of the lip in the vertical dimension. The lengthening effect of the muscle repair appears to be the result of the loose skin redraping over the dissected muscle and further explains elimination of the orbicularis bulge and superior scar formation in the functional cleft lip repair.     

Unilateral cleft lip repair

The marking of the medial lip segment of the Millard rotation advancement procedure for repair of the unilateral cleft lip has been altered in the uppermost portion by utilizing tissue from the columellar base. Once adequate length has been obtained, cutback is utilized at approximately 90 degrees. With adequate full-thickness release of this medial lip segment and subsequent rotation into the proper position, the C flap is advanced into the donor defect of the columellar base and is also used to lengthen the shortened columella on the cleft side. This results in placement of a scar that will closely simulate the "mirror image" of the noninvolved philtral column. Fifty-seven patients with unilateral cleft lip have been repaired utilizing this technique during the past 14 years. Several of these children have required secondary surgeries because of mucosal irregularities or residual nasal deformities, but none has required additional surgery because of inadequate rotation of the medial lip segment or for correction of any donor-site defect at the base of the columella.     

Lip pressure changes following lip repair in infants with unilateral clefts of the lip and palate

The present study was designed to quantitatively assess lip pressure changes following cleft lip repair in infants with unilateral cleft lip, alveolus, and palate. Lip pressure measurements were taken using an electronic transducer system developed especially for this study. Lip pressure was monitored from 3 months (preoperatively) through 2 years of age in cleft and normal control children. Findings from the present study confirm the hypothesis that lip repair in infants with unilateral cleft lip and palate significantly increases lip pressure and that increased lip pressure remains significantly higher than in normal control children for the 2-year duration of the study. Thus increased lip pressure when the palate is unrepaired has to be considered as a factor modulating subsequent craniofacial growth.     

Histologic, histochemical, and ultrastructural analysis of soft tissues from cleft and normal lips.

The cause of cleft lip remains speculative. The nature and extent of pathophysiologic changes in cleft lip muscle are controversial. This study was undertaken to better understand the developmental processes at work. There were two groups of patients. In group 1, 40 fresh tissue specimens were taken from 22 patients who were 2 to 5 months old-their age at the time of their primary cleft lip repair. In group 2, eight control specimens were collected from six children who were seen in the emergency department with lip lacerations. Fresh specimens fixed in neutral buffered formalin were evaluated by the use of hematoxylin and eosin with Luxol fast blue, Bielschowsky, and Masson trichrome stains. Fresh frozen tissue was histochemically assessed by the use of hematoxylin and eosin, modified Gomori trichrome, and adenosine triphosphatase. Ultrastructural analysis was performed on fine sections of glutaraldehyde-fixed tissue. Histologic examination revealed increased endomysial and perimysial collagen in cleft specimens with evidence of muscle-bundle size variation and nonneurogenic atrophy. Insignificant differences were observed between cleft-side and noncleft-side specimens when the means of 200 counts of neural-tissue bundles in the subdermis were compared (p = 0.093). Histochemical examination revealed no typical checkerboard pattern, but a preponderance of type 2 fiber was seen. By means of electron microscopy, increased numbers of subsarcolemmal mitochondria were found in cleft, noncleft, and control specimens. Increased absolute numbers of mitochondria and variations in size, shape, and crystal arrangement were identified. In conclusion, there is no evidence of deficient neural supply in the cleft lip. There is also no evidence of neurogenic muscle atrophy or a metabolic abnormality. There are characteristic myopathic changes. These, in concert with the observed interstitial fibrosis, may have far-reaching implications for growth and function.     

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.     

A long-term, controlled-outcome analysis of in utero versus neonatal cleft lip repair using an ovine model.

