[Professional socialization of patients with cleft lip, cleft lip and alveolus, cleft lip, alveolus and palate as well as their fathers].
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BACKGROUND: Cleft lip and cleft palate are common birth defects, affecting about one baby of every 700 born. Feeding these babies is an immediate concern and there is evidence of delay in growth of children with a cleft as compared to those without clefting. In an effort to combat reduced weight for height, a variety of advice and devices are recommended to aid feeding of babies with clefts. OBJECTIVES: This review aims to assess the effects of these feeding interventions in babies with cleft lip and/or palate on growth, development and parental satisfaction. SEARCH STRATEGY: We searched the Cochrane Oral Health Group's Trials register (June 2001), the Cochrane Central Register of Controlled Trials (CENTRAL) (The Cochrane Library, Issue 2, 2004), MEDLINE (1966 to May 24th 2004), EMBASE (1980 to August 7th 2002), CINAHL (1982 to August 7th 2002), PsychINFO (1967 to August 13th 2002), AMED (1985 to August 13th 2002). Attempts were made to identify both unpublished and ongoing studies. There was no restriction with regard to language of publication. SELECTION CRITERIA: Studies were included if they were randomised controlled trials (RCTs) of feeding interventions for babies born with cleft lip, cleft palate or cleft lip and palate up to the age of 6 months (from term). DATA COLLECTION AND ANALYSIS: Studies were assessed for relevance independently and in duplicate. All studies meeting the inclusion criteria were data extracted and assessed for validity independently by each member of the review team. Authors were contacted for clarification or missing information whenever possible. MAIN RESULTS: Four RCTs with a total of 232 babies, were included in the review. Comparisons made within the RCTs were squeezable versus rigid feeding bottles (two studies), breastfeeding versus spoon-feeding (one study) and maxillary plate versus no plate (one study). No statistically significant differences were shown for any of the primary outcomes when comparing bottle types, although squeezable bottles were less likely to require modification. No statistically significant difference was shown for infants fitted with a maxillary plate compared to no plate. A statistically significant difference in weight (kg) at 6 weeks post-surgery was shown in favour of breastfeeding when compared to spoon-feeding (mean difference 0.47; 95% CI: 0.20, 0.74). REVIEWERS' CONCLUSIONS: Squeezable bottles appear easier to use than rigid feeding bottles for babies born with clefts of the lip and/or palate, however, there is no evidence of a difference in growth outcomes between the bottle types. There is weak evidence that babies should be breastfed rather than spoon-fed following surgery for cleft lip. No evidence was found to assess the use of any types of maternal advice and/or support for these babies.
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.
The relation between chronologic age and the development of permanent teeth in patients with cleft lip, cleft palate, or both was investigated according to the method described by Demirjian et al (1973). The patients were divided into three groups: (1) those with bilateral cleft lip and palate (BCLP), (2) those with unilateral cleft lip and palate (UCLP), and (3) those with cleft palate only (CP). Panoramic radiographs of 107 Caucasian children (66 boys and 41 girls) aged 4 to 12 years were evaluated. The degree of maturation of each of the permanent teeth on the left side of the mandible was determined, and a dental maturation score was computed for each child. The scores were compared with those obtained in a previous study of dental maturation in Caucasian children without cleft from the Chicago area (Loevy, 1983). Evaluation of the data demonstrated that there was no significant difference in dental maturity between girls with cleft lip, cleft palate, or both and with the sample of those free from cleft. There was a significant difference in dental maturity in boys when all cleft groups were evaluated together. There was no significant difference from the normal sample in boys with bilateral cleft lip and cleft palate or with cleft palate alone, but there was a highly significant difference from the normal sample in the group of boys with unilateral 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.
