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[The effect of lip repair on maxillary growth in patients with complete unilateral cleft lip and(or) palate].

OBJECTIVE: To investigate the different effects of lip repair on maxillary growth among patients with cleft lip and(or) palate, and also the possible mechanisms accounting for that differences. METHODS: The lateral cephalometric analysis was applied to 84 Chinese patients with unilateral cleft lip and(or) palate at the early stage of permanent dentition, which included 32 UCLA, 20 UCLP (CP unrepaired), and 32 UCLP, and 37 normal Chinese of the same age as controls. RESULTS: The patients with unilateral cleft lip and palate who had lip repaired while the palate was left unrepaired exhibited the similar inhibition on maxillary growth to those who had both lip and palate repaired but the patients with unilateral cleft lip and alveolus had much less inhibition on their maxilla. CONCLUSIONS: The cleft and tissue defect of patients with cleft lip and palate might be the main cause that induced the inhibitive effects of lip repair on maxillary growth.

Cleft Lip↗

Aetiological, modifying and lethal factors in cleft lip and palate.

Prenatal factors influencing cleft lip (CL), cleft lip and palate (CLP) and isolated cleft palate (CP) may account for the development of the defect (aetiological factor) for a change in the degree or type of the defect (modifying factor), or for the death of the embryo (lethal factor). Each active factor appears to act in all three directions, all be it at different ratios. This is used for analysis of groups of cleft-effected individuals registered at the Department of Plastic Surgery, Prague, from the catchment area of Bohemia. In terms of cleft defect teratogenesis (aetiology, modification, lethality) a "protective influence" appears probable in female embryos, in blood group A (ABO system), in HLA antigens A9, A11, B35. An "embryotoxic influence" i.e., an increase in all the three influences is seen in male embryos, regional influences, in the B and AB blood groups, in HLA antigens B17, in primiparae, in multiple pregnancies, in older mothers and in cytomegalovirus infections. The Epstein-Barr virus seem to increase, in particular, CLP embryo lethality. The activity of the factors was rated by means of correlations between the cleft frequency in first-degree relatives and the frequency of the factor in six subgroups classified by the cleft defect subtypes, by means of interactions between two and more factors and by a study of the differences between male and female probands. A model of the threshold of CL and CP teratogenesis was proposed, a model in agreement with prenatal reciprocal equilibrium.

Cleft Lip↗

Alar base flap and suspending suture: a strategy to restore symmetry to the nasal alar contour in primary cleft-lip rhinoplasty.

OBJECTIVE: Patients presenting with cleft-lip deformity usually present with a characteristic nasal deformity. We describe the mechanism and contribution of different surgical techniques to restore alar symmetry in primary cleft-lip rhinoplasty. STUDY DESIGN: We evaluate surgical results using a retrospective, randomized, blinded surgical grading system. We describe a surgical technique designed to restore nasal symmetry in patients undergoing primary cleft-lip rhinoplasty. Patients were selected retrospectively. METHODS: A series of patients were identified with nasal asymmetry associated with cleft-lip deformity. All patients underwent cleft-lip repair with concurrent primary cleft-lip rhinoplasty. Patients who underwent alar base flap suspending suture (ABF-SS) were grouped and selected consecutively after a modification in the senior author's surgical technique. A control group was matched for age, sex, and cleft characteristics. Primary rhinoplasty was carried out concurrently for both study groups while undergoing unilateral cleft-lip repair. The control group did not undergo the described ABF-SS technique. All patients were operated on by the same surgeon over a period of 5 years. Surgical outcomes were evaluated by a panel including lay people as well as trained health care workers experienced in the critical evaluation of esthetic results after cleft-lip rhinoplasty. RESULTS: Forty-six records were reviewed of patients undergoing complete unilateral cleft-lip repair. After applying strict inclusion/exclusion criteria, nine patients underwent the described ABF-SS technique. All patients in the preoperative group had a clinically and statistically comparable degree of deformity (P > .05). There was a clinical and statistically significant improvement in nostril size, shape, symmetry, alar base symmetry, and nasal tip/dome symmetry for patients undergoing repair with the described technique compared with the control group. No clinical or statistically significant difference was observed in the scarring scores between groups. CONCLUSIONS: Patients presenting with cleft-lip deformity usually present with a characteristic nasal deformity. Execution of the described surgical techniques restores nasal alar symmetry in patients undergoing concurrent primary cleft-lip rhinoplasty.

Cleft Lip↗

[Inhibitive effects of lip repair on maxillary growth in patients with complete unilateral cleft lip and palate].

OBJECTIVE: The aim of this study was to assess the isolated effects of lip repair on inhibition of maxillary growth in patients with complete unilateral cleft lip and palate. METHODS: The lateral cephalometric analysis were applied to 20 patients with unilateral cleft lip and palate who only had lip repaired in childhood, 32 patients with unilateral cleft lip and palate who had both lip and palate repaired in childhood, as well as 37 normal Chinese of the same age as controls. RESULTS: Both complete unilateral cleft lip and palate groups had almost the same significant degree of maxillary retrusion compared with the normal control group. CONCLUSION: Lip repair is one of important factors that could inhibit maxillary growth in patients with complete unilateral cleft lip and palate.

