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PubMed2003

Prosthodontic treatment of the edentulous adult cleft palate patient.

Clefts of the upper lip and plate are relatively common, yet dental treatment of these patients is still very poor and many grow up suffering dental neglect. Dental practitioners should become involved in the treatment team as dental needs are present from birth to death. Adult cleft patients often need tooth replacement with obturation of any residual clefts. They are best treated with tooth-supported removable appliances including partial and complete overdentures, thus preservation of their natural dentition is desirable. Edentulous cleft palate patients present with restorative difficulties due to their compromised maxillary arches as well as the presence of scar tissue in their palates and lips. An outline of these complications and guidelines for their treatment is illustrated in the form of three case reports from members of one family all presenting with varying cleft lip and palate defects. This article highlights the need for dental students to be exposed to dentally compromised patients so that they will feel confident enough to treat them in private practice.

Adult↗
PubMed2003

Craniofacial morphology and growth comparisons in children with Robin Sequence, isolated cleft palate, and unilateral complete cleft lip and palate.

OBJECTIVE: Comparison of early craniofacial morphology and growth in children with nonsyndromic Robin Sequence (RS), isolated cleft palate (ICP), and unilateral complete cleft lip and palate (UCCLP). SUBJECTS: One hundred eight children with cleft: 7 with RS, 53 with ICP, and 48 with UCCLP were included in the study. The children were drawn from the group of all Danish children with cleft born 1976 through 1981. METHOD: Three-projection infant cephalometry. RESULTS: The craniofacial morphology in the RS, ICP, and UCCLP groups had some common characteristics: a wide maxilla with decreased length and posterior height, wide nasal cavity, short mandible, bimaxillary retrognathia, and reduced pharyngeal airway. The shortest mandible was found in RS followed by ICP and UCCLP; the pharyngeal airway was reduced in RS and ICP, compared with UCCLP; and the maxillary complex and nasal cavity were wider in UCCLP than in the other groups. The amount of facial growth in all three groups was similar; however, the direction was more vertical in UCCLP than in RS and ICP. CONCLUSION: Except for a shorter RS mandible, the facial morphology of infants with RS and ICP was similar, as was the amount of facial growth and the growth pattern. The differences in facial morphology can be ascribed to the difference in the primary anomaly. The amount of facial growth was similar in the three groups; however, the growth pattern showed a more vertical direction in UCCLP than in RS and ICP. It is hypothesized that the mandibular retrognathia in RS represents the outer end of that of the ICP distribution.

Analysis of Variance↗
PubMed2006

Study of the PVRL1 gene in Italian nonsyndromic cleft lip patients with or without cleft palate.

Nonsyndromic cleft lip with or without cleft palate (CL/P) is a complex genetic trait and little is known about its aetiology. Recent investigations on rare clefting syndromes provided interesting clues about genes involved in face development. The PVRL1 gene encodes nectin1, a cell-to-cell adhesion molecule. Mutations in its sequence have been shown to cause the rare autosomal recessive syndrome CL/P-ectodermal dysplasia syndrome (CLPED1), while heterozygosity for the mutation W185X seemed to increase the risk of non syndromic CL/P in a population from northern Venezuela. In the present study, we screened 143 Italian CL/P patients for mutations in PVRL1. Three rare sequence variants in exon 3 that create amino-acid changes were detected in a total of 7 patients. Two of these mutations were not found in a panel of 292 unaffected controls, while the third was found in two controls. This study describes new mutations that may represent genetic risk factors for CL/P. Even though a study to look at the effects of the mutations on nectin1 function was not feasible, supporting evidence was reported, thus confirming the involvement of PVRL1 in the aetiology of non-syndromic CL/P malformation.

Cell Adhesion Molecules↗
PubMed1978

A rapid method of measuring the palatal surface area of cleft palate infants.