OBJECTIVE: The purposes of this study were to investigate the caries prevalence in cleft lip, cleft palate, or both in children under the age of 2 years and to evaluate parental attitudes toward bottle-feeding, dental care, and their relationship to baby bottle tooth decay (BBTD) in Taiwan. DESIGN: Randomized and prospective study. SETTING: Institutional setting. PATIENTS AND METHODS: One hundred twenty-three 2-year-old children (68 boys and 55 girls) with cleft lip, cleft palate, or both were selected for this study. A questionnaire that asked questions about knowledge of oral health, knowledge and beliefs about BBTD, children's feeding habits, children's dental care, and parenting attitudes toward children with clefts was completed by the parents or caretakers. Children were divided into bottle-feeding and non-bottle-feeding groups according to the questionnaire responses of parents or caretakers. Each child was examined with a dental mirror and explorer under focused flashlight using defs index to determine the presence of BBTD. RESULTS: Thirty-nine percent (48) subjects reported a bottle-feeding habit; the overall prevalence of BBTD was 15.4%. The habit of bottle-feeding was significantly related to BBTD (p = .019). The defs score for children who were bottle-fed was significantly higher than children who were not bottle-fed (p = .045). Parents or caretakers of both bottle-feeding and non-bottle-feeding children showed no significant differences in their attitudes toward bottle-feeding and feeding habits (p > .05). However, parents of non-bottle-fed children had significantly better dental care than parents of bottle-fed children in brushing frequency (p < .001) and brushing before bed (p < .001). CONCLUSIONS: Children with clefts who took a bottle to bed showed an increased risk of developing BBTD. The parents or caretakers of bottle-fed children also showed a lack of motivation to perform regular preventive dental home care for their children. This suggests that oral health promotion programs should begin in infancy for children with clefts and their parents.
OBJECTIVES: To determine the incidence of cleft lip, cleft palate, spina bifida and hydrocephalus; and to compare these with those of Asian and European subjects. DESIGN: A retrospective study. SETTING: The Queen Elizabeth Central Hospital (QECH) in Blantyre, Malawi. SUBJECTS: Delivery and nursery records compiled between January 1998 and December 1999 were studied. MAIN OUTCOME MEASURES: To measure the incidence of cleft lip, cleft palate, spina bifida and hydrocephalus. RESULTS: Of the total 25562 births, 39 (0.2%) had defects; eight boys and 31 girls. Clefts (all types) comprised the largest group, 43.6%, with an incidence of 0.67/1 000; spina bifida, 30.8% with an incidence of 0.47/1000; hydrocephalus, 15.4%, and an incidence of 0.23/1000; and spina bifida with hydrocephalus, 10.3%, with 0.16/1000 incidence. The respective mean (SD) birth weights for clefts (all types), spina bifida, hydrocephalus and spina bifida with hydrocephalus were 2806.47 (569.07) g, 2650.00 (360.55) g 2610.00 (720.97) g and 3362.50 (696.87) g respectively. CONCLUSION: Our findings show a higher incidence of spina bifida in Malawians than in Caucasians and Orientals, but the incidence was lower than Omani subjects. The incidence of clefts and hydrocephalus in black Malawians, however, was lower than previously reported in other population groups, and hydrocephalus probably showed the lowest incidence among blacks in the continent. Furthermore, congenital malformations affected more girls than boys 4:1, but the infants had normal birth weight.
Numerous investigators have reported on a low frequency of other anomalies in patients with cleft lip, cleft palate, or both. The data have been somewhat inconsistent, ranging from a 3% to over 30% frequency of associated malformations. However, a recent study concluded that over half of the children with clefts at a large metropolitan center have associated anomalies. In an effort to elucidate further the genetic and morphologic characteristics of patients with clefts, 1,000 patients with clefts of the lip, palate, or both were examined and reviewed. The results indicate that associated anomalies occur in 63.4% of the sample. Approximately half of the patients with multiple anomalies have recognized syndromes, sequences, or associations, while the other half have physical examination (apparently one-of-a-kind) syndromes. The high frequency of associated anomalies has obvious implications for the genetic counseling offered to all patients at cleft palate and craniofacial centers. The frequency of associated anomalies also raises questions regarding the validity of past genetic research involving populations of subjects with clefts.
This paper reports a rare malformation syndrome which is observed in two sibs (brother and sister) of a family. It consists of nearly symmetric reductive defects of the limbs, flexon contractures of various joints, cleft lip and cleft palate, multiple minor abnormalities including capillary hemangioma of the forehead, hypoplastic cartilages of ears and nose, micrognathia, intrauterine growth retardation, and possibly mental retardation. Chromosomes of both parents and propositi are normal. Genetic data suggest autosomal recessive inheritance.
