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At least 19 recordsLinked to original sources

Long-term results of segmental repositioning of the maxilla in cleft palate patients without previously grafted alveolo-palatal clefts.

Eleven patients (9 UCLP, 2 BCLP) were treated with segmental osteotomies with or without osteotomies at the Le Fort I level and simultaneous bone grafting of the alveolo-palatal clefts at adult age. These patients were clinically and radiographically evaluated after a mean follow-up period of 59 months (range 39-110 months). One patient showed complete dentoalveolar relapse, whereas the skeletal stability after miniplate fixation proved to be adequate in all cases. Only one patient presented with a persisting oro-nasal fistula. In six cases, the alar base asymmetry had improved to such an extent that further nasal corrections were not necessary. The procedure described is a reliable technique to graft the alveolo-palatal cleft and reposition the dentoalveolar segments simultaneously in those adult cleft palate patients who had no previous alveolar bone grafting.

Adolescent↗

An in vitro mouse model of cleft palate: defining a critical intershelf distance necessary for palatal clefting.

It is unclear whether cleft palate formation is attributable to intrinsic biomolecular defects in the embryonic elevating palatal shelves or to an inability of the shelves to overcome a mechanical obstruction (such as the tongue in Pierre Robin sequence) to normal fusion. Regardless of the specific mechanism, presumably embryonic palatal shelves are ultimately unable to bridge a critical distance and remain unapproximated, resulting in a clefting defect at birth. We propose to use a palate organ culture system to determine the critical distance beyond which embryonic palatal shelves fail to fuse (i.e., the minimal critical intershelf distance). In doing so, we hope to establish an in vitro cleft palate model that could then be used to investigate the contributions of various signaling pathways to cleft formation and to study novel in utero treatment strategies. Palatal shelves from CD-1 mouse embryos were microdissected on day 13.5 of gestation (E13.5; term = 19.5 days), before fusion. Using a standardized microscope ocular grid, paired palatal shelves were placed on a filter insert at precisely graded distances ranging from 0 (in contact) to 1.9 mm (0, 0.095, 0.19, 0.26, 0.38, 0.48, 0.57, 0.76, 0.95, and 1.9 mm). A total of 68 paired palatal shelves were placed in serum-free organ culture for 96 hours (n = 68). Sample sizes of 10 were used for each intershelf distance up to and including 0.48 mm (n = 60). For intershelf distances of 0.57 mm and greater, two-paired palatal shelves were cultured (n = 8). All specimens were assessed grossly and histologically for palatal fusion. Palatal fusion occurred in our model only when intershelf distances were 0.38 mm or less. At 0.38 mm, eight of 10 palates appeared grossly adherent, whereas six of 10 demonstrated clear fusion histologically with resolution of the medial epithelial seam and continuity of the palatal mesenchyme. None of the 18 palates fused when placed at intershelf distances of 0.48 mm or greater. Using our selected intershelf distances as a guideline, we have established an approximate minimal critical intershelf distance (0.48 mm) at which we can reliably expect no palatal fusion. Culturing palatal shelves at intershelf distances of 0.48 mm or greater results in nonfusion or clefting in vitro. This model will allow us to study biomolecular characteristics of unfused or cleft palatal shelves in comparison with fused shelves. Furthermore, we plan to study the efficacy of grafting with exogenous embryonic mesenchyme or candidate factors to overcome clefting in vitro as a first step toward future in utero treatment strategies.

Animals↗

Relationship between genetic anomalies of different levels and deviations in dermatoglyphic traits. Part 6: Dermatoglyphic peculiarities of males and females with cleft lip (with or without cleft palate) and cleft palate--family study.

The present study was carried out to evaluate the effect of polygenic morbidity with respect to Cleft Palate and Cleft Lip with or without Cleft Palate (CL) in males and females based on dermatoglyphic traits (DT) and indices of intraindividual diversity (Div), fluctuating (FA) and directional (DA) asymmetry. The main objectives of the present study were as follows: a) to find DT and FA indices, which could be "marker" traits and could indicate the degree of developmental instability of the organism; b) to explore the possibility of using DT, FA, Div and DA indices of CL patients and their parents and to predict the likelihood of the disease appearing in the offsprings of apparently healthy individuals. The samples were of 106 CL patients (59 males and 47 females) and 156 of their parents (67 fathers and 89 mothers), all Israeli Jews. The prints were collected in the Beilinson (Petah-Tikva) and Rambam (Haifa) and Hadassah (Mount Scopus, Jerusalem) Hospitals, or in the abodes of the CL patients. The results were compared with the control group of healthy women and men whose data are detailed in our previous publication. Interpretation of the prints were done according to the methods and included identification of patterns, ridge counts and the measurements of distances and angles in the palms, 79 DT for every individual, 28 continuous traits, 9 discrete traits, 11 indices of Div, 15 DA indices and 16 FA indices. In CL groups increased FA indices values were found and a decreased sexual dimorphism in DT of the CL and parental groups as compared to the control--this both in terms of the number of significant differences, as well as in values of the traits (e.g. smaller differences between the male and female values). The above mentioned findings were partly confirmed also by the discriminant analysis. The values of DT parents were generally similar to those of the control. The best discrimination was obtained between the CL and control groups (70.44% between CL males and control males and 83.47% between CL females and control females). Over 50% of the DT variables were found to be suitable for including into the discriminant function.

