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A comparison of intelligence and social maturity in children with unilateral complete clefts and those with isolated cleft palates.

Intelligence and social development were evaluated in 226 subjects with palatal clefts. The subjects were divided into four groups composed of 111 with unilateral complete clefts of the lip and palate (Unilateral Group); 16 with unilateral complete clefts with associated congenital malformations (Unilateral-C Group); 76 with clefts of the palate only (Palatal Group; and 39 with clefts of the palate only with other congenital malformations (Palatal-C Group). Comparisons among groups suggested that subjects in the Unilateral Group were most competent both mentally and socially followed by those in the Palatal, Unilateral-C, and Palatal-C Groups. The presence of congenital abnormalities other than cleft increased the risk of developmental disabilities, particularly in subjects with isolated palatal clefts.

Child

Simplified method of feeding infants born with cleft palate with or without cleft lip.

Most infants born with a cleft palate with or without cleft lip are undergrown and have histories of difficult feeding. For the past two years, all of the infants born with isolated cleft palate with or without cleft lip, referred to the Boston Floating Cleft Palate Clinic, were fed using a simplified method. A standard nipple that was cross cut and a standard glass baby bottle were used. The infants were fed in the sitting position and burped frequently. Data indicating that nutrition in these infants is adequate as judged by weight gain was provided.

Bottle Feeding

Styloid, velar, and pharyngeal muscles in cleft palate. Anatomical findings in elderly cadaver with unrepaired cleft palate.

Dessection of the musculature of the palate, pharynx, and styloid process was carried out in a cadaver aged 78 years with an unrepaired complete cleft palate. A new muscle named "the accessory stylohyoid" was found to be attached to the styloid process and the lesser cornu of the hyoid bone bilaterally. Anatomical changes in other muscles especially the stylopharyngeus and palatopharyngeus are described. The functions of these muscles are discussed on the basis of the anatomical findings and previous observations of other investigators.

Aged

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

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

A lining vomer flap for palate pushback in unilateral cleft palate repair.

A combinaation vomer mucoperiosteal flap and nasal floor mucoperiosteal flap is described which is used to achieve nasal coverage in unilateral cleft palate patients requiring pushbacks. A posteriorly based readily accessible vomer flap is raised on the cleft side and used as nasal lining for the palatal mucoperiosteal flap on the non-cleft side. On the cleft side, a symmetrically sized nasal floor flap is easily elevated under direct vision and used to cover the nasal aspect of the corresponding mucoperiosteal palatal flap.

Cleft Palate

The mechanism of palatal shelf elevation and the pathogenesis of cleft palate.

Both normal Wistar rat fetuses and those with cleft palate induced by 5-Fluoro-2-Desoxyuridine were studied with a view to elucidating the mechanism of palatal shelf elevation and the pathogenesis of cleft palate. It was postulated that normal shelf elevation is brought about rapidly by an intrinsic turgor shelf force generated by binding of water to mucopolysaccharides. Interference with mucopolysaccharide synthesis would seem to be an important factor in the pathogenesis of some types of cleft palate.

Animals

Stage of palate closure as one indication of "liability" to cleft palate.

A new inbred mouse strain, SW/Fr, developed from a random-bred SW stock has a 6% incidence of spontaneous cleft palate without cleft lip. SW/Fr mice close their palates comparatively late in development. After cortisone treatment, the mean of the distribution (mean time to reach palate stage 5) is shifted towards later gestational ages. There is no change in the variance of the distribution. These data lend further support to the hypothesis that cleft palate in mice may fit a model where a continuous distribution is separated into discontinuous parts by a developmental threshold, and that time of palate closure is an important component of liability to cleft palate.

Animals

HLA phenotype frequencies in individuals with cleft lip and/or cleft palate.

