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[Cleft palate and cleft lip. Clinical review].

UNLABELLED: The aim of this study is to do an analytical study of cleft palate and cleft lip in our hospital. PATIENTS AND METHODS: 85 clinical charts of patients attended in our hospital born between 1976 and 2001 in Aragon and Rioja were reviewed. We studied the incidence of oral cleft, associated malformations and morbidity, familial antecedents and perinatal data, phonatory disfunctions, serose otitis, growth failure and psychiatry problems. RESULTS: The mean incidence was 0.5/1000 newborns. 41.5% presented associated malformations and 19.3% were associated with a specific syndrome, being more frequent in patients affected of cleft palate and cleft lip (50%) than patients with only cleft palate (41.2%) or only cleft lip (8.8%). The most frequent malformations were: facial defects (50%), skeletal (33%), congenital cardiopathies (33%). 19% were born prematurely. The percentage of serose otitis that required control at hospital was 37.3%. 34.2% presented phonatory problems. There was a high incidence of growth failure and psychiatry problems. CONCLUSION: Oral clefts represent a complex clinical condition with a high percentage of medical complications that require a multidisciplinary treatment. The high incidence of congenital defects associated with this condition demand an exhaustive screening in the newborns affected.

Abnormalities, Multiple↗

[Neonatal cleft lip repair: psychological impact on mothers].

AIM: To investigate the psychological impact on mothers of an early neonatal cleft lip repair. METHODS: Between January and December 2004 mothers of 24 infants born with a cleft lip completed psychological interviews and questionnaires 1 month after neonatal repair. We evaluated the psychological impact on mothers of antenatal and postnatal diagnosis. We noted the parental preferences for the timing of cleft repair and their satisfaction after neonatal repair. RESULTS: Stress, incomprehension and anxiety were often noted. Most mothers preferred their infant to receive neonatal repair. We noted a great satisfaction after neonatal cleft lip repair. DISCUSSION: We are currently encouraging early repair in the full-term baby as the optimum, reliable and safe method of management of newborns with cleft lip. Mothers expressed a preference for and greater satisfaction with neonatal repair. Parental preferences should routinely be considered in deciding the timing of this procedure.

Adolescent↗

Internal midface distraction in correction of severe maxillary hypoplasia secondary to cleft lip and palate.

BACKGROUND: Maxillary hypoplasia is a familiar deformity in patients with cleft lip and palate. A large amount of maxilla advancement is often needed to correct the severe deformity, but local soft-tissue scars around the maxilla restrict maxilla advancement and increase the relapse rate. By gradually lengthening both the bones and the soft tissues, midface distraction can greatly increase postoperative stability and lower the relapse rate. METHODS: Ten patients with severe maxillary hypoplasia secondary to cleft lip and palate were treated with midface distraction using three kinds of internal distraction devices. Among them, six patients received an alveolar bone graft from the iliac crest during their Le Fort I osteotomy, and a bilateral sagittal split ramus osteotomy was performed simultaneously to push back the mandible in five patients with prognathia, to obtain a normal soft-tissue profile and occlusal relationship. RESULTS: Successful maxillary advancements ranging from 5 to 15 mm were measured from preoperative and postoperative cephalograms. Patients' sella-nasion-point A angles increased from an average of 71.25 degrees preoperatively to 79.05 degrees postoperatively. Orthodontic therapies were adopted before and/or after midface distraction. After the consolidation period, dense new bone was found to have formed in the distraction gap. During the follow-up period, the position of the maxilla and the final occlusal relationship were stable and acceptable, and no obvious relapses were seen. CONCLUSION: Midface distraction is an ideal choice for the correction of severe maxillary hypoplasia secondary to cleft lip and/or palate.

Adolescent↗

Presurgical nasoalveolar molding therapy for the treatment of bilateral cleft lip and palate: A preliminary study.

