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Bone regeneration in the hard palate after cleft palate surgery.

BACKGROUND: The purpose of this study was to observe bony healing in the hard palate after cleft palate repair and to discuss the factors affecting it. METHODS: Fifty-two patients with repaired cleft palate were examined at least 1 year postoperatively by means of computed tomographic scanning. The incidence of bone regeneration automatically after repair was calculated, and the region of bone formation and the quality of bone tissue were measured according to the scanned image. RESULTS: The formation of a bone bridge was found in 37 of 52 patients (71 percent). The length of regenerated bone tissue (in the anteroposterior direction) ranged from 2 to 20 mm, with an average length of 8.3 mm. The male-to-female ratio of the patients who had a bone bridge was 1:1. There was no obvious difference between the two different cleft types. Considering the relationship of age at operation and bone formation, the most popular age at which bone bridging occurred was 4 to 7 years, and the location in the hard palate with the highest percentage of bone formation was the area between the premolar and anterior part of the molar. CONCLUSIONS: Regenerated bone tissue can occur after palatal repair in cleft palate patients. The age at operation could be an important factor affecting regeneration of bone tissue. The authors were not able to find a significant effect of sex or clinical type of cleft palate based on the sample size in this study.

Adolescent↗

Craniofacial cephalometric morphology in six-year-old girls with submucous cleft palate and isolated cleft palate.

Ninety-three girls with cleft palate (53 submucous cleft palate (SMCP) and 40 isolated cleft palate (ICP)), mean age 6.2 years (range 5.5-7.5), were compared retrospectively from lateral cephalograms. Forty-three patients with SMCP had had surgical treatment at the mean age of 3.4 years (range 0.9-6.8), 10 of the SMCP patients were unoperated. Twenty-six patients with ICP had clefts of the hard and soft palate, and 14 had clefts of the soft palate only. Palatal closure of ICP had been done at the mean age of 1.5 years (range 1.0-2.1). The skeletal craniofacial morphology was similar in SMCP and ICP. The maxilla and mandible were well related to each other but slightly retrusive in relation to the cranial base. The slight skeletal retrusion was significantly more masked by soft tissue in patients with SMCP. The patients with SMCP showed higher values for soft tissue maxillary and mandibular prominence.

Cephalometry↗

[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↗

The pattern of palatal rugae in submucous cleft palates and isolated cleft palates.

The early diagnosis of submucous cleft palate is important. In children too young to tolerate nasendoscopy and videofluoroscopy the diagnosis depends on the clinical history and intraoral examination. We have studied the pattern of the hard palate rugae to investigate their possible diagnostic significance. Maxillary dental casts were obtained from 16 patients with submucous cleft palate, 17 patients with isolated clefts of the secondary palate, and 10 non-cleft controls. The hard palate mucosa had a unique feature in 14 (87.5%) of the submucous cleft palates: one or more of the palate rugae curved towards the region of the bony notch in the posterior border of the hard palate. The 2 cases without this rugae pattern did not have a detectable bony notch. In 100% of the isolated cleft palate cases, one or more of the rugae curved towards the anterior end of the cleft. This feature was not seen in any of the non-cleft controls. We consider this rugae pattern to be an additional diagnostic feature of submucous cleft palate.

Child↗

Study of four genes belonging to the folate pathway: transcobalamin 2 is involved in the onset of non-syndromic cleft lip with or without cleft palate.

Cleft lip with or without cleft palate (CL/P) is the most common inborn craniofacial anomaly. Affected individuals require extensive medical and psychosocial support. Although CL/P has a complex and poorly understood etiology, increasing evidence of folate pathway involvement has been collected. So far, only the MTHFR gene has been extensively investigated as a risk factor for CL/P, while little has been done to test genetic variations in the folate biosynthetic pathways that may influence the infant's susceptibility to these birth defects. To date, this paper presents the first attempt to verify the involvement of four genes belonging to the folate pathway in nonsyndromic cleft onset. We used a case-parent triad design to test for linkage disequilibrium in the case of seven SNPs mapping on four different genes: transcobalamin 1 and 2 (TCN1 and TCN2), methionine synthase (MTR), and MTR reductase (MTRR). Our finding suggests that TCN2 is involved in causing CL/P. Indeed, significant overtransmission of the C allele was observed at the polymorphism c.776C>G (p.Pro259Arg) to the affected offspring (P=0.01). Results obtained with additional TCN2 polymorphisms suggest that c.776C>G may be functionally related to CL/P. However, because conflicting data exist with regard to the effect of the polymorphism in transcobalamin 2 function or in perturbing plasma levels of key molecules in the folate pathway, further investigation is warranted to confirm our data.

5-Methyltetrahydrofolate-Homocysteine S-Methyltran↗

Dental arches in six-year-old children with operated and unoperated submucous cleft palate and isolated cleft palate.