Adolescent↗

Evaluation of prenatal diagnosis of cleft lip with or without cleft palate and cleft palate by ultrasound: experience from 20 European registries. EUROSCAN study group.

Ultrasound scans in the mid-trimester of pregnancy are now a routine part of antenatal care in most European countries. Using data from registries of congenital anomalies a study was undertaken in Europe. The objective of the study was to evaluate prenatal detection of cleft lip with or without cleft palate (CL(P)) and cleft palate (CP). All CL(P) and CPs suspected prenatally and identified at birth in the period 1996-98 were registered from 20 Congenital Malformation Registers from the following European countries: Austria, Croatia, Denmark, France, Germany, Italy, Lithuania, Spain, Switzerland, The Netherlands, UK, Ukraine. These registries followed the same methodology. A total of 709,027 births were covered; 7758 cases with congenital malformations were registered. Included in the study were 751 cases reported with facial clefts: 553 CL(P) and 198 CP. The prenatal diagnosis by transabdominal ultrasound of CL(P) was made in 65/366 cases with an isolated malformation, in 32/62 cases with chromosomal anomaly, in 30/89 cases with multiple malformations and in 21/36 syndromic cases. The prenatal diagnosis of CP was made in 13/198 cases. One hundred pregnancies were terminated (13%); in 97 of these the cleft was associated with other malformations.

Cleft Lip↗

Issues and controversies in the management of cleft palate.

Cleft palate management is complex. There is no current agreement on the appropriate treatment strategy. Extensive disagreement on the pathophysiology, timing of intervention, and techniques of surgical repair have added to the confusion. To provide a comprehensive guide to the management of cleft palate is difficult. However, several main points should be emphasized. Normal speech should be the most important consideration in the therapeutic plan. Growth disturbance should be minimized, but not at the expense of speech impairment, because the facial distortion can be satisfactorily managed with further surgery, whereas speech impairment can often be irreversible. We believe repair of cleft palate to establish a competent velopharyngeal sphincter should be completed from 6 to 12 months of age. This is done early enough to minimize the development of an often irreversible pathologic compensatory speech pattern, but late enough not to increase significantly the surgical risk to the infant. Surgical interventions should be designed to cause minimal disruption of the palate, to decrease the severity of subsequent growth problems. There is a need for well-controlled, prospective studies to establish the validity of the widely different claims of superior results from various techniques. We believe strongly that cleft patients should be managed in a center with a multidisciplinary team. The benefits of these teams have been elaborated. Cleft palate embodies one of the major tenets of plastic surgery, the achievement of an aesthetic result with minimal interference with function. Cleft palate remains a significant and interesting challenge for current and future plastic surgeons.

Cleft Palate↗

X-linked cleft palate.

Cleft lip with or without cleft palate is twice as frequent among Indians of British Columbia as among non-Indians, although the reverse is the case whenever we consider isolated cleft palate. A family is described with 12 affected males. Close examination of this family revealed that the most likely explanation for the cleft palate was an X-linked recessive gene. It is concluded that isolated cleft palate among the Indians of British Columbia is an extremely rare anomaly.

Chromosome Aberrations↗

[Risk of recurrence of several congenital malformations: anencephaly, spina bifida, cleft palate and cleft lip].

Recurrence risks were estimated for some congenital malformation from a multifactorial model according to the method given by Smith (1971). Risks were estimated for cleft lip with or without cleft palate, cleft palate alone, anencephaly and spina bifida from data on frequency and heritability collected in France. Results are given in tables representing risks for some 180 specific family histories.

Anencephaly↗

Dose-response relations of palatal slit, cleft palate, and fetal mortality in mice treated with a glucocorticoid.

C57BL/6 (C57BL) and SWV mice were treated subcutaneously with triamcinolone acetonide in a single dose of 1.0-7.0 mg/kg on day 12 of pregnancy, and the palate of their fetuses was examined at term. In C57BL mice palatal slit occurred spontaneously and its frequency increased with increasing doses of triamcinolone. However, this defect was not seen in SWV fetuses, even when dams were treated with the doses that induced cleft palate. The frequency of cleft palate increased in both C57BL and SWV as the dose of triamcinolone increased. Fetal mortality increased in SWV, but not in C57BL, with increasing doses of triamcinolone. Dose-response relations were analyzed by the log-probit transformation method. In C57BL mice, the slope of the dose-response curve of palatal slit was significantly different from that of cleft palate. In contrast, the dose-response curves of cleft palate were similar in both C57BL and SWV; the median effective dose was significantly greater in C57BL than in SWV. The mechanism of induced palatal slit appears to be different from that of induced cleft palate; the mechanism of cleft palate induction may be the same in both C57BL and SWV. The slope of the dose-response curve of fetal mortality in SWV mice was different from that of cleft palate; the mechanisms underlying the resorption and cleft palate responses must be different.

