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Repairing the cleft lip nasal deformity.

OBJECTIVE: The repair of the cleft lip nose and nasal deformity remains a challenging endeavor for reconstructive surgeons. Psychosocially, this complex, multifaceted deformity significantly stigmatizes the patient. Numerous techniques have been advocated by multiple authors for the treatment and reconstruction of these deformities, usually requiring serial staged reconstructions. METHOD: Described is our technique for early primary repair of the cleft lip nasal deformity. The use of multiple suspension sutures to repair the nasal defect facilitates the repair of even very wide cleft lips. CONCLUSIONS: These maneuvers provide an aesthetic and functional repair of the nasal defect in conjunction with the lip repair. Long-term results have minimized the need for surgical revision.

Cleft Lip↗

Prevalence of palatal and alveolar cysts in babies with cleft lip and palate.

OBJECTIVE: To evaluate the prevalence of palatal and alveolar cysts in babies with cleft lip and/or palate. DESIGN: Cross-sectional. SETTING: Hospital for Rehabilitation of Craniofacial Anomalies, University of São Paulo (HRAC-USP), Bauru, São Paulo, Brazil. PARTICIPANTS: Two hundred ninety-one Caucasian babies divided into four groups according to the type of cleft: cleft lip with or without cleft alveolus (70), complete unilateral cleft lip and palate (112), complete bilateral cleft lip and palate (56), and cleft palate (53). RESULTS: A low prevalence of palatal and alveolar cysts was observed among patients with the four different types of clefts, with no statistically significant difference between genders. The maxilla and the anterior area of the mouth were more affected than the mandible and the posterior area. CONCLUSIONS: The low prevalence of palatal and alveolar cysts in the four groups of babies with clefts included in this study may have been due to the high mean age of the sample.

Alveolar Process↗

TGF-beta-3 promotes scarless repair of cleft lip in mouse fetuses.

TGF-beta3 mediates epithelial-mesenchymal transformation during normal fusion of lip and palate, but how TGF-beta3 functions during cleft lip repair remains unexplored. We hypothesize that TGF-beta3 promotes fetal cleft lip repair and fusion by increasing the availability of mesenchymal cells. In this investigation, we demonstrated that cleft lips in mouse fetuses were repaired by fetal surgery, producing scarless fusion. At the site of the operation, we first observed an infusion of platelets expressing TGF-beta3, followed by increased expression of cyclin D1 and tenascin-C, and coupled with increased mesenchymal cell proliferation. In an ex vivo serumless culture system, cleft lip explants fused in the presence of exogenous TGF-beta3. Cultured lips also showed up-regulation in cyclin D1 and tenascin-C expression. These findings suggest that microsurgical repair of cleft lip in the fetus that produced scarless fusion is mediated by TGF-beta3 regulation of mesenchymal cell proliferation and migration at the site of repair.

Animals↗

A morphometric analysis of craniofacial growth in cleft lip and noncleft mice.

Differences in face shape are considered a factor in cleft lip malformation. The purpose of this study was to analyze craniofacial growth in two strains: A/WySn with 28% cleft lip and C57BL/6J without cleft lip. Standardized photographs of 27 A/WySn and 25 C57BL/6J embryos with 34-46 somites (S) were taken in the superior, frontal, and lateral views. Landmarks were located and digitized for computerized analysis of growth change relative to somite number and at stages of face development before, during, and after primary palate closure. The results showed that both strains had similar overall growth patterns with increases in head width and face width, and decreases in nasal pit width. During early palatal closure in C57BL/6J mice, the nasal pit width was unchanged as brain width increased rapidly; and then later, the nasal pit width decreased as brain width increased slowly. However, during early closure in A/WySn mice, the nasal pit width decreased rapidly as brain width increased slowly; and then later, the nasal pit width was unchanged as brain width increased more rapidly. During early palatal closure, the narrower nasal pit width in A/WySn mice appeared to result from delayed growth of the supporting forebrain as the nasal pits become more medially positioned with normal face development. From the lateral view, the maxillary prominence depth was also smaller in the A/WySn strain during early palatal closure. This deficient forward growth of the maxillary prominences and the narrower positioning of the medial nasal prominences in A/WySn embryos appear to reduce the contact between the prominences and thus predispose this strain to cleft lip malformation.

