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Articulation proficiency and error pattern of cleft palate children with delayed hard palate closure.

Prior to 1982, children with cleft palate received delayed hard palate closure at Chang Gung Memorial Hospital. In this treatment, soft palate is usually repaired with Widmaier palatoplasty at about 18 months with surgery; hard palate is not closed until the age of 6 or 7. Our previous study indicated that the articulation skill of the cleft palate children before delayed hard palate closure is generally far inferior to the non-cleft children of the same age. Further investigation was carried out to look into the articulation proficiency and error patterns of the cleft palate children undergoing delayed hard palate closure. Data of 47 children (32 boys, 15 girls) were analyzed. Articulation proficiency was compared in four phoneme categories: nasals, plosives, fricatives and affricates. The results indicated that affricates are most difficult for Chinese cleft palate children to pronounce. At age 5, just before the hard palate closure, none of the phonemes is produced correctly except nasals.

Age Factors↗

The role of RXR-alpha in retinoic acid-induced cleft palate as assessed with the RXR-alpha knockout mouse.

Treatment of pregnant mice with retinoic acid (RA) in mid-gestation produces cleft palate and limb defects in the fetuses. RXR-alpha has been previously shown to mediate the teratogenic effects of RA in the limb. In this study, we show that RXR-alpha is also involved in retinoid-induced palatal clefting. Treatment of RXR-alpha knockout mice with a teratogenic dose of RA on gestation day 11 or 12 induces cleft palate at a lower frequency than that seen in wild-type animals.

Animals↗

Speech development of children with cleft palate before and after palatal surgery.

OBJECTIVE: This study examined the speech production abilities of children with cleft palate before and after palate repair. PARTICIPANTS: Twenty-eight children participated in the study, 14 with cleft palate and 14 without clefts matched for age, gender, and mothers' educational level. MAIN OUTCOME MEASURES: Comparisons were made between the children with cleft palate before and after surgery for canonical babbling ratios, size of consonant inventories, place and manner characteristics, and early developing sounds [p, b, t, d, k, g, m, n, eta]. Also, comparisons were made between the children with cleft palate and their peers without clefts at postsurgery/17 months for the measures described above. RESULTS: Paired t tests revealed differences in pre- and postsurgery performance of the children with cleft palate for production of canonical syllables and size of consonant inventories. Although no significant differences were noted for place and manner features, production of oral stops doubled from the time before surgery to that after surgery. Specifically, production of the bilabial stop [b] showed significant change over time. Results of independent t tests indicated no difference between groups for production of canonical syllables and size of consonant inventories at 17 months. However, significant group differences were noted for production of stops, oral stops, nasals, glides, and alveolars. The children without clefts produced more stops, oral stops, and alveolars. In contrast, more nasals and glides were seen in the vocalizations of the children with cleft palate. CONCLUSIONS: Children with cleft palate made gains in production of canonical syllables and size of consonant inventories postsurgery; however, they continued to show deficits in production of stops and alveolar place features.

Case-Control Studies↗

Anomalous middle ear gas absorption in a non-human primate model of cleft palate.

Following surgical clefting of the palates of nine Rhesus monkeys (Macaca mulatta), daily recordings of the middle ear pressure by tympanometry have shown fluctuations between high positive and negative values. High positive middle ear pressures were recorded during the recovery from otitis media, and it was suggested that these pressures were the result of a learned or reflexive autoinflation of the middle ear cavity. To test this hypothesis, animals presenting high positive middle ear pressures were anesthetized with sodium pentobarbital, and serial tympanograms were obtained at 5-minute intervals for a period of four hours. The results showed a substantial and rapid decrease in middle ear pressure following anesthesia (200 to 400 mm H2O). These findings were compared to those of similar experiments in normal animals, with near zero resting middle ear pressures or following air and oxygen politzerizations. The changes in middle ear pressure of cleft palate animals were significantly greater than those recorded for normal animals with near zero resting middle ear pressure and were essentially the same as those recorded for normal animals following air politzerization. These data suggest that abnormally high positive middle ear pressures are the result of an introduced bolus of air. This mechanism may serve as a means of ventilating the middle ear cleft in lieu of a debilitated active dilatory mechanism, and as such, may aid in the resolution of the middle ear effusion.

