[Epidemiologic studies on cleft lip and/or palate. I. The classification of cleft types in the general population].
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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.
According to the multifactorial threshold (MFT) model, cleft lip and palate (CLP) result from the influence of exogenous and endogenous genetic factors. Based on nearly 1000 cases of cleft lip with or without cleft palate treated in the clinic for maxillofacial surgery of the Martin-Luther University Halle-Wittenberg during the period 1946-1995, we investigated the influence of season on the occurrence of this malformation. Seasons of the year reflect various exogenous factors that may be accessible to prevention. The calculated date of conception served as comparison value between the study group and controls. The clefts of type III (CLP; P = 0.48), isolated CP (P = 0.933) or male patients with clefts of type I showed no significant deviation from the distribution in the control group over the year. We proved a significant maximum in spring, summer and autumn and a minimum in winter for the conception date of female patients with type I clefts (P = 0.033, relative risk = 2.6). Some of the seasonal factors implicated in the aetiology that play a role in the development of clefts could be:
The appearance of the nostril in cleft lip patients is very important in the subjective assessment of naso-labial forms and patient satisfaction. To improve the outcome of plastic surgery, a computer aided diagnostic system was developed. Facial forms were measured with a three-dimensional optical scanner (Ogis Range Finder RFX-IV) XYZ coordinates (256x240) and RGB (red, green, blue) image (512x480) data sets were then obtained with the apparatus. The nostril area was determined by discriminant analysis of the RGB data, and the landmarks of the nostril were extracted under geometric conditions. To assess the reliability of this technique with head inclination, five volunteers were measured in seven postures. Landmark stability was within approximately 1 mm when the Frankfort plane was 45-60 degrees. Subsequently, this system was applied to two cleft lip patients who had undergone a secondary nasal correction. For control data, 37 healthy adults (22 males and 15 females) were measured in the same manner. Nasal asymmetry in the unilateral case and wide and flat nostrils in the bilateral case were greatly improved after surgery. Conversely, the volume of the nasal tip decreased. This system was a great help in the diagnosis of nostril abnormalities.
Children with cleft lip and/or palate are at risk for societal disapproval and/or discrimination and emotional adjustment problems due to facial disfigurement, speech impairment, and learning difficulties. This article reviews the potential psychosocial problems from a developmental perspective and offers suggestions as to the role of nurses at each developmental stage. Nurses may be prone to the same negative attitudes and biases as the general population and may engage in subtle discrimination against children with cleft lip and/or palate.
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.
OBJECTIVE: The aim of this study was to compare two groups of adult male patients with complete bilateral cleft lip and palate (BCLP) on the basis of lateral cephalometric radiographs. PATIENTS: The first group of adult male patients with complete BCLP was comprised of 13 unoperated patients with an average age of 21 years. The second group was comprised of 14 patients with an average age of 21 years 7 months, who had been operated only on the lip prior to 2 years of age. DESIGN: The following measurements were evaluated: angle and length of cranial base; maxillary spatial positioning and length; mandibular spatial positioning; morphology and length; maxillomandibular relationship; vertical facial length; dental positioning; interdental arch relationship; and soft profile. RESULTS: The results suggest that lip repair has a significant influence on certain areas of the craniofacial complex, mainly the premaxilla and the upper incisors. CONCLUSIONS: The most significant findings consequent to lip repair consisted of reduction of the premaxillary anterior projection and lingual tipping of the upper incisors. Retropositioning of the premaxilla, especially in the alveolar part, is a desired effect of lip repair in complete BCLP. Such effect on the projected premaxilla is usually beneficial, except when the exceedingly severe lip pressure, unfavorable growth pattern, or both retropositions the midface profile beyond acceptable sagittal limits.