Animals↗

Long-term effects of palate repair on craniofacial morphology in patients with unilateral cleft lip and palate.

OBJECTIVE: To identify the long-term effects of palate repair on craniofacial growth in patients with unilateral cleft lip and palate (UCLP). DESIGN: Retrospective cross-sectional study. SETTING: Sri Lankan Cleft Lip and Palate Project. SUBJECTS: Forty-eight adults with nonsyndromic unilateral cleft lip and palate, 29 men and 19 women, had lip repair only (LRO group). Fifty-eight adults with nonsyndromic unilateral cleft lip and palate, 35 men and 23 women, had lip and palate repairs by the age of 9 (LPR group). MAIN OUTCOME MEASURES: Clinical notes were used to record surgical treatment histories. Cephalometry was used to determine craniofacial morphology. RESULTS: In the lip and palate repair group, the depth of the bony pharynx (Ba-PMP), the maxillary length at the alveolar level (PMP-A), the effective length of the maxilla (Ar-IZ, Ar-ANS, Ar-A), the maxillary protrusion (S-N-ANS, SNA), the anteroposterior jaw relation (ANS-N-Pog, ANB), and the overjet were smaller than in the lip repair only group. There were no significant differences in the maxillary length at the basal level (PMP-IZ, PMP-ANS) and the anterior and posterior maxillary heights (N-ANS and R-PMP, respectively) in the two groups. CONCLUSION: Palate repair inhibits the forward displacement of the basal maxilla and anteroposterior development of the maxillary dentoalveolus in patients with unilateral cleft lip and palate. Palate repair has no detrimental effects on the downward displacement of the basal maxilla or on palatal remodeling in patients with unilateral cleft lip and palate.

Adolescent↗

A modification of the primary nasal correction in the rotation-advancement unilateral cleft lip repair.

Primary correction of the unilateral cleft lip nasal deformity remains a challenge to the cleft surgeon. We wish to present a method of primary nasal correction utilizing presurgical orthopedics with the Latham dentomaxillary advancement appliance, concomitant rotation-advancement cleft lip repair, gingivoperiosteoplasty, and immediate correction of the nasal cartilage distortion with an "open technique" utilizing the rotation-advancement incisions. Direct elevation and suture fixation of the cleft side alar cartilage is performed as well as recreation of the normal upper lateral/lower lateral cartilage relationship, repair of nasal web, and release of the vestibular lining utilizing an L-flap pedicled to the alar base. Details of the operative technique, observations of unilateral cleft nasal anatomy as visualized with the "open technique," and preliminary results are illustrated in patients followed as long as 5 years.

Cartilage↗

Incidence of cleft lip or palate in 303738 Japanese babies born between 1994 and 1995.

To investigate the incidence of cleft lip or palate or both (CLP) in Japan, 303738 babies born in 1532 institutions between 1994 and 1995 were examined and 437 (0.14%) were found to have abnormalities. Of these babies, 32.1% had cleft lip, 43.3% had cleft lip and palate, and 24.8% had cleft palate (Table 2). These results show that the incidence of cleft lip and palate has declined compared with the period from 1981 to 1982.

Cleft Lip↗

Ectopic eruption of the maxillary first permanent molars in children with cleft lip and/or palate.

The purpose of this study was to analyse the prevalence of ectopic eruption of maxillary first permanent molars in children with cleft lip and palate, and evaluate the influence of both local and genetic factors. Radiographs from 225 children, 125 girls and 100 boys, were studied. The prevalence in cleft children was found to be more than four times higher than in non-cleft children (4.3 per cent). In children with cleft lip or cleft lip and alveolar process the prevalence was 15.4 per cent and in children with cleft palate only it was 28.8 per cent. A notable finding is that the ectopically erupting molar was not related to the cleft side. In children with surgically corrected cleft lip in combination with cleft palate or with cleft palate only, the surgery may have influenced the prevalence to some extent, but the main explanation for the increased prevalence of ectopic eruption in cleft children is a genetic aetiology.