Absorption↗

A retrospective study of hearing impairment in three groups of cleft palate patients.

Three groups of cleft palate patients were evaluated for hearing impairment and secretory otitis at age five to determine the effect of myringotomy and tube placement. Each group consisted of 23 patients (46 ears). Hearing levels and otoscopic evaluations were obtained retrospectively from medical records. Group I had had no myringotomies. Group II had myringotomies after the initial palate closure and only when deemed necessary. Group III had had myringotomies at the initial palate closure and subsequently as needed. Optimal timing for myringotomy in cleft palate patients to maximize hearing at school age could not be determined. The need for myringotomy must be determined by clinical judgement on an individual basis.

Child, Preschool↗

[The effect of the postoperative use of a palatal plate for the prevention of maxillary contraction in cleft palate].

We have already reported that the maxillary was markedly contracted 1 month after a cleft palate operation. Since we thought that this was mainly attributable to cicatrized contraction of the operation region, we prepared a palatal plate and inserted it from an early stage after palate plasty. In this paper we present our summarized findings on the use of the palatal plate. We report on its use both immediately after the operation, and for a period of 3 months after the operation. Its use in both cases is intended to prevent maxillary contraction following surgical treatment of the cleft palate.

Cleft Palate↗

[An experimental study of the influence and mechanism of surgically induced cleft palate and palatoplasty on teeth and dental arch in dogs].

The dogs were selected as the experimental subjects in this study. The authors continuously observed the maxillary teeth and dental arch form in preoperation and 2, 4, 6, 10, 14, 18, 22, 26, 30, 34 weeks of post operation to all the animals. 40 puppies were divided into four groups, which were unoperated control group, surgically induced cleft palate group, cleft palate repaired group and cleft palate implanted bone group. The experimental result showed: there were remarkable differences at rate of itself growth between deciduous dentition and permanent dentition in the four groups. The result suggested that the cleft palate was a reason for disturbances of dental arch development, an exposed bone wound in hard palate of maxillary after palatoplasty was an important reason that it made maxillary dental arch growth problem heavier, authors could prevent the disturbs the transverse development of the maxillary dental arch from cleft palate who had or had not repaired by implanted bone in total cleft palate.

Animals↗

Influence of cleft palate closure by osteotomy technique on growth of the dentomaxillary complex in human beings.

Properly done, osteotomy cleft palate closure in human beings reproduces a normal dentomaxillary complex in patients with an incomplete cleft palate and in those with a narrow unilateral complete lip and palate cleft. In wider complete clefts, the dentomaxillary complex is influenced by the constrictive action of the lip muscle during closure rather than by the osteotomy procedure. Plaster casts of osteotomy surgical cases late postoperatively demonstrate the normal growth pattern achieved. Variations in cleft palate osteotomy have been worked out for every type of cleft palate.

Child, Preschool↗

Patterns of abnormal myogenesis in human cleft palates.

To test the hypothesis that soft palate muscles are abnormal in cleft palate, we compared soft palate morphogenesis in fetuses with cleft palate (n = 4) to age-matched (n = 3) and nonmatched (n = 1) control specimens. The morphologic status of all soft palate and masticatory structures were classified into one of six stages based on the level of histogenesis. At 54 mm crown-rump length (CRL), the levator veli palatini (L), palatopharyngeus (PP), and palatoglossus (PG) in cleft subjects demonstrated mesenchymal condensation into myoblastic fields, lagging behind the control specimens (97 mm CRL), which displayed definitive fields of myoblasts and myotube formation. In the 175 mm and 225 mm cleft and the 170 mm and 192 mm control specimens, muscular morphology was similar and had reached its postnatal appearance for the tensor veli palatini (175 m only) and L, PP, PG (225 mm only). Muscle fiber directions were, however, disoriented and disorganized, especially close to the medial epithelial edge of the cleft. The levator veli palatini, could not be distinguished as a discrete muscle in the cleft specimens, and what we believed to be the PP and PG seemed "normal" at the level of light microscopy, but malpositioned in a superior direction. This preliminary study demonstrates for the first time that early myogenesis in cleft palates differs from normal.