OBJECTIVE: To evaluate the outcome of presurgical nasoalveolar molding therapy in the treatment of patients with bilateral cleft lip and palate. DESIGN: A prospective study with blinded measurements. SETTING: The Cleft and Craniofacial Clinic at the University of Texas at Houston Medical School, Houston, Texas. PATIENTS: Eight patients with bilateral cleft lip and palate, treated between 2002 and 2004. INTERVENTIONS: The starting age for presurgical nasoalveolar molding therapy was 34.9 days and the average length of the therapy was 212.5 days. MAIN OUTCOME MEASURES: Measurements of intraoral and extraoral casts were performed, and statistical analyses were used to compare the differences between measurements before and after therapy. RESULTS: Intraoral measurements demonstrated that there was a statistically significant reduction of the premaxillary protrusion and deviation. There was also a significant reduction in the width of the larger cleft. Extraoral measurements revealed that there was a significant increase in the bi-alar width and in the columellar length and width. Moreover, there was a significant improvement in columellar deviation. Finally, the nostril heights of both sides were increased. CONCLUSION: The authors have quantitatively shown that presurgical nasoalveolar molding therapy has significant advantages in the treatment of bilateral cleft lip and palate patients. It improves the nasal asymmetry and deficient nasal tip projection associated with bilateral cleft lip and palate. It also forces the protruded premaxillary segment into alignment with the dental alveolar segments, improving the shape of the maxillary arch. As a result, the changes associated with presurgical nasoalveolar molding therapy help decrease the complexity of subsequent surgeries.

Acrylic Resins↗

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.

Adolescent↗

Cerebral rhinocele, hydrocephalus, and cleft lip and palate in infants with cardiac fibroma.

Cardiac transplantation was performed in two infants with unresectable fibromas of the myocardium. In one patient, lip surgery was also required for unilateral cleft lip and palate. At autopsy, communicating hydrocephalus of mild to moderate degree was found in both cases. In the patient with facial clefts, there was also a large, ipsilateral cyst, or rhinocele, of the olfactory lobe. This unusual lesion, which seems to represent a previously unreported malformation, was apparently formed by segmental dilatation of a persistent olfactory ventricle. Cerebral or cranial anomalies are thought to be rare in cases of cardiac fibroma; however, macrocephaly was present in five patients. Furthermore, presenting abnormalities among previously reported cases included hydrocephalus in one case, and cleft lip and palate in another. These and other findings suggest that, at least in some cases, cardiac fibroma is a manifestation of a more extensive developmental disorder.

Brain↗

[Correction of naso-labial disfigurement subsequent to bilateral cleft lip repair with combined upper lip flap and Abbe flap].

Anomalies of the nose and lip after repair of bilateral cleft lip and palate is a psychological injury to the patients affecting their social intercourse. The author has designed an operative method using a flap of the fore-lip combined with Abbe flap of the lower lip to repair the anomaly with successful result. In two patients, free Abbe lip flap was used, with one complete success and partial necrosis of the other.

Adolescent↗

The V.L.S. classification for secondary deformities in the unilateral cleft lip.

A classification designed to be used as a simple and reproducible method to evaluate the residual lip deformities following unilateral cleft lip repair is presented. It was developed by analysing the appearance of the upper lips of 81 patients referred to the author with repaired unilateral clefts. The assessment of each lip was divided into 3 separate components: the vermilion (V), the lip itself (L), and the scar (S), and the resulting classification was termed the V.L.S. classification.

Adolescent↗

Fetal cleft lip and palate detection by three-dimensional ultrasonography.

OBJECTIVES: To demonstrate a standardized approach for the evaluation of cleft lip and palate by three-dimensional (3D) ultrasonography. DESIGN: This was a retrospective study of seven fetuses with confirmed facial cleft anomalies. Post-natal findings were compared to a blinded review of 3D volume data from abnormal fetuses with seven other normal fetuses that were matched for gestational age. Upper lip integrity was examined by 3D multiplanar imaging. Sequential axial views were used to evaluate the maxillary tooth-bearing alveolar ridge contour and anterior tooth socket alignment. Alveolar ridge disruption suggested cleft palate. Premaxillary protrusion, either by multiplanar imaging or surface rendering, indicated bilateral cleft lip and palate. RESULTS: Post-natal findings confirmed bilateral cleft lip and palate (four cases), unilateral cleft lip and palate (one case), and unilateral cleft lip (two cases). Multiplanar review identified all three fetuses with unilateral cleft lip, three of four fetuses with bilateral cleft lip, one fetus with unilateral cleft palate, and three of four fetuses with bilateral cleft palate. Surface rendering correctly identified all cleft lips, with the exception of one fetus, who was thought to have a unilateral cleft lip and palate, despite the actual presence of a bilateral lesion. One cleft palate defect was directly visualized by 3D surface rendering. No false-positives occurred. CONCLUSION: Interactive review of standardized 3D multiplanar images allows one to evaluate labial defects, abnormalities of the maxillary tooth-bearing alveolar ridge, and presence of premaxillary protrusion for detecting cleft lip and palate anomalies. Surface rendering may increase diagnostic confidence for normal or abnormal studies. This technology provides an array of visualization tools that may improve the prenatal characterization of facial clefts, particularly of the palate.