A method is described for rapidly measuring the surface area of the palate by adapting a piece of soft plastic to a model of the upper jaw using a vacuum moulding technique. Tests with a hemisphere of known surface area showed the method to be consistent and to have a low degree of systematic error. When measuring models with unrepaired cleft palates, the error was found to be 2.1 per cent (compared with 3 per cent using stereophotogrammetry) but 7.1 percent when measuring intact palates. Because of the consistency of the method, the true palatal area could be calculated from the measured area by the use of a multiplying factor. These findings are described, and the application of the technique to the pretreatment assessment of cleft cases is illustrated by showing that, in a series of 30 cleft palate and 30 normal infants, a tissue deficiency of 16.8 per cent existed in the cleft subjects at birth.

Cleft Palate↗
PubMed1997

Evidence of linkage to 6p23 and genetic heterogeneity in nonsyndromic cleft lip with or without cleft palate.

Nonsyndromic cleft lip with or without cleft palate (CL+/-P) is a congenital orofacial anomaly that derives from an embryopathy with failure of nasal process and palatal shelves fusion. CL+/-P is one of the most common malformations, affecting 1/700-1/1000 live births among Caucasians. Early investigations have suggested that a clefting gene may be located on the short arm of chromosome 6 (6p), as well as in other regions. In this study, we analyzed a large sample of families by using eight PCR markers that map on chromosome region 6p23-p24. The admixture test, as implemented in the HOMOG program, was significant when tested against multipoint data (alpha = 0.60, P value 0.00004); the lod score calculated, assuming heterogeneity, was 3.60 at 1 cM telomeric to D6S259. Taken together these data demonstrate the presence of a locus for CL+/-P in the 6p23 chromosome region.

Chromosomes, Human, Pair 6↗
PubMed1996

[Language development in children with cleft palate].

Children with cleft palate often suffer from hearing and speech and language problems. Detailed knowledge about the long-term outcome of various therapeutic strategies including velopharyngoplasty or speech therapy seems mandatory to design an efficient rehabilitation plan for affected children. In this follow-up study 417 children with cleft palate (excluding isolated cleft lip) were examined in an interdisciplinary approach by maxillofacial surgeons, orthodontists, otolaryngologists, audiologists and speech and language pathologists. We found that 92% of the children had speech or language disorders. 80% of those children frequently had mild or severe conductive hearing loss, with or without clinical signs of otitis media with effusion. In 58 children (14%) with rhinolalia aperta not being improved by speech therapy for at least 1 year, velopharyngoplasty with a cranially based pharyngeal flap was performed. Language skills did not correlate with the type of cleft palate, but with the frequency and degree of hearing loss. Using this interdisciplinary approach for early detection and therapy of disorders related to cleft palate, only 49% of affected children had to undergo speech and language therapy. Based on the results of this follow-up study presented here we propose that otologic examination, such a thorough audiometric testing, and a detailed speech and language evaluation are important cornerstones in the rehabilitation management of children with cleft palate.

Adolescent↗
PubMed1991

Misarticulation caused by abnormal lingual-palatal contact in patients with cleft palate with adequate velopharyngeal function.

Misarticulations produced by three patients with cleft palate (2 isolated cleft palate; 1 unilateral cleft lip, alveolus, and palate) who attained adequate velopharyngeal function and normal palatal vault by early surgical repairs were examined using electropalatography (EPG) and sound spectrography (SG). Common characteristics of lingual-palatal contact in which the contact area was broader and/or was more posterior than normal were observed. These misarticulations can be divided into three types based on the direction of the breath emission: palatalized misarticulation (in which air passes along the midline of the palate), lateral misarticulation (in which air flows laterally through the occluded dental arch), and nasopharyngeal misarticulation (in which air flows out the nose). These three are considered to be similar to intractable posterior pattern of articulation in cleft palate patients previously reported. However, these types of misarticulations can be produced by cleft patients who have achieved adequate velopharyngeal function and normal palatal vault.

Articulation Disorders↗
PubMed2000

Cleft palate speech.

Cleft palate is a known pathology but there exists a lot of controversy about the therapy. The aim of this paper is to give a brief overview of the pathology itself and its consequences particularly in speech. At this point the velar and velopharyngeal function are stressed.