Case-Control Studies↗

An assessment of informative-counseling procedures for cleft palate children.

32 children with cleft palate and their mothers were interviewed for the purpose of learning what information they had about cleft and cleft management. Their responses were compared with standards specified by eight cleft palate specialists from four disciplines. The findings indicate that the specialists were somewhat inconsistent in what they expected the families to know about clefts and in their routine counseling procedures to provide the information. In general, the mothers had appropriate knowledge about clefts but predicted that their children knew more about clefts and cleft management than was actually the case. The children had less than adequate information about the general impact of a cleft, the etiology of clefts, the rationale for cleft management they had received, and future management of their problem. The results of the study indicate the need or more systematic procedures for providing information to the child with a cleft. The results also suggest the need for additional research about the general counseling process.

Adolescent↗

Community-based team approach to the management of children with cleft palate.

The child born with a cleft palate may have many associated problems in feeding, respiration, dentition, hearing, speech, language, and intellectual development as well as in psycho-social development. If the cleft is associated with a specific craniofacial syndrome, other physical problems may be present such as cardiac, kidney, digital, and visual involvement. The need for an extensive treatment program can extend from birth through adulthood. Important to the successful habilitation of these children is the early involvement of a multitude of specialists working together with the family in a team approach. The primary task of the team is to make decisions concerning timing and sequence of intervention for each individual patient. Several models for the delivery of health care services to the patient with a cleft palate are now in use. A community-based team model will be described in this article as a unique approach to the management of this population and as one that may be implemented in a cost-effective manner in communities where cleft palate teams presently do not exist.

Child↗

Prevalence of duplications and deletions of the 22q11 DiGeorge syndrome region in a population-based sample of infants with cleft palate.

The prevalence of duplications and deletions of the 22q11.2 (DiGeorge syndrome) region was studied among babies born in Norway with open cleft palate without cleft lip (cleft palate only, CPO). During a 5-year period (1996-2001), there were 245 live births with CPO that were referred for surgery. DNA was available from 174 cases with overt cleft palate. DNA copy number was analyzed with the multiplex ligation-dependent probe amplification (MLPA) technique, and an unambiguous result was obtained in 169 (97%) of the samples. We found no 22q11.2 duplications, and one known, and two previously undiagnosed cases with 22q11.2 deletions. All three del22q11-syndrome cases also had heart malformations, which represent one-third of the 10 babies with heart malformations in our study population. The prevalence of del22q11-syndrome among babies with cleft palate with or without additional malformations was 1 of 57 (1.8%). Because the prevalence of CPO in the 35 22q11.2 duplication cases published was 20%, we also investigated if dup22q11-testing was warranted in this group. However, no 22q11.2 duplications were found, indicating that the duplication cases ascertained so far might not be representative of the dup22q11-group as a whole. We conclude that neither del22q11 nor dup22q11 testing is warranted in babies with overt cleft palate as the only finding.

Chromosome Deletion↗

Cleft palate in two syndromes.

Patients with cleft palates with or without cleft lip need more special attention at birth when the cleft is associated with other malformations. For example, the Pierre-Robin and the Klippel-Feil Syndromes are both well known for the special problems they present. Reported here are three cases, with Crouzon's and two with Franceschetti Syndromes, all of whom have palatal clefts.

Adult↗

Comparison of cleft palate induction by Nicotiana glauca in goats and sheep.