Adolescent↗

Progress toward discerning the genetics of cleft lip.

PURPOSE OF REVIEW: Orofacial clefts are common birth defects with a known genetic component to their etiology. Most orofacial clefts are nonsyndromic, isolated defects, which can be separated into two different phenotypes: (1) cleft lip with or without cleft palate and (2) cleft palate only. Both are genetically complex traits, which has limited the ability to identify disease loci or genes. The purpose of this review is to summarize recent progress of human genetic studies in identifying causal genes for isolated or nonsyndromic cleft lip with or without cleft palate. RECENT FINDINGS: The results of multiple genome scans and a subsequent meta-analysis have significantly advanced our knowledge by revealing novel loci. Furthermore, candidate gene approaches have identified important roles for IRF6 and MSX1. To date, causal mutations with a known functional effect have not yet been described. SUMMARY: With the implementation of genome-wide association studies and inexpensive sequencing, future studies will identify disease genes and characterize both gene-environment and gene-gene interactions to provide knowledge for risk counseling and the development of preventive therapies.

B-Cell Lymphoma 3 Protein↗

Estimation of polygenic recurrence risk for cleft lip and palate.

Data on cleft lip with or without cleft palate are utilized to evaluate a new method for estimating recurrence risk under the polygenic threshold model. The influence of critical factors on the accuracy of the estimated risks are evaluated. The model is relatively sensitive to the estimate of heritability employed and relatively robust to the population prevalence estimate and to family size. The calculated risks for cleft lip with or without cleft palate are contrasted with the empiric figures from studies on Caucasians and Japanese populations.

Cleft Lip↗

Length of the cleft in relation to the incidence of hypodontia of the second premolar and to inheritance of cleft lip and palate in children with isolated cleft palate.

We studied severity of the isolated cleft palate expressed as the length of the cleft in relation to hypodontia in the second premolar regions and known inheritance of any type of cleft lip and palate. The material consisted of 47 children at 10 years of age born with non-syndromic isolated cleft palate of varying extent, who all had hypodontia of at least one second premolar, and had panoramic radiographs taken at 10 years of age. Information about length of cleft and inheritance of cleft lip and palate was collected from surgical files initiated at birth. The higher the number of missing second premolars, the more extended was the length of the cleft. More children had hypodontia of the second premolar in the mandible than in the maxilla. The family history had little influence on the length of the cleft.

Anodontia↗

Predisposing factors to formation of cleft lip and palate: inherited craniofacial skeletal morphology.

OBJECTIVE: To identify inherited craniofacial morphologic features in individuals at high risk for cleft of the lip, cleft palate, or both. SUBJECTS: Twins without cleft from 33 pairs of monozygotic twins discordant for cleft lip, cleft palate, or both were studied. There were 17 males and 16 females of Caucasian origin, ranging from 3 to 18 years (15 with cleft lip and palate [CLP], 10 with cleft lip [CL], and 8 with cleft palate [CP]), collected from five craniofacial centers (United States and Canada). DESIGN: The twin without cleft (noncleft) from each set was compared with an age- and sex-matched control individual from the Burlington Growth Centre, Toronto, Ontario. Posteroanterior and lateral cephalograms were traced and digitized using a computer custom analysis. Descriptive statistics, Student's t tests, and analysis of variance were used to test 40 variables in a pilot study comparing the noncleft twin groups with the controls. Preliminary analysis permitted pooling of the CLP and CL groups (n = 25). To minimize false-positive significance, only 14 variables (from the maxillofacial area) were tested. RESULTS: Using the raw probabilities, eight variables showed significant differences between the pooled noncleft CLP and CL (CL[P]) twin group and the controls. However, when the level of significance was adjusted, only four (nasal width [p < .01], cranial base length [p < .05], cranial base width/length ratio [p < .001], and maxillary width/length ratio [p < .05]) were significantly different. No significant differences were confirmed between the noncleft CP twin group and the controls. CONCLUSIONS: The noncleft member of a discordant monozygotic pair has a number of facial characteristics that differ from the general population. These may predispose to the formation of a cleft lip or palate and may result from a deficiency or distortion of the mesenchyme that forms the craniofacial structures.

Adolescent↗

The contour lines of the upper lip and a revised method of cleft lip repair.

Many techniques for cleft lip repair have been reported, but these techniques do not consider the contour lines of the upper lip and, in fact, destroy them. The upper lip has complicated contour lines including the vermilion free margin, vermilion border, upper lip horizontal groove (upper lip groove), philtrum (dimple and columns), nostril sills, and nasolabial grooves. Incision lines should be designed so that the postoperative scars do not cross these contour lines. We feel that our incision lines and postoperative scars provide better aesthetic results and diminish hypertrophic scar formation.