Articulation Disorders↗
PubMed1998

Survey of genetic counselors and clinical geneticists regarding recurrence risks for families with nonsyndromic cleft lip with or without cleft palate.

Nonsyndromic cleft lip with or without cleft palate (CL/P) is a common congenital malformation affecting about 1/1,000 caucasian infants. Although the familial clustering of CL/P has been studied thoroughly, estimation of recurrence risk for genetic counseling purposes can be difficult. A survey was mailed to 912 board-certified genetic counselors, 542 non-board-certified genetic counselors, and 776 board-certified clinical geneticists to investigate the recurrence risks they would assign to three example families with CL/P. Responses were received from 155 (17%) board-certified genetic counselors, 36 (6.6%) non-board-certified genetic counselors, and 100 (18.5%) board-certified clinical geneticists. No major differences were found in their responses, suggesting that for these three families, geneticists would provide similar estimates of risk, regardless of their amount of experience with oral clefts patients, where they are currently employed, or their board certification status.

Cleft Lip↗
PubMed1994

The 3-dimensional stability of maxillary osteotomies in cleft palate patients with residual alveolar clefts.

OBJECTIVES: To evaluate the stability of maxillary osteotomies in cleft palate patients using miniplate fixation. DESIGN: A prospective clinical study. SUBJECTS: 46 consecutive cleft palate patients with residual alveolar clefts and maxillary hypoplasia in one or more dimensions. These patients underwent standardised maxillary osteotomies and simultaneous bone grafting of the alveolar cleft over 44 months during 1988-1992. Titanium mini-plate fixation was used for the maxilla in all patients. Follow-up ranged from 6 to 51 months with a mean of 28 months. MAIN OUTCOME MEASURES: The 3-dimensional stability of maxillary osteotomies in cleft palate patients in the long term. RESULTS: In the unilateral clefts, relapse in the horizontal plane was 22% and in the vertical plane 22.5%; in bilateral clefts, the relapse was 17.5% and 7% respectively, with no statistically significant difference between the two groups. Longitudinal analysis of the repositioned maxilla over a 3-year period showed that most of the relapse occurred in the first 6 months and stabilised at 2 years postoperatively. Relapse in the transverse plane, based on analysis of the study models of 26 cases, ranged from 13.4% to 33.6%. A clockwise rotational relapse of the maxilla was noted in bilateral cases. Postoperative orthodontics compensated for the horizontal relapse by increasing incisor proclination to maintain positive overjet. There was no significant difference between the relapse of bimaxillary cases and that of maxillary osteotomies alone. CONCLUSION: The long-term 3-dimensional surgical stability, using miniplate fixation, has decreased the relapse of cleft maxillary osteotomies with simultaneous alveolar bone grafting to a level comparable to that of maxillary osteotomies in non-cleft patients.

Adult↗
PubMed1979

Maternal and child locus of control and field-dependence in cleft palate children.

The cleft palate experience includes events which are beyond the control of cleft palate children and their mothers. To investigate whether the experiences relate to other maternal and child variables, 44 cleft palate children, 9--18 years of age, and their mothers were compared to 61 control children and their mothers. Maternal variables included locus of control and perceived reactions to the child's birth. Child variables included locus of control, field dependence, parental perceptions, and teacher ratings. Mothers of children with cleft palates indicated a significantly greater negative impact at their children's births but did not indicate greater externality. Children with clefts were significantly more external in control, were more field-dependent, perceived parental reactions as more negative, and had more negative academic ratings. Mothers who were more external in locus of control had children who were more field-dependent and had more external control.

Adolescent↗
PubMedDate not supplied

Eustachian tube function in cleft palate children.