Animals↗

Homocysteine oxidation and apoptosis: a potential cause of cleft palate.

Cleft palate is the most common craniofacial anomaly. Affected individuals require extensive medical and psychosocial support. Although cleft palate has a complex and poorly understood etiology, low maternal folate is known to be a risk factor for craniofacial anomalies. Folate deficiency results in elevated homocysteine levels, which may disturb palatogenesis by several mechanisms, including oxidative stress and perturbation of matrix metabolism. We examined the effect of homocysteine-induced oxidative stress on human embryonic palatal mesenchyme (HEPM) cells and demonstrated that biologically relevant levels of homocysteine (20-100 microM) with copper (10 microM) resulted in dose-dependent apoptosis, which was prevented by addition of catalase but not superoxide dismutase. Incubation of murine palates in organ culture with homocysteine (100 micro) and CuSO(4) (10 microM) resulted in a decrease in palate fusion, which was not significant. Gelatin gel zymograms of HEPM cell-conditioned media and extracts of cultured murine palates, however, showed no change in the expression or activation of pro-matrix metalloproteinase-2 with homocysteine (20 microM-1 mM) with or without CuSO(4) (10 microM). We have demonstrated that biologically relevant levels of homocysteine in combination with copper can result in apoptosis as a result of oxidative stress; therefore, homocysteine has the potential to disrupt normal palate development.

Apoptosis↗

[Epidemiology of cleft palate and cleft lip inthe Rhône-Alpes/Auvergne/Jura region. Apropos of 903 cases registered 1978-1987].

Data from the Rhône-Alpes/Auvergne birth defects registry have been used to realise an epidemiological analysis of facial clefts (cleft palate and total cleft lip). Between 1978 and 1987, 903 cases have been ascertained giving an incidence of 0.67 per 1,000 for cleft lips with/without cleft palate (CLP) and 0.44 per 1,000 for cleft palates (CP). Several epidemiological characteristics have been studied: CLP are more frequent in males, and CP are more frequent in females. There is no detectable time trend, and birthweights are significantly lower in affected children than in the general population. There are more twins, more maternal epilepsy and more stimulations of ovulation in the studied sample than in the general population. The ranks of birth are higher in CP and CLP than in general population. The incidence of facial clefts in first degree relatives is 50 to 60 times the one in the general population, which is comparable to the literature findings.

Cleft Lip↗

The association of submucous cleft palate and clefting of the primary palate.

478 records of patients with cleft palate were reviewed to determine the prevalence and significance of submucous cleft palate associated with clefting of the primary palate. The prevalence of submucous cleft palate in the 71 patients with clefts of the primary palate (SMCP-CL) was 13 per cent. This is two to three times greater than the prevalence of isolated submucous cleft palate found in cleft palate clinic patients. Patients with SMCP-CL often had the symptoms of velopharyngeal incompetence (VPI) and middle ear disease. The increased prevalence of SMCP and frequent symptomatology of patients with clefting of the primary palate make it essential that patients with cleft lip have early, thorough evaluation for SMCP. Early detection of SMCP associated with cleft lip and close follow-up permits the prevention of ear problems and the proper management of VPI should it develop.

Child, Preschool↗

Incidence of cleft lip, cleft palate, and cleft lip and palate among races: a review.

A review of the literature pertaining to the incidence of cleft lip, cleft palate, and cleft lip and palate in different races is presented. The studies have been evaluated according to the method used to record the incidence rate. Half of the studies include in their base population livebirths, stillbirths, and abortions, or livebirths and stillbirths to record the incidence rate. In addition, in most of the studies, clefts with associated malformations and possible syndromes are included in the reported incidence. There is evidence, however, to suggest that the risk of developing clefts in stillbirths and abortions is three times as frequent as in livebirths and that clefts with associated malformations behave differently epidemiologically from clefts without associated malformations. It is suggested, therefore, that the incidence of cleft lip, cleft palate, and cleft lip and palate should be studied separately for each group, namely for livebirths, stillbirths, and abortions and should be reported separately for clefts without associated malformations, clefts with associated malformations, and syndromes. More research is needed to study the risk of developing clefts among the various groups that exhibit different epidemiologic behavior for each race.

Africa↗

Microform cleft lip associated with a complete cleft palate.

Clefting of the lip with or without an associated cleft palate may be present in varying degrees of severity. The so-called microform cleft lip or forme fruste has been characteristically described as having the appearance of a repaired cleft lip. The following case describes a patient with microform cleft lip and a complete cleft of the hard and soft palates. To the best of our knowledge, this is the first report of such an association.

Cleft Lip↗