Child, Preschool↗

[Hearing disorders in patients with cleft lip and palate].

Hearing threshold in cleft lip/or palate was assessed in 316 ears by pure tone audiometry. Normal hearing threshold was found in almost a half (164/51,9%) of the ears examined. Hearing defects were most frequently observed in cases of complete unilateral cleft palate (48/60%), whereas they were least frequently recorded in cases of cleft lip (15/19,2%). Complete bilateral cleft lip and palate was associated with conduction deafness in 29 (42,9%) ears examined, thus occupying the last but one place according to the frequency of association between deafness and cleft defects. A hearing conduction gap or more than 40 dB was most frequently present in complete cleft lip and palate (7/5,6%), followed by cleft palate (4/5%). Submucous cleft palate was observed to be less frequently accompanied by conduction deafness (9/56,2%) as compared to complete cleft palate, despite a common feature of bone and muscle support discontinuity. Cleft lip was least frequently accompanied by conduction deafness, but it was still present to a relatively high percentage when compared to the cleft-free population.

Adolescent↗

[Trends in the therapy of cleft lip].

The treatment of cleft lips is a complex problem that need an interdisciplinary approach, involving the effort of many specialists. The constant improvement of plastic surgery has today reached highly qualified techniques that permit satisfactory results in most cases. We report our long experience, including more than 500 cases of cleft lips, stressing the right sequence and the basilar principles of their correction.

Cleft Lip↗

The relationship between nasal airway size and nasal-oral breathing in cleft lip and palate.

Clefts of the lip and palate generally result in reduced size of the nasal airway. Procedures such as the placement of a pharyngeal flap tend to further compromise nasal breathing. The purpose of this study was to determine how size of the nasal airway affects the mode of breathing in adults with cleft lip and/or palate. A heterogeneous population of 50 adult subjects with cleft lip and/or palate was studied. Nineteen of the subjects had pharyngeal flaps. Respiratory inductive plethysmography was used in combination with an integrating pneumotachograph to measure percent nasal breathing. Pressure-flow studies were used to estimate nasal airway size. The data revealed that a majority of subjects had an airway size of less than 0.4 cm2, which constitutes impairment. Mean cross-sectional area for all subjects was 0.38 cm2 +/- 0.20 SD. Seventy percent of the subjects studied were oral breathers to some extent. A Spearman rank correlation coefficient of 0.725 (p less than 0.0001) indicated that oral-nasal breathing mode was related to airway size. Airway size in the subgroup with pharyngeal flaps was even smaller (0.31 cm2), while percent nasal breathing was lower. Mouthbreathing was observed in all subjects whose airway size was less than 0.38 cm2.

Adolescent↗

New directions in the management of cleft lip.

Advances in cleft lip and palate have included improved understanding of its etiopathogenesis, genetics, and the surgical evolution of technical refinements in the correction of this deformity.

Cleft Lip↗

Correction of the unilateral cleft lip nose.

The cleft lip nasal deformity is best repaired secondarily in teenagers. Some more severe cases may be repaired during childhood. Optimal repair requires adequate exposure, best obtained with transcolumellar flying-bird incisions. The major anatomic defect, the misplaced lateral crus, needs to be advanced to a normal position. The vestibular lining of the lateral crus should remain attached to add circulation and support, especially when scoring of the cartilage is needed. The lateral defect left after advancement of the lateral crus should be closed with sutures. Accessory procedures, including septoplasty, augmentation or reshifting of the alar base attachment, and occasionally, cartilage grafts, are critical to achieving an aesthetic result. Fifty-three patients operated on using the technique described are reviewed.

Adolescent↗

Asymmetries of the upper lip and nose in noncleft and postsurgical unilateral cleft lip and palate individuals.

The purpose of this study was to retrospectively evaluate post-surgical asymmetries of the upper lip and nose in complete unilateral cleft lip and palate individuals using documentation collected over a period of years. Twenty-three complete unilateral cleft lip and palate subjects (UCLP) and 34 noncleft subjects who served as controls and had undergone orthodontic treatment were included in the study. Nasal asymmetry, deviation of the tip of the nose, and the deviation of the midpoint of the vermilion border of the upper lip were compared to chronologic age and skeletal maturation in cleft subjects and controls. Comparison between cleft and control cases was also assessed. Results indicate that nasal asymmetry peaks at postpubertal growth stages in the control groups, and the difference between UCLP and controls decreases with time and maturation. The deviation of the nasal tip peaks during the pubertal growth spurt for both UCLP and controls, and the difference between UCLP and controls also decreases with time. The deviation of the midpoint of the vermilion border shows relatively small change with time and maturation, and there is no significant difference between UCLP and controls. In general, the facial asymmetries studied were not as great as expected, and the difference between UCLP and controls decreased with time, growth, and maturation.

Adolescent↗