The sizes of dental arches in 129 children with cleft palate were evaluated retrospectively from dental casts taken at the mean age 6.2 years (range 5.2-7.5). The material included 61 children with submucous cleft palate (SMCP) and 68 children with isolated cleft palate (ICP). Twenty of the children with SMCP were not operated on, while 41 had had surgical treatment, either palatal repair (n = 16, mean age at operation: 1.6 years, range 0.8-3.9) or pharyngeal flap (VPP) surgery (n = 25, mean age at operation: 4.5 years, range 2.6-6.2). In children with ICP, one-stage hard-palate and soft-palate closure had been done at the mean age of 1.5 years (range 1.0-2.1). Decreased maxillary intermolar widths were seen in children with SMCP after VPP, and especially after palatal repair. The children with ICP had the smallest maxillary dental arch widths. No significant differences were observed in the maxillary arch length or mandibular intermolar arch dimensions in children with SMCP or ICP. Surgery is associated with decreased maxillary intermolar arch widths in children with SMCP. Children with ICP had smaller maxillary dental arch widths than children SMCP.

Child↗

Midface distraction in patients with cleft palate.

Cleft palate patients with a severe maxillary hypoplasia can pose a very challenging problem for the surgeon. Traditional orthodontic surgery methods frequently do not fulfil expectations from the point of view of achieving normal aesthetic proportions of the face. Therefore distraction is nowadays an important technique in the treatment of craniofacial skeletal dysplasias. To treat maxillary hypoplasia, either intraoral midface distractor IMD or rigid external distractor RED can be used. This study presents 16 patients. In five of them, we used the IMD and in 11 of them the RED. In all of the patients we achieved good functional as well as aesthetic results. While using this method, complications were minimal. In this study, we analyse surgical and orthodontic methods as well as the advantages and disadvantages of both surgical methods.

Adolescent↗

Familial recurrence-pattern analysis of cleft lip with or without cleft palate.

Cleft lip with or without cleft palate (CL/P) is a common congenital malformation with an incidence in European white populations of about 1/1,000. The familial clustering of CL/P has been extensively characterized, and epidemiological studies have proposed monogenic models (with reduced penetrance), multifactorial/threshold models, and mixed major-gene/multifactorial models to explain its inheritance. The recognition of an association between two RFLPs at the transforming growth factor alpha (TGFA) locus and CL/P supports a major-gene component to the etiology of CL/P. Risch has shown that the recurrence risk ratio lambda R (risk to relatives, vs. population prevalence) is a useful pointer to the mode of inheritance. Here we further develop the use of lambda R to analyze recurrence-risk data for CL/P. Recurrence risks for first-, second-, and third-degree relatives equate well with oligogenic models with as few as four loci. A monogenic/additive model is strongly rejected. The limited available twin data are also consistent with this model. A "major gene" interacting epistatically with an oligogenic background is shown to be a plausible alternative. Power calculations for a linkage study to map the CL/P major-risk locus suggest that a sample of 50 affected sib pairs will be adequate, but linkage to minor-risk loci will require very much larger samples.

Chromosome Mapping↗

[Isolated cleft palates compared to cleft palates associated with Pierre Robin syndrome].

The purpose of this study was to evaluate two variables associated with isolated cleft palate and cleft palate related to Pierre Robin syndrome, namely, sexual incidence and dental anomalies. The study demonstrated that there are more dental anomalies associated with the Pierre Robin syndrome and that girls are more affected than boys in both instances.

Cleft Palate↗

Differentiation between cleft lip with or without cleft palate and isolated cleft palate using parental cephalometric parameters.

OBJECTIVE: The purpose of this study was to identify and compare lateral cephalometric measurements in noncleft parents of children with cleft palate (CP) and cleft lip (CL), and cleft lip and palate (CLP). The hypothesis was that discriminant analysis would enable identification of morphometric features that predispose to orofacial clefting and that differ for CP, CL, and CLP and are unevenly distributed within parental pairs. DESIGN: This was a prospective, parametric analysis. SETTING: The study was conducted by the Department of Dental Health, University of Dundee, and the Department of Statistics, University of Glasgow, Scotland. SUBJECTS: From a completely ascertained sample of 286 children with cleft lip and/or palate born in the West of Scotland between January 1, 1980, and December 31, 1984, a sample of 83 parents of children with non-syndromic clefts volunteered for lateral cephalometric examination. METHODS: Thirty-seven cranial and 99 facial landmarks were identified and 37 linear, angular, and area parameters were used to describe the craniofacial skeleton. Analysis of variance was used for a three-way comparison of CL/CLP/CP, and stepwise discriminant analysis was used to determine which variables discriminate best between cleft lip with or without cleft palate [CL(P)] and isolated cleft palate (CP) parents. RESULTS: There were no significant differences whatsoever in the craniofacial morphology between the parents of children with CL and CLP, but differences were found between the CL(P) and CP groups. The most significant of these were in mandibular length, ramus length, mandibular area, and cranial area. Mandibular ramus length alone discriminated between the two groups in 71.4% of CP and in 62.5% of CL(P) cases, while separate analysis of fathers and mothers showed that ramus length and cranial height together reliably distinguish between mothers in 75% of CP and 80% of CL(P) cases. CONCLUSIONS: Previous studies suggests that unaffected parents with non-syndromic children with cleft lip and/or palate have differences in their craniofacial morphology when compared to the general population. This study indicates that these morphologic features differ for CP and CL(P).