Chi-Square Distribution↗

Incidence of cleft lip and palate and risks of additional malformations.

OBJECTIVE AND METHODS: Children with cleft lip and/or palate (n = 251) born between 1991 and 1995 in the county of Stockholm, Sweden, were studied with reference to incidence and rate ratios (RRs) of different types of clefts, gender, birth weight, mother's age, and length of pregnancy. Children who had clefts and additional malformations were compared with children who had clefts but no additional malformations. RESULTS: The incidence of clefts was 2.0/1000 live births, and it was higher among males than among females. The RR, an index of relative risk, was 1.58. The main groups, children with isolated cleft lip, children with cleft lip and palate, and children with isolated cleft palate, showed similar incidence values (0.6-0.7/1000 live births). Children with bilateral clefts had an incidence of 0.3/1000 live births. Additional malformations were found in approximately every sixth newborn with a cleft when children with Robin sequence were excluded. There was a tendency for newborns with bilateral clefts to have additional malformations (RR = 1.36; confidence interval = 0.74-2.49). Children with clefts and additional malformations had lower birth weight and were born earlier than children with clefts only. CONCLUSION: Preterm cleft children with low birth weight should be screened for the presence of other birth defects.

Adult↗

[A study of individual operation designs for bilateral cleft lip repair].

OBJECTIVE: To design an individual operation procedure that can be adaptable to various deformity of bilateral cleft lip and to assure the symmetry of lip and cupid's bow postoperatively in bilateral cleft lip. METHODS: The distance from both sides of columella to cupid's bow peaks in prolabium as well as both sides of alar base to cupid's bow peaks in lateral lip has been used as standard of classification of the deformity of bilateral cleft lip, then operative procedures respectively was designed in accordance with each special characteristic of bilateral deformity. RESULTS: Five kinds of operation procedures were designed, and each procedure was suitable only for one kind of preoperation deformity of bilateral cleft lip. CONCLUSION: The individual operation designs should be corresponding to the types of deformity before operation in bilateral cleft lip repair.

Cleft Lip↗

Association between alleles of the transforming growth factor-alpha locus and the occurrence of cleft lip.

DNA samples from 100 patients with cleft lip with or without cleft palate (CL/P) were compared with those of 98 unaffected control individuals with respect to transforming growth factor alpha (TGFA) genotypes. Among the Caucasians in this population (83 CL/P, 84 controls), there was a significant difference in the restriction fragment length polymorphisms (RFLPs) observed after digestion with TaqI (chi 2 = 4.68, P = 0.03). The frequency of the C2 allele in the Caucasian CL/P population was 0.169, whereas that in the control group was 0.089. When the data for Caucasians, African-Americans, and Asians were examined jointly, the chi 2 value for the pooled sample was 5.08 (P = 0.02). This confirms the hypothesis of Ardinger et al. [1989, Am J Hum Genet, 45:348-353] that TFGA itself or a closely linked gene contributes to the development of CL/P in humans.

Alleles↗

Social ratings of children with cleft lip by school peers.

A group of 78 students from the second, third, and fourth grades rated color slides of 3 children with bilateral cleft lip, 3 children with unilateral cleft lip and 3 children with normal facial features on 15 independent semantic differential variables. Results indicated that children with cleft lip were viewed more negatively than the nonclefted children. The bilateral cleft lip group was rated more negatively than the unilateral cleft lip group on approximately half of the adjectival scales. The older judges rated the treatment groups higher than the younger judges on all except one of the variables. Implications for these findings are discussed.

Attitude↗

A model for fetal cleft lip repair in lambs.