Cleft Lip↗

The mathematics of cleft lip and palate treatment evaluation: measuring the desirability of treatment outcomes.

A study was undertaken to investigate the applicability of utility theory to cleft lip and palate treatment decision making. Questionnaires were used to assess value judgements and attitudes toward risk from 119 individuals associated with seventeen cleft lip and palate treatment centers. Significant differences were found to exist between the values expressed by clinicians grouped according to their specialty or the facility to which they were affiliated. Fathers of cleft lip and palate children differed from cleft palate teams in their judgements regarding the relative values of speech and cosmetic treatment outcomes. No such differences were found between team members and mothers, however. It is suggested that these differences affect the desirability of treatment decisions and the outcomes that follow from them. Therefore, cleft lip and palate treatment planning and the evaluation of alternative therapeutic interventions might be based on a mathematical theory of decision making that explicitly incorporates the subjective assessment of a child's habilitative needs and the goals of the habilitative process.

Cleft Lip↗

[Construction of the new dental arch in the prosthetic rehabilitation of cleft lip and palate. Case report].

The authors describe the prosthodontic rehabilitation of an adult patient with unilateral cleft lip and palate. After the surgical and orthodontic treatment of cleft lip and palate patients the prosthodontic treatment was started to correct the palatal defect, the malocclusion and the missing teeth. The authors emphasize on preliminary steps by using diagnostic casts and try-in dentures and they also suggest other directives in the dental management of cleft lip and palate patients.

Adult↗

Hyperoxia and hypoxia in pregnancy: simple experimental manipulation alters the incidence of cleft lip and palate in CL/Fr mice.

This paper describes alterations in the incidence of cleft lip and palate in CL/Fr mice subsequent to experimental manipulation of maternal respiratory oxygen levels during a critical period of pregnancy. Only a few previous studies have shown that the incidence of some "genetically determined" malformations in mammals can be decreased by environmental procedures. In addition to demonstrating a decreased incidence of cleft lip and palate subsequent to maternal hyperoxia on gestational days 10 and 11 in a genetically susceptible strain, the results of the present study show that hypoxia at this time increases the incidence of cleft lip and palate.

Animals↗

Application of the Health Belief Model in a study on parents' intentions to utilize prenatal diagnosis of cleft lip and/or palate.

Parents of children with cleft lip and/or palate (42 women and 35 men) participated in a study on intentions to use prenatal diagnosis of cleft by ultrasound in subsequent pregnancies. Based on the Health Belief Model (HBM) [Rosenstock, 1974], parents' cognitions on 4 factors were measured by questionnaires: "susceptibility" and "severity perceptions," "benefits" and "barriers" evaluations. Most parents perceived the defect as severe. Over-estimation of recurrence risks was predominant even among parents who had received genetic counseling. Results showed that most parents intend to utilize prenatal diagnosis but do not intend to abort an affected fetus. Subjects' reported reasons represented 3 thematic categories: cognitive (the need to know), emotional, and behavioral. Parents' intentions to diagnose and to terminate were related to the factors predicted by the HBM model. Regression analyses indicated that 38% of the variance in intentions to diagnose and 56% of the variance in intentions to terminate could be explained by the studied variables. The best predictor of both intentions was the perceived benefits of the diagnosis. Implications of these findings for genetic counseling are discussed.

Abortion, Therapeutic↗

Thyroxin-induced differential mortality of mouse embryos with cleft lip.

Administration of thyroxin in midgestation has been reported to prevent spontaneous cleft lip in term fetuses of genetically predisposed mice. Attempts to repeat this finding have given ambiguous results. The experiment was therefore repeated using CL/Fr and A/HeJ mice, and various thyroxin dosages. The embryos were examined on days 13 and 14, earlier in gestation than in previous studies. The usual complement of cleft-lip embryos, 21% in CL/Fr and 10% in A/HeJ, was present, but they were often dead. At all dosages of thyroxin that caused embryonic mortality, differential mortality of cleft-lip embryos at term after thyroxin treatment is thus due to their increased mortality, not to prevention of the lip defect.

Animals↗

A jointed fan-type expander: a newly designed expansion appliance for the upper dental arch of patients with cleft lip and/or palate.