INTRODUCTION: Anatomical abnormalities and heterogeneous tissue deficiencies of the bilateral cleft lip nasal deformity challenges the cranio-maxillofacial plastic surgeon to create a functional, yet aesthetically pleasing nose. The authors propose a comprehensive rhinoplasty technique to correct the bilateral cleft lip nasal deformity using composite conchal grafts. PATIENTS: Five children with bilateral cleft lip nasal deformities had nasal reconstruction using conchal composite grafts, averaging 5 years in age at time of surgery. Patient follow-up averaged 21 months. METHODS: An open tip rhinoplasty was performed using a 'V' shaped columellar incision. The conchal composite graft was obtained from the lateral aspect of the ear and was used to reconstruct the lateral alar mucosal defects. Conchal cartilage was used as a columellar strut. The columellar skin was closed in a 'V-Y' fashion, giving greater columellar length. RESULTS: Visual inspection confirmed that the cleft lip nasal deformity was improved in all patients. There were no postoperative complications. All patients had complete composite graft take with minimal donor site morbidity and deformity. CONCLUSIONS: This comprehensive rhinoplasty technique improves the abnormalities found in bilateral cleft lip nasal deformity by using the successful aspects of other methods and introducing the composite conchal graft.
Secondary repair of the cleft-lip nose has to date concerned itself only with correction of the lip and external nasal deformity. However, intensive corrective surgery of the deformed nasal septum, which may cause functional and cosmetic handicaps, is also essential in reshaping the drooped nasal tip and alar wing. This paper presents the basic techniques for correcting the deformed nose and nasal floor including the oronasal fistula dn its supportive structures--deviated nasal septums, tilted columellas, and so on. Several patients are presented with cleft-lip nose deformities satisfactorily repaired using these techniques.
Unlike cleft palate, relatively few teratogens have been found to induce cleft lip in mice. The present study was designed to assess the teratologic, topographic (SEM), and histologic effects on lip morphogenesis following the administration of triamcinolone hexacetonide on the eighth day of gestation. The frequency of cleft lip in treated A/J mice was found to be more than three times greater than the spontaneous frequency in untreated controls. Comparable studies with other murine strains suggest no association between the cleft lip response and either a maternal effect or the H-2 complex. Affected A/J embryos showed a severe reduction in the size of the lateral nasal processes; affected embryos also demonstrated localized cell type-specific alterations, particularly in the epithelia and at the interface between epithelium and mesenchyme.
Prenatal diagnoses of cleft lip and palate can occur during both routine screening obstetrical ultrasound and high resolution obstetrical ultrasound done for other reasons. The affected family or obstetrician may request prenatal consultation with the plastic surgeon. To define this population, a survey was done of all families who were referred to our cleft program with a prenatal diagnosis of cleft between 1990 and 1994. Of 80 newborn referrals, 13 had a prenatal diagnosis of cleft. These children had a higher incidence of bilateral cleft than our average population (53.8 percent versus 28.7 percent, p < 0.03, chi square test). No isolated cleft palates were identified. Nine families were available for follow-up. Only one-third of the families felt that they had been given adequate information about clefts from their obstetrician or ultrasonographer. All who had prenatal contact with the cleft team felt it was valuable. A review of prenatal diagnosis of cleft is given including limitations. Specific counseling information is discussed.
OBJECTIVE: To investigate the method for simultaneous correction of nasal deformity and unilateral cleft lip so as to decrease the secondary operation for the deformity. METHODS: The Millard procedure (or Millard plus triangle flap insertion) was used to repair the unilateral cleft lip. Through the incisions, the greater alar and nasalis were repositioned to the normal anatomical positions. The deviated septum and columella were corrected by cutting the abnormal attachment of the orbicular muscle of mouth to the anterior nasal spine. The mattress sutures through the tip of the columella and ala nasi helped to recover the shape of the nostril. RESULTS: 108 patients were treated with this method. They aged from one month to 19 years, included 30 with second degree cleft lip and 78 with third degree cleft lip. The follow-up for as long as 3 years showed satisfactory results. CONCLUSIONS: This technique can eliminate the severe cleft nasal deformity and elevate the displaced alar cartilage at the time of lip repair without interference with nasal growth. It is recommended for the treatment of unilateral cleft lip with severe nasal deformity.