The more common consequences of mesodermal migrational delay or failure in the fourth to seventh weeks of gestational life and of plate fusion failure in the seventh to twelfth weeks of gestational life are cleft lip and cleft palate, respectively. The team approach with a balanced emphasis allowing optimal development of deglutition, speech, hearing, facial growth, and dental development combined with acceptable esthetic results provides comprehensive treatment and an improved outlook for this long term problem.
This article proposes a set of minimal standards for reporting the results of surgery on patients with cleft lip with or without cleft palate and cleft palate only. These standards do not represent what is technically possible, only what is considered minimally acceptable for presentation in a public forum. They have a clinical focus and should be attainable by any well-constituted cleft palate team. As the title indicates, this document is a proposal. It is hoped that the readership will respond to the recommendations presented so that some set of standards can be adopted in the near future. Once adopted, these standards should be reviewed and updated periodically.
OBJECTIVE: The aim of this study was to determine whether adult men with cleft lip and palate (CLP) have aberrant cerebral morphology. DESIGN: Brain morphology of 14 adult men with isolated CLP were analyzed and compared with 14 healthy controls matched for sex, age, and parental socioeconomic status. SETTING: The research took place at a large, tertiary care hospital, with participation on an outpatient basis. PARTICIPANTS: The 14 males with CLP were recruited from a large cleft lip and palate registry, while their 14 matched controls were selected from a registry of healthy volunteers collected via the Mental Health Clinical Research Center. RESULTS: The males with CLP have significantly smaller cerebellar size (p = .04), significantly larger frontal lobes (p = .02), and significantly smaller temporal and occipital lobes (p = .02; p = .009, respectively). No significant difference in gray/white matter ratios or laterality were found. CONCLUSIONS: Adult males with CLP have a significantly different pattern of brain morphology, compared with healthy controls, which is most likely due to aberrant cerebral development. This study highlights the complex interaction and interdependence of craniofacial and cerebral development.
OBJECTIVE: To evaluate the oral health of children with cleft lip and palate. DESIGN: DMFT (dmft) and DMFS (dmfs) were calculated on the basis of visual examination. Periodontal disease was assessed by classical clinical parameters: volume gain, bleeding, probing depth, loss of insertion, gingival recession. and calculus. PATIENTS: Seventy-eight individuals aged 1 to 32 years took part in the study. RESULTS: The dmft was 2.91 (+/- 3.99) in the 1- to 5-year-old age group and 2.77 (+/- 3.15) in the 6- to 12-year-old group. The DMFT averages for the age groups of 6 to 12 years, 13 to 18 years, and 19 to 32 years were 1.87 (+/- 1.78), 6.46 (+/- 3.11), and 13.62 (+/- 6.51), respectively. A total of 5.3% of individuals presented a healthy periodontium, and 86.6% presented with gingivitis and 8% presented with periodontitis. CONCLUSIONS: The dental and periodontal condition of individuals with cleft lip and palate was similar to the general population in the region in which this study was conducted. Good preventive measures should result in improved oral health similar to the general population and should be emphasized.
In recent years there has been some controversy over the analytical designs and the meaning of varying results with regard to studies of facial clefting and other common congenital malformations. Regardless, it is still unclear as to the nature of the genetic and environmental components of the etiology as well as the nature of the relevant pathogenetic mechanisms. Despite claims to the contrary, the predictions of a particular multifactorial/threshold inheritance (MF/T) model delineated by Carter [1977d] and others are not well supported by studies worldwide. The present study population consists of 1,895 persons born in Denmark with cleft lip with or without cleft palate (CL +/- P) between 1941 and 1968. A test of the MF/T predictions revealed the following: 1) the incidence of CL +/- P in siblings was 40 X greater than that in the general population 2) the risk to siblings of CL +/- P females was not significantly different from the risk to siblings of CL +/- P males; 3) recurrence risk for siblings of CL +/- P probands was dependent upon the proband's cleft type; 4) only 0.4% of the variation in risk to the siblings born after the proband could be accounted for by the number of previously affected siblings; 5) the consanguinity rate was 6 times less than the general population rate; 6) heritability estimates from siblings and parents by sex suggest, either the presence of significant dominance effects, or a common sibling environment component in the etiology of the disorder. Further, testing with a multiple-sex threshold method, designed and provided us by [Kidd and Spence, 1976] revealed that neither the MF/T nor single-major locus with random environmental variation provided a good fit. In light of recent experimental mouse and human data, an alternative model of monogenic-dependent susceptibility to a variety of teratogens is discussed.