Successful open repair of a cleft lip in utero has the advantage of scarless wound healing in the fetus. Unfortunately, no long-term outcome studies have been performed to evaluate the efficacy of these repairs. Moreover, no study to date has compared the long-term results of an in utero cleft lip repair to a similar, control-matched, newborn cleft repair. This study was performed to evaluate the 9-month outcome of in utero cleft lip surgery compared with an identical cleft lip repair performed on infant lambs. In utero epithelialized cleft lips were created through an open hysterotomy in sixteen 65-day-old fetal lambs (term = 140 days) using methods described by Longaker et al. Eight of 16 animals underwent subsequent in utero repair of these clefts at 90 days gestational age. The repair of the remaining eight animals was delayed until 1 week postpartum. At 9 months, the animals were analyzed for changes in lip contour and for the degree of scarring by hematoxylin and eosin and Masson's trichrome collagen staining. Two animals in each group died from preterm labor. Of the animals that survived to term, all repaired lips had some degree of abnormality postoperatively. One of six lips repaired in utero dehisced before delivery. Three of six neonatal repairs dehisced in the first postoperative month. In the remaining animals with intact lip repairs, the vertical lip height on the repaired side was an average of 9 to 12 mm shorter than the normal lip in both the in utero and neonatally repaired animals. Phenotypically, the postnatally repaired animals had more lip distortion and visible notching. Histologically, the in utero repair was scarless and the neonatal repairs had scar throughout the entire vertical height of the lip with an associated loss of hair in this region. Maxillary growth was also evaluated. There was no inhibition of maxillary growth in the animals that underwent in utero cleft lip repair. However, in the neonatal repair group, significant maxillary retrusion was evident. Compared with the cleft side of the maxilla, horizontal growth was decreased by 11 percent (p = 0.01). Compared with the intrauterine repair group, there was a 17-percent decrease in horizontal maxillary width (p = 0.01). Straight-line in utero repair of a cleft lip produces a better long-term result in terms of maxillary growth than a similar repair performed postnatally in the ovine model. There was no diminution in maxillary growth in the animals treated in utero. Histologically, in utero repair of clefts was indeed scarless. However, both lip repairs produced lips that were significantly shorter than their contralateral noncleft sides. This degree of lip shortening would require a secondary lip revision, thereby defeating the purpose of performing an intrauterine repair. Comparisons now need to be made between in utero and neonatal repairs using a Millard-type rotation advancement technique before intrauterine treatment can be considered to be more beneficial than our current treatment modalities.     

Experience with the functional cleft lip repair

The first 12 functional cleft lip repairs performed on unselected consecutive patients immediately following the completion of training by the author are presented. Previous reports on this cleft lip repair have shown excellent results but have always been based on patients operated on by the originator of the procedure. This report gives credence to the ease with which a cleft lip repair that gives reproducible good results can be taught and learned even by plastic surgeons with limited experience. It reviews the technical steps of the procedure, which emphasizes wide undermining and release of the orbicularis oris muscle on the lateral side of the cleft to allow redraping and lengthening of the lip skin, step-by-step layered closure of the mucosa, muscle, and skin, and further vertical lengthening of the lip with a Z-plasty skin closure. Three elements that are difficult to achieve or restore with cleft lip revision are evaluated: (1) achievement of a good skin scar, (2) maintenance of the alar-facial groove, and (3) achievement of adequate lip height without sacrificing horizontal lip length. Ten of the 12 patients had a satisfactory scar, 9 patients had a good alar-facial groove, and all patients had a normal-appearing horizontal lip length. Nine patients required secondary surgery; however, in six patients, this included correction of the nasal deformity that was not corrected at the time of cleft lip repair.     

Pathophysiology of cleft lip muscle

Although attention has been focused for decades on the correction of cleft lip deformities, our knowledge about the etiology of such deformities has remained presumptive. Sixty-six muscle biopsy specimens from cleft lip infants were obtained at the time of primary closure. Histochemical stains, histographic analysis, and electron microscopy were performed. A nonneurogenic muscle atrophy was seen that varied in severity, with muscle fibers near the cleft being the most atrophic and disorganized. Muscle fibers stained with the modified Gomori trichrome technique also demonstrated "ragged red" fibers typical of a mitochondrial myopathy. Electron microscopy confirmed large accumulations of mitochondria distorting the fibrils. These mitochondria also were increased in size and densely packed with cristae. This study thus demonstrates that the muscles in cleft lip deformities are not normal. Instead, they reflect either myopathy in the facial mesenchymal mitochondrion or at least a delay in maturation. We hypothesize that some of the morphologic deformities associated with cleft lip may cause a failure of mesenchymal reinforcement of the facial processes at a critical time in development.     