Most patients with repaired cleft lip and/or palate have malocclusion and show various forms of occlusal discrepancy between the upper and lower dentition. In unilateral clefts, the teeth in the collapsed small segment are usually in lingual crossbite, although in most cases they are in normal buccolingual occlusal relation in the molar region. Expansion appliances should fulfill the following functions: the application of expansion force to the alveolar bone of the maxillary complex without buccal tipping of the teeth (to obtain the differential segmental movement); force to the lateral segments of the maxilla without their distortion; and expansion of the smaller segment without creating posterior crossbite. Unfortunately, many appliances do not satisfy these functions. A new fan-type expansion appliance has been designed by the authors to solve these problems. This appliance consists of three parts, as follow: an expansion screw; four bands (anchorage); and a joint connecting both buccal segments. The expansion screw is set in a direction tangent to the curve drawn by the intended fan-type movement of the collapsed small segment. The joint converts the expansion force from parallel to an arched direction.

Cleft Lip↗

Facial growth in patients with bilateral cleft lip and palate: a cephalometric study.

In this study, sagittal facial growth of bilateral cleft lip and palate (BCLP) patients between 6 and 20 years of age is analyzed. The data of Nijmegen were derived from 131 lateral cephalograms taken in 21 BCLP patients who were treated in the Cleft Lip and Palate Center of the University Hospital of Nijmegen. Reported data of 90 BCLP patients treated at the Center of Oslo were used as a reference for comparison. Results of this investigation showed mandibular growth to be similar in both centers. In the premaxillary region some differences were found: The Nijmegen patients presented a more protrusive premaxilla than those at Oslo. The upper front teeth and hence, the premaxilla, were more retroclined in the Nijmegen sample. There were also statistically significant differences in the soft tissue profile. The mean z-score was positive for the nasolabial angle and negative for the angle N'-Sn-Pg'. At 18 years of age, these differences are still apparent. In comparison with Broadbent's values of normal individuals, the SNPg-angle was smaller and the mandibular angle greater in Nijmegen and Oslo. The profiles of the BCLP patients are more convex in Nijmegen and more concave in Oslo than in the noncleft group. Finally, the different treatment strategies of the Cleft Lip and Palate Centers of Nijmegen and Oslo are compared and discussed in terms of their long-term results.

Adolescent↗

Skeletal asymmetries of the nasomaxillary complex in noncleft and postsurgical unilateral cleft lip and palate individuals.

Asymmetries in the nasomaxillary skeleton are very common in individuals with unilateral cleft lip and palate. The purpose of this study was to retrospectively evaluate postsurgical asymmetries of the nasomaxillary skeleton in unilateral cleft lip and palate patients. Included in this study were 36 complete cleft lip and palate subjects, along with 36 noncleft (control) subjects. Skeletal asymmetry, deviations of the anterior nasal spine, and the premaxillary area were compared by chronologic age and skeletal maturation in cleft subjects and controls. Comparison between cleft and control cases was assessed. Results indicated that skeletal asymmetry in unilateral clefts and controls peaks during the pubertal growth spurt. The anterior nasal spine and nasal septum always tend to deviate toward the noncleft side of the nasal cavity, although individual variation exists. Cleft subjects were found to be more asymmetric than noncleft subjects to a statistically significant degree.

Adolescent↗

Anthropometric profile evaluation of the midface in patients with cleft lip and palate.

This study was done to determine those profile anthropometric measurements that are abnormal in the midface profile in patients with cleft lip and palate. The sample population consisted of 30 randomly selected skeletally mature white patients with cleft lip and palate who had been treated by the same team who were accredited by the American Cleft Palate-Craniofacial Association. Twenty patients had unilateral and 10 had bilateral complete clefts. None of these patients had previously undergone orthognathic surgery or definitive rhinoplasty surgery. Fifteen facial anthropometric features were measured on each person's face. The result from this study showed that in patients with cleft lip and palate right versus left side differences did not exist and only four statistically significant differences existed between the unilateral and bilateral cases. However, in all patients, four of these esthetic facial features were consistently and significantly abnormal: obtuse nasofrontal angle: obtuse nasomental angle; a posteriorly positioned infraorbitale relative to globe; and an obtuse general facial angle. Several other features were abnormal in a high percentage of persons in this study. These were lack of supratip break, flat to concave paranasal contour, increased subnasale-alargroove:subnasale-pronasale ratio, decreased nasal protrusion:nasal length ratio, decreased nasolabial angle ratio, decreased maxillary length ratio, increased nasal bridge projection:nasal protrusion ratio, and deficient cheek contour. This data indicates that the major deformity in persons with adult cleft lip and palate exist in the nose and secondarily in other components of the midface.

Adolescent↗