A classical twin study is one of the best methods to address the open question of the role of genes and environment in the cause of cleft lip with or without cleft palate [CL(P)]. In addition, when twin concordance rates are combined with information about the risk for CL(P) to more remote relatives, they can help to establish the most likely mode of inheritance for CL(P). The present study was based on three nation wide ascertainment sources of CL(P) in twins in Denmark during the period 1970-1990. The Danish surgical files were found suitable for ascertaining twins with non-syndromic CL(P) and a total of 39 pairs was identified through these files. In more than 70% of the cases, the zygosity assignment was based on unlike-sex or an extensive panel of blood, serum, and enzyme types. More mono- and dizygotic twin (CL(P) cases than expected were found, although the difference was not significant. The proband concordance rate for CL(P) was 60% in monozygotic twins and 10% in dizygotic twins; six cases were of unknown zygosity. This finding indicates that genetic factors play a major role in the cause of CL(P) but environmental and/or stochastic factors are probably acting too. When the familial recurrence patterns analyzed by Mitchell and Risch [1992: Am J Hum Genet 51:323-332] are interpreted in light of these new estimates of monozygotic concordance, they provide further evidence for the finding that no single locus can account for more than a six-fold increase in risk to first-degree relatives.
OBJECTIVE: The purpose of this study was to examine the prevalence of cervical vertebral anomalies in individuals with cleft palate only (CPO) and bilateral (BCLP) and unilateral (UCLP) complete cleft lip and palate and make a comparison with a group without cleft. SETTING: This retrospective comparison was performed at the Dental Unit, Department of Plastic Surgery, National Hospital and at the Department of Orthodontics, Faculty of Dentistry, University of Oslo, Oslo, Norway. MATERIAL AND METHODS: Six hundred eleven subjects (334 boys, 277 girls) with three different cleft subtypes at age 6 years or older and 264 children (121 boys, 143 girls) without clefts were included in this study. Their lateral cephalometric radiographs were studied for cervical vertebral anomalies and categorized into posterior arch deficiencies or fusions. RESULTS: In the total cleft sample, 111 subjects (18.2%) had cervical vertebral anomalies; of these, 10 subjects had more than one anomaly. Posterior arch deficiency was found in 7.7% and fusions in 12.1%. In the sample without cleft, 9.1% had cervical vertebral anomalies, 5% posterior arch deficiency, and 4.1% fusions. When the cleft sample was divided into the three cleft subtypes, the prevalence of cervical vertebral anomalies was 25.6% in the CPO group, 16.3% in the BCLP group, and 11.1% in the UCLP group. Differences were statistically significant between the CPO and the group without cleft for both posterior arch deficiency and fusion anomalies (p <.01). CONCLUSION: Cervical vertebral anomalies occur more frequently in individuals with clefts as compared with those without clefts. This was statistically significant for the CPO group.
Good results in any surgical or orthodontic procedure require expert technique, well adapted to the problem and scrupulously executed. A technique that would achieve the best results can be described as "ideal" and can serve as a theoretical model for all similar cases. But, in dealing with apparently similar problems: cleft lips and palates, Class II or Class III cases... in reality, we are treating individual patients, none quite the same as any other. These differences derive from the varying characteristics of individual patients and from the varying and unpredictable responses of their tissues, and from their varying capacities to accommodate to and withstand insults, suffering, and the sensory-motor effects of their deformities and of the treatment they undergo, and, finally, from their variable readiness to submit to and to pay for treatment with their time and with their money. Any therapeutic technique must take into account these realities which sometimes oblige us to modify an ideal technique so that it will fit the specialized needs of a patient, an accommodation that can be defined as "therapeutic realism". When we ignore this reality, we risk the paradox of providing patients with technically ideal results that they find unsatisfactory or discover that what we thought was a technically mediocre outcome has delighted our patient: ultimately, it is the patient's judgment that determines the "therapeutic result" and is, in effect, the Final Evaluation of the technical result.
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