Fetal wounds heal without inflammation and scar formation. This phenomenon may, in the future, be applicable to human cleft lip and palate repair. However, extensive experimental work must first be done to document the benefits of in utero repair. We developed a large animal model for creation and repair of a complete cleft lip and alveolus using fetal lambs. The cleft lip and alveolus deformity was created in eight 75-day-gestation fetuses (term = 145 days) and either repaired in three layers or left unrepaired. There were four sham-operated fetuses, and all animals were alive at harvest. Repaired, unrepaired, and control fetuses were harvested at 7, 14, 21, and 70 days following surgery. The unrepaired fetuses demonstrated a complete cleft lip and alveolus with an oronasal fistula. The maxilla was asymmetrical, with the greater segment deviated toward the cleft and with decreased anterior maxillary width. In contrast, repaired cleft lip and alveolus animals showed no scar, normal thickness of the lip, and a symmetrical maxilla. Histologic analysis of the repaired wounds showed evidence of tissue regeneration without scar formation. The results of this preliminary study indicate that the fetal lamb cleft lip and alveolus model is technically feasible with an excellent survival rate. Healing occurs without scar formation. In the repaired animals, the maxilla was symmetrical. This model will be used to document facial growth following in utero repair of a cleft lip and alveolus.

Alveolar Process↗

Cleft lip nasal reconstruction using porous high-density polyethylene.

BACKGROUND: The multitude of factors involved with a unilateral cleft lip nasal defect has spurred various surgical techniques in the past. Recently, synthetic materials have been introduced for use in nasal reconstruction. OBJECTIVE: To report on and illustrate the use of porous high-density polyethylene implants in cleft lip nasal reconstruction. DESIGN: A retrospective review of cleft lip nasal reconstruction using porous high-density polyethylene in patients with a unilateral cleft lip defect from January 1, 1993, through June 30, 2000. SETTING: Facial plastic surgery private practice. PATIENTS: Eighteen patients with a unilateral cleft lip without a history of formal rhinoplasty. INTERVENTIONS: All 18 patients required multiple implants, including a columellar strut, premaxillary and prealveolar plumper grafts, a dorsal tip implant, and a unilateral nasal valve batten, using the open rhinoplasty approach. RESULTS: Favorable aesthetic results, as judged by one of us (T.R.), were achieved in all patients. All implants were well tolerated. Postoperative follow-up ranged from 6 months to 7 years. A complication occurred in 1 patient (6%), which resolved with removal of a single implant and intravenous antibiotic therapy. No other complications, including skin erosion or implant extrusion, have been noted. CONCLUSIONS: Porous high-density polyethylene implants for cleft lip nasal reconstruction are well tolerated and achieve good aesthetic results. Porous high-density polyethylene implants lend stability through fibrovascular ingrowth, with integration of the implants to the surrounding tissue.

Cleft Lip↗

[Application of the nasal lining flap to correct the wide unilateral complete cleft lip].

OBJECTIVE: To study use of nasal lining flap in correction of the wide complete unilateral cleft lip. METHODS: 78 patients with wide complete cleft lip were. operated, firstly to be sure. That the lip length on both sides was equal. Then the nasal lining flap was used to enhance the height of the lip on cleft side for keeping symmetry with the non-cleft side. The rest of procedures was the same as "Millard" method. RESULTS: 84.6 percent of patients had good appearance at one and half years after surgery. CONCLUSIONS: It is an effective way to take advantage of the nasal lining flap to correct the wide complete unilateral cleft lip.

Cleft Lip↗

Major genes that cause cleft lip in mice: progress in the construction of a congenic strain and in linkage mapping.

The major gene that causes cleft lip in the A/WySn mouse strain is being transferred to the unrelated normal AEJ/GnRk strain background by the backcross-testcross method. The result, a congenic strain pair differing only at the major cleft lip locus and closely linked loci, will allow the unequivocal identification of the genetic lesion that causes cleft lip. Four backcross generations have been completed, and the background geneotype is calculated to be 97% of AEJ/GnRk origin. The recovery of the cleft lip trait in testcrosses of each generation is compatible with the expected values for the transfer of a single major gene with 8% penetrance. Multilocus inheritance beyond two loci is ruled out. In separate experiments using mostly Robertsonian chromosomes as markers of the normal allele at the cleft lip locus, portions of the genome have been screened for the map location of the cleft lip gene. The gene is probably not within 10-20 cM of the centromere of 12 different identified autosomal chromosomes nor linked to Ph on chromosome 5. Previous studies have suggested that the cleft lip gene is not linked to c (chromosome 7), b (chromosome 4), d (chromosome 9), T, H-2 (chromosome 17), or N (chromosome 15).

Alleles↗