Analysis of Variance↗

Speech after repair of isolated cleft palate and cleft lip and palate.

The speech of children with isolated cleft palate (CP) repaired by one surgeon has been compared with the speech of children with some form of unilateral cleft lip and palate (CLP) repaired by the same surgeon. All palate repairs included an intravelar veloplasty. We identified 57 children (5--12 years old) with cleft palates repaired in infancy, of which three patients with other medical problems were excluded. Of the 54 patients, 44 (81%) attended for review (27 CP, 17 CLP). Video recordings were analysed by two speech and language therapists, using the Cleft Audit Protocol for Speech. The CP patients had no evidence of permanent fistulas. Final speech outcomes were similar for CP and CLP patients. Intelligibility was normal in 10 (37%) CP and nine (53%) CLP patients. Mild consistent hypernasality was present in five (18.5%) CP and four (23.5%) CLP patients. No patients had moderate or severe hypernasality or nasal emission. Mild consistent hyponasality was present in five (18.5%) CP and five (29%) CLP patients. Moderate consistent hyponasality was present in one (4%) CP patient. Dysphonia was present in eight (30%) CP and seven (41%) CLP patients. Cleft-type characteristics were noted in 11 (41%) CP and nine (53%) CLP patients. No CLP patients but 10 (37%) CP patients had required a pharyngoplasty (P=0.004, Fisher's exact test). Possible reasons for this (age, cleft type, surgeon and surgery) are discussed.

Child↗

[Otological findings in adults with isolated cleft palate or cleft lip, jaw, and palate].

BACKGROUND: Children with cleft palate often develop middle ear ventilation disorders due to chronic Eustachian tube dysfunction. This may lead to hearing loss. The insertion of ventilation tubes is a widely accepted measure to avoid sequelae of middle ear ventilation disorders and hearing loss. On the other hand, long-term therapy with ventilation tubes may inflict iatrogenic complications. The objective of the study was the evaluation of otoscopic and audiometric long-term findings in adult cleft patients who had been treated with ventilation tubes since childhood when chronic otitis media with effusion had been observed. PATIENTS AND METHODS: Ninety-two cleft palate patients had been followed up otoscopically and audiometrically for years. The average age was 19.3 years (minimum: 14, maximum: 39 years) at the time the last status was taken. RESULTS: Otoscopy revealed a perforation of the tympanic membrane in 3.8% of the 184 ears. 12% of the patients developed cholesteatoma, however three quarters of these occurred after age 11. 86.4% of the 92 patients had normal hearing in pure tone audiometry. CONCLUSION: Compared with adult cleft patients who did not receive ventilation tubes, our patients had a similar low incidence of eardrum perforations but a higher incidence of cholesteatomas while hearing loss occurred less often. Whether the higher incidence of cholesteatomas is caused iatrogenically or due to a longer follow-up period remains unclear. Whether the use of long-lasting ventilation tubes affects the incidence of cholesteatomas must be proved in further studies.

Adolescent↗

[Comparative rhinomanometric measurements in children with cleft palate after cleft closure with and without velopharyngoplasty].

To answer the question if a cranially based pharyngeal flap in patients with cleft palate could affect nasal breathing, 49 children were examined by active anterior rhinomanometry with and without decongestion of the nasal mucosa. All patients were between 10 and 12 years old. In 18 of them palatoplasty with a cranially based pharyngeal flap was performed at the average age of 3.5 years. The control group consisted of 21 children who had been treated with an intravelar veloplasty without any flap at the age of 11-13 months. None of the patients had any appreciable narrowing of the nasal airways. Using the U test we found no significant difference between the inspiratory breathing volume of both groups. The difference between in- and expiratory volume in each group showed no significance either while the volumes we examined before and after decongestion differed significantly. Thus, we cannot conclude that the cranially based flap affects nasal breathing in cleft palate patients compared to those without any flap.

Child↗

[Obturators for cleft lip and cleft palate].

Cleft Lip and palate are most common congenital anomalies of the faces. Infants born with cleft lip and palate always have feeding problem. They were referred to dentists for obturators. Obturators usually have definite retention, lead to easily dislodgement. The author suggested the method of fabricating more retentive obturator.

Cleft Lip↗

Feeding infants with cleft lip, cleft palate, or cleft lip and palate.

In assessing 143 infants with cleft lip and palate, we found feeding problems to vary with the patients' anatomic lesion. Effective feeding techniques were identified by first assessing the infant's ability to generate negative intraoral pressure and to move the tongue against the nipple and then by matching these deficits to appropriate feeding devices.

Bottle Feeding↗