OBJECTIVE: To investigate the pattern of breast-feeding and sugar intake among babies with cleft lip and palate. PARTICIPANTS: Caretakers of 200 babies with cleft lip and palate enrolled at the Hospital for Rehabilitation of Craniofacial Anomalies, University of São Paulo, Bauru, Brazil. RESULTS: A low prevalence of breast-feeding was found. The most reported reason was the sucking inability of the baby. Complete cleft lip and palate was the primary cause affecting sucking. The first contact with sugar occurred mainly through the baby bottle with milk during the first month of life. For nutritional supplement, the children were given sugar and fruit juices in the bottle. CONCLUSIONS: It was observed that dietary habits in babies with cleft lip and palate are more risky. This highlights the role played by early education and constant oral hygiene follow-up for prevention in these patients.
Comparing the survey results of 224 Centers of Cleft Lip and Palate in the United States with the current status in China, and reviewing the experience and lessons systemically, we could get a conclusion that the repair of cleft lip and palate depends on the experience and excellent skills of the surgeons, and its important role in combined and sequential treatment (team approach) of cleft lip and palate should not be ignored. The team approach program requires multidisciplinary cooperation and good compliance of the patients. In order to improve the treatment outcomes of cleft lip and palate, it is necessary for us to study the advanced program and methods and combined the situation of our country.
Twelve cases of secondary deformities of the cleft lip were repaired with the apron mucosal flap of the lower lip. The operation method is described. The advantages and disadvantages are discussed. Good results were obtained in patients with mild or medium secondary deformities.
The complete cleft lip and palate children, ranging from 6-14 years of age were studied to evaluate the effect of variation in the timing of palatal repair on craniofacial morphology and compared to the noncleft children. It was observed that all the groups early (8 to < or = 24 months), medium (> 24 to < or = 36 months) and late repair (> 36 to < or = 78 months) had significantly larger cranial base, retruded maxillomandibular relations, skeletodental and incisal relationships compared to the noncleft children. However, intercomparison among the cleft groups showed insignificant difference amongst them suggesting that the timing of palatal repairs does not effect the anterioposterior (sagittal) relationship.
Cleft lip with or without cleft palate (CL +/- P) is of high prevalence in an isolated, inbred indigenous population of the Venezuelan rain forest: 12 contemporary probands are known in a population of 1200. CL +/- P is familial in this population. One kindred contains four affected siblings and a total of eight probands. The mode of transmission of CL +/- P is under investigation through the collection and biological verification of genealogies and through screening for selected sequence-based polymorphisms. Polymerase chain reaction (PCR) assays were successfully applied to recover DNA from blood dried onto filter paper in the field. Eleven samples from probands and their first-degree relatives have been analyzed for DNA sequence-based polymorphisms for TGF-A and HOX7. No significant linkages for either locus were found. Evaluation of the distribution of probands in four genealogies indicates that CL +/- P could be transmitted as an autosomal recessive trait in this population. We believe this is the first report of this mode of transmission for nonsyndromic CL +/- P.
A lip-adhesion procedure before definitive repair of unilateral complete cleft lip has been widely used but rarely analyzed over the past 25 years. This report is a quantitative prospective assessment of one possible benefit of lip adhesion, an increased vertical height of the cleft labial elements. Lip adhesion was performed on 43 consecutive infants with unilateral complete cleft of the primary palate. Markings for rotation-advancement repair were placed before lip adhesion and again at the time of the complete nasolabial correction. Using a standardized caliper, anthropometric measurements of the vertical height of the medial and lateral lip elements were made from these markings. The relative increase in vertical height of the medial and lateral labial segments during the mean interoperative interval of 3.2 months was calculated. Analysis of these measurements revealed a small disproportionate increase in vertical lip height of the cleft side compared with the normal side after lip adhesion. Discrepancy of the lateral lip height between the cleft and noncleft sides decreased from 3.0 to 2.7 mm (10 percent, p = 0.05), and the prolabial or medial height discrepancy decreased from 3.4 to 2.8 mm (17 percent, p < 0.01). Subgroup analysis of infants whose adhesion was done either at a young age, without premaxillary orthopedics, or with an intact secondary palate, revealed no statistically significant improvement in the labial height measures. It is arguable whether the small relative increase in height of the medial and lateral cleft elements justifies lip adhesion before definitive repair of unilateral complete cleft lip.