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.     

Muscle reconstruction in cleft lip repair

This article presents a method of muscle reconstruction in the cleft lip patient that grossly reproduces the interlacing muscle anatomy in the central portion of the upper lip and thereby addresses both the static and the functional requirements of cleft lip repair. This lip repair procedure is combined with paranasal muscle reconstruction, preoperative nasoalveolar molding, and postoperative nasal stenting to constitute a comprehensive approach to cleft lip/nose repair.     

Development of the orbicularis oris muscle in normal and cleft lip and palate human fetuses using three-dimensional computer reconstruction

As part of an ongoing study of cleft lip and palate fetal morphology, normal and dysmorphic development of the human fetal orbicularis oris muscle was studied in a cross-sectional sample of 29 human fetuses (20 "normal" and 9 cleft lip and palate) ranging in age from 8 to 21 postmenstrual weeks. The specimens were embedded in celloidin and sectioned at 20 microns, and every tenth section was stained with hematoxylin and eosin. A computer reconstruction technique was applied to produce three-dimensional representations of the orbicularis oris muscle. The orbicularis oris muscle in the normal fetal sample with discernible lip fibers (N = 15) increased symmetrically in both fiber density and complexity from 12 to 21 weeks. Metrically, muscle volume and thickness growth curves were consistent with qualitative observations. In contrast, the unilateral cleft lip and palate fetal specimens with discernible lip fibers (N = 3) exhibited a 3.5-week delay in overall muscle development, asymmetrical fiber distribution, and abnormal fiber insertions. However, quantitatively, no significant (p greater than 0.05) differences were noted in orbicularis oris muscle thickness or volume between the normal and cleft lip and palate fetal specimens through 21 weeks. Findings suggest that orbicularis muscle deficiency, noted clinically in cleft lip and palate neonates, may be a result of perinatal functional dysmorphogenesis rather than congenital mesenchymal reduction or deficiency.     

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.     

The cleft lip lower-lip deformity

A patient with a repaired upper lip cleft may acquire a deformity in the lower lip. Records of 63 cleft lip patients who exhibited lower-lip abnormalities were analyzed in order to characterize the soft-tissue and skeletal configuration. The typical deformity was a hypertrophied, superiorly displaced, and anteriorly rotated lower lip. Skeletal analysis of the mandible revealed a variable and inconsistent degree of vertical elongation with posterior displacement of the chin. Operative correction of the cleft lip lower-lip deformity required evaluation of both the soft-tissue and skeletal abnormalities. Soft-tissue procedures gave permanent correction, provided there was a normal maxillary-mandibular relationship. Skeletal correction alone did not completely rectify the abnormal lower-lip posture.     

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.     

New technique for correction of the microform cleft lip using vertical interdigitation of the orbicularis oris muscle through the intraoral incision