The cleft palate population has a high prevalence of chronic otitis media with effusion (OME). The present study attempts to relate this pathology to a functional obstruction of the eustachian tube (ET). Employing two methods, the forced-response test and the inflation-deflation test, various parameters of ET function were evaluated in 41 children and adolescents with cleft palates. The results indicated that cleft palate children have a limited ability to open the ET actively by swallowing, as evidenced by an inability to equilibrate applied positive or negative pressures. These children demonstrated little ability to alter pressure in the middle ear (ME) by either the Valsalva or Toynbee maneuver. Passive ET airflow resistance in these children is not different from that of a traumatic perforation group. However, in the majority of cases (73%), the resistance of the tube increased with swallowing, suggesting a constriction of the ET rather than a dilation. Further, for those individuals capable of tubal dilation, the efficiency of dilation as measured by the resistance ratio was significantly less than that reported for the traumatic perforation group. Eustachian tube function in this population was not demonstrably dependent on age, sex, or type of cleft. The cleft palate children of the present study had severe functional obstruction of the ET. This obstruction was primarily related to the inability of the tensor veli palatini (TVP) muscle to dilate the ET actively during swallowing and appears to be the major factor responsible for the pathogenesis of OME in this population.

Adolescent↗
PubMed1997

[The child with lip, maxillary, palatal cleft].

Clefting of lip, alveolus and palate may occur in multiple variations. It causes aesthetic and functional detractions. Soft palate clefts may result in hearing-, speech- and swallowing-disorders. Therefore the otolaryngologist is a very important member in the interdisciplinary team directory. A cleft-palate child belongs to an interdisciplinary consulting hour in special hospitals, where different medical specialties are involved. Most important for a sufficient medical rehabilitation are maxillofacial surgery, otolaryngology, paediatrics, plastic surgery, speech therapy, psychology and human genetics. Also many other specialties may be involved. The cleft demands a complete follow up from the child's birth until it is grown up. Hearing disorders are caused by eustachian tube disfunction. There is a high prevalence of hearing loss and middle ear diseases in cleft palate patients. Hearing losses due to middle ear effusions in the very young child. Without therapy up to 50% of the cleft palate population will develop chronic middle ear diseases with and without cholesteatomas. Early and consequent therapy with myringotomy and insertion of a tympanostomy ventilation tube is necessary and helps to avoid chronic hearing problems. There is no general accepted system of speech disorders in cleft palate patients because of the difference in shaping of the cleft and rehabilitation development. Essential for speech rehabilitation are an intact velopharyngeal system and a keen sense of hearing. Both of it is disturbed in cleft palate children. Speech disorders are treated by speech therapists with prior consultation of the interdisciplinary team. The author presents a system of primary, secondary and tertiary speech disorders in cleft palate children. Primary speech disorders are caused by faulty velopharyngeal valving, offering in hypernasality, weak plosives, fricatives and affricates. Secondary speech disorders are substitute mechanisms for plosives, nasal and pharyngeal sounds. Tertiary speech disorders are hyper- and hypofunctional dysphonias following primary and secondary speech dysfunctions.

Adolescent↗
PubMed2003

Three-dimensional morphology of the palate in subjects with isolated cleft palate at the stage of permanent dentition.

OBJECTIVE: Three-dimensional analysis of palate size and shape in patients with isolated cleft palate at the stage of permanent dentition. DESIGN: Cross-sectional study using Fourier transform profilometry. SUBJECTS: Twenty-nine randomly selected dental casts of approximately 15-year-old boys with isolated cleft palate and 28 dental casts of normal boys of the same age. INTERVENTIONS: All patients were operated on by the same method (pushback and pharyngeal flap surgery) at a mean age of 4.5 years. MAIN OUTCOME MEASURES: Data on the palate height in 210 defined locations (pixels). RESULTS: The palate in isolated clefts is narrower throughout its whole extent and lower from the level of the first premolars. The difference, as compared with controls, increases in a posterior direction. At the level of the first molars, palatal height is reduced by one-quarter, the area of the transversal section by more than one-third. The shaping of the palate vault is, on average, symmetrical with a marked interindividual variability. Palatal height does not depend on the width of the dentoalveolar arch, and the height of the primary palate is not reduced. CONCLUSION: The smaller width and reduced height from the level of the first premolars posteriorly confirm the substantially reduced space available for the tongue in patients with isolated cleft palate. Deviations are on the average symmetrical, and the anterior part of the palate is not shallower.

Adolescent↗