The induction of cleft palate by Nicotiana glauca (wild tree tobacco) during the first trimester of pregnancy was compared between Spanish-type goats and crossbred western-type sheep. Cleft palate was induced in 100% of the embryonic/fetal goats when their pregnant mothers were gavaged with N. glauca plant material or with anabasine-rich extracts from the latter, during gestation days 32-41. Seventy-five percent of newborn goats had cleft palate after maternal dosing with N. glauca during gestation days 35-41, while no cleft palates were induced when dosing periods included days 36-40, 37-39, or day 38 only. The induced cleft palates were bilateral, involving the entire secondary palates with complete detachment of the vomer. Eleven percent of the newborn goats from does gavaged during gestation days 32-41 had extracranial abnormalities, most often contractures of the metacarpal joints. Most of these contractures resolved spontaneously by 4-6 weeks postpartum. One newborn kid also had an asymmetric skull due to apparent fetal positioning. No cleft palates were induced in lambs whose mothers were gavaged with N. glauca plant or anabasine-rich extracts during gestation days 34-41, 35-40, 35-41, 36-41, 35-51, or 37-50. Only one of five lambs born to three ewes gavaged with N. glauca plant material during gestation days 34-55 had a cleft palate, but all five of these lambs had moderate to severe contractures in the metacarpal joints. The slight to moderate contracture defects resolved spontaneously by 4-6 weeks postpartum, but the severe contractures resolved only partially. Embryonic/fetal death and resorption (determined by ultrasound) occurred in 25% of pregnant goats fed N. glauca compared to only 4% of pregnant sheep. Nicotiana glauca plant material contained the teratogenic alkaloid anabasine at 0.175% to 0.23%, dry weight, demonstrating that Spanish-type goats are susceptible to cleft palate induction by the natural toxin anabasine, while crossbred western-type sheep are resistant. However, clinical signs of toxicity were equally severe in goats and sheep, even though maternal alkaloid tolerance was generally lower in sheep. We postulate that an alkaloid-induced reduction in fetal movement during the period of normal palate closure is the cause of the cleft palate and multiple flexion contractures. Teratology 61:203-210, 2000. Published 2000 Wiley-Liss, Inc.

Abnormalities, Drug-Induced↗

[The influence of implantal autograft bone in total cleft palate on maxillary growth an experiment study in dogs].

OBJECTIVE: To test the hypothesis that implant autograft bone in total cleft palate at primary cleft palate repair which may resists the disturbance of maxillary growth by cleft and denud bony wound. METHODS: 40 dogs were randomly divided into four groups: three control groups and one experimental group. The first control group was unoperated, the second group had a surgically created cleft plate, the third group also had a surgically created cleft palate. Using a two-flap procedure immidiately, the experiment group had a surgically created cleft palate, but the palate was recontracted by implanting a piece of autograft rib bone with two-flap repair. All animals were sacrificed on the 34th week after operation (43 weeks postnatal). Thirty-four metricranial variables were measured directy from the cleaned skulls. RESULTS: The method is useful for maxillary growth and development by bone implant in total cleft palate, but it can cause maxillary deformity. CONCLUSION: Clinical doctors should be cautious in using the method of primary-delay bone grafting in reparing total cleft of cleft palate.

Animals↗

Double tongue, intraoral anomalies, and cleft palate--case reports and a discussion of developmental pathology.

OBJECTIVE: Isolated cleft palate is the most common presentation of the nonsyndromic cleft lip/palate combinations and is multifactorial in etiology. We report two cases of children with clefts of the secondary palate coexistent with double tongue and in either case mandibular epulis or superiorly displaced salivary gland. RESULTS AND DISCUSSION: In each case, the palatal cleft correlated anatomically with the intraoral space-occupying lesion. The ratio of tongue volume to intraoral volume during palatogenesis is discussed with reference to the pathogenesis of cleft palate. These clinical cases propose the model of a unifying sequence of developmental events whereby deformation of palatal shelf elevation results in secondary palatal clefting.

Choristoma↗

Malformation of the vomer in submucous cleft palate.

BACKGROUND: Several criteria are described in the literature to diagnose a submucous cleft palate. Commonly the differences in the extent of the submucous cleft will not be as overt as in open clefts. Nevertheless, complete submucous cleft palate may cause imperfect palato-pharyngeal closure so that the affected person needs to undergo speech training and surgical treatment. PATIENTS: We investigated 30 patients who underwent palatal repair to correct this disorder. They were evaluated according to the Koch's documentation system. RESULTS: In all patients an additional malformation of the inner nose was found: The vomer was not fused with the palatal shelves. There were different degrees of severity of this vomerine malformation and they were not necessarily correlated with the extent of the palatal cleft. CONCLUSION: In our opinion, this malformation of the vomer should be seen as a typical symptom of classical submucous cleft palate. Discussion is needed on how the vomerine malformation should be incorporated into the surgical procedure. Since we know from septal surgery that a basal septal perforation will lead to disturbances of nasal breathing.

Adolescent↗