The HLA types of 133 patients with cleft lip and/or cleft palate were determined. Caucasian patients with isolated cleft palate showed a possible association with HLA. Ten of 11 male patients had HLA--A2 as compared to one out of eight female patients (P less than 0.005). Caucasian males with cleft lip and cleft palate had a slight increased frequency of HLA--Aw24 (P = 0.07) and Mexican-American males with cleft lip and/or cleft palate showed an increase of antigen HLA-A28 (P = 0.07), though neither were statistically significant. Females with cleft lip and/or cleft palate from either racial group had no differences from the controls. The serum from 90 mothers of patients with cleft lip and/or cleft palate were reacted against their child's lymphocytes. Of these crossmatch tests, 12% were found to be positive. These preliminary results suggest that male patients with isolated cleft palate are worthy of further studies with respect of HLA associations.

Cleft Lip

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

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

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

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

Written language skills of children with cleft palate.

The written language abilities of children with cleft palates aged eight to 13 were studied to compare performance with norms for the Picture Story Language Test. Although intelligence of this sample, as measured by the performance portion of the WISC, was skewed toward the superior and very superior ranges, the group ranked below the 50th percentile in total words used, words per sentence, and syntax quotients. Also, percentile scores on the PSLT declined as the age of the subjects increased. These results differ from the Ebert, McWilliams, and Woolf (1974) study where younger children with cleft palates (ages six to eight) were found to exhibit age-appropriate written language. The influence of factors affecting language development among the cleft palate population, such as methods and success of management, hearing, articulation, intelligence, sex, and psychosocial adjustment needs to be further investigated.

Adolescent

6-Aminonicotinamide-induced cleft palate in the mouse: the nature of the difference between the A/J and C57Bl/6J strains in frequency of response and its genetic basis.

Cleft palate induction by 6-aminonicotinamide (6-AN) was examined in the A/J and C57BL/6J strains of mice to determine the nature of the strain difference in frequency of cleft-palate response. Probit analysis of the cleft-palate response to dose of different genotypes revealed a family of linear and parallel dose-response curves. The genotypes differ only in dosage tolerance (log ED50) to 6-AN that is required for the cleft-palate response. No evidence for a maternal cytoplasmic effect on 6-AN-induced cleft palate was found under the conditions of the present study. When the difference is dosage tolerance to 6-AN between A/J and C57BL/6J was examined with a single dose and measured by differences in frequency of induced cleft palate on a probit scale, there was some departure from genetic additivity. There was an indication off dominance deviation of the F1 embryos in the direction of C57BL/6J.A3-locus, epistatic model is proposed to account for the difference in embryonic tolerance ot 6-AN-induced cleft palate. There was a suggestion of association with the brown (b) locus.

6-Aminonicotinamide

Some aspects of corticosteroid-induced cleft palate: a review.

Since the discovery 25 years ago that cortisone can produce cleft palate in mouse embryos investigations into possible mechanisms of this corticosteroid-induced defect have been many and varied. However, the teratogenic mode of action remains not fully clarified. It is with this thought in mind that we have reflected upon what is known concerning corticosteroids and cleft palate. The major metabolic pathways upon which glucocorticoids act as well as their intracellular mode of action are well known. Differential sensitivity of various mouse strains to cortisone treatment as well as recent results from interstrain blastocyst transfer experiments demonstrate that corticosteroid action is influenced by both the fetal and maternal genomes. Labeling experiments indicate that corticosteroid-induced cleft palate is the result of direct action of the steroid molecule on the fetus, whose own sensitivity to insult, perhaps owing to differences in binding of corticosteroids to tissue proteins, determines the final effect. Possible mechanisms that have been proposed by which corticoids may produce cleft palate include: disruption of glycosaminoglycan or collagen synthesis or both, intracellular lysosomal membrane stabilization, myopathy, weakened midline fusion, and loss of amniotic fluid. Also discussed is the role of stress and stress-induced corticosteroids and their possible role in the production of cleft palate.

Adrenal Cortex Hormones