A microform cleft lip has three major components: (1) a minor defect of the upper vermilion border with loss of the mucocutaneous ridge; (2) a narrow ridge of tissue, resembling an exaggerated philtral column extending to the nostril sill; and (3) a deformity of the nostril. To attain the muscle continuity without an external scar on the upper lip, the author introduced a new method for the correction of a microform cleft lip deformity using vertical interdigitation of the orbicularis oris muscle through the intraoral incision to create the philtrum. Through the intraoral incision, a full-thickness incision is made down to the mucosa and the posterior portion of the muscle. Then, the remaining portion of the muscle is dissected. The medial and lateral muscle flaps are also detached from the oral mucosa and completely exposed and split into two leaves. The upper leaf of the lateral muscle flap is sutured to the dermis on the philtral dimple and base of the upper leaf of the medial muscle flap. Two leaves of each muscle flap are sutured together to create a vertical interdigitation to increase the thickness of the philtral column and to provide continuity of the muscle. A total of 12 patients with microform cleft lip were treated between August of 2001 and October of 2002. Seven of the patients were male and five were female, with an age range of 1 to 43 years. The follow-up period ranged from 6 months to 15 months, with an average follow-up of 9 months. The results of vertical interdigitation of the muscle were examined. All patients were satisfied with their results. The orbicularis oris muscle provided continuity and preserved good function. In all cases, the operation scar was not visible on the depressed philtral groove on the cleft side. Correction of cleft lip nasal deformity was performed in four patients and alar base advancement was performed in two patients. The advantages of the proposed procedure include the creation of an anatomically natural philtrum without an external visible scar through the intraoral incision, preservation of the continuity and function of the muscle, and sufficient augmentation of the philtral column by the vertical interdigitation of the muscle.     

新生儿唇腭裂术前矫治方法与技术的临床探讨

目的:评价新生儿唇腭裂术前进行鼻-牙槽突-腭畸形矫治方法的疗效。方法:对28例单双侧唇腭裂新生儿进行术前鼻-牙槽突-腭畸形矫治治疗,在面部确定基点,利用数码相机拍射照片,通过image-Pro Plus5.1软件测量相关距离及角度,测量治疗前后的鼻小柱倾斜度、鼻小柱长度、鼻孔宽度和鼻孔高度。治疗前后取上颌石膏模型进行牙槽骨裂隙宽度的测量,比较矫治治疗前后腭部裂隙最大处及牙槽突裂隙的变化。结果:鼻小柱倾斜度平均减小27.11°,鼻孔宽度平均减小4.39 mm(单)或5.29 mm(双),鼻孔高度平均增加2.56 mm(单)或3.57 mm(双),牙槽突裂隙平均减少3.18 mm,腭部裂隙最大处平均减少5.77 mm。治疗前后的各项差异均有统计学意义(P0.05),鼻塌陷畸形程度也得到显著改善。结论:术前进行鼻-牙槽突-腭畸形矫治治疗可为唇腭裂患者手术治疗创造有利条件,提高其整复效果。     

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.     

Reconstruction of vermilion in unilateral and bilateral cleft lips

The white skin roll is a useful term to describe the cutaneo-vermilion border of the lip. The muco-vermilion border line parallels the white skin roll and is described as the red line. The lip vermilion should be constructed so that these lines are parallel and widest at the base of the philtral column. It is suggested that the triangular lateral lip vermilion flap be used in unilateral cleft lips. In bilateral cleft lips, a white skin roll vermilion-mucosal muscle flap is used for reconstruction of vermilion.     

Signaling through Tgf-beta type I receptor Alk5 is required for upper lip fusion

Cleft lip with or without cleft palate is one of the most common congenital malformations in newborns. While numerous studies on secondary palatogenesis exist, data regarding normal upper lip formation and cleft lip is limited. We previously showed that conditional inactivation of Tgf-beta type I receptor Alk5 in the ectomesenchyme resulted in total facial clefting. While the role of Tgf-beta signaling in palatal fusion is relatively well understood, its role in upper lip fusion remains unknown. In order to investigate a role for Tgf-beta signaling in upper lip formation, we used the Nes-Cre transgenic mouse line to delete the Alk5 gene in developing facial prominences. We show that Alk5/Nes-Cre mutants display incompletely penetrant unilateral or bilateral cleft lip. Increased cell death seen in the medial nasal process and the maxillary process may explain the hypoplastic maxillary process observed in mutants. The resultant reduced contact is insufficient for normal lip fusion leading to cleft lip. These mice also display retarded development of palatal shelves and die at E15. Our findings support a role for Alk5 in normal upper lip formation not previously reported.