Associated medical conditions among 10-year-old children with oral clefts - a retrospective review across three cleft centres: Part 2.

Introduction In many cases, children with oral clefts present with accompanying medical conditions. These associated conditions can add complexity to the patient's dental management, both in terms of their treatment need and risk. Recognition and careful consideration of associated medical conditions is therefore crucial in providing safe and effective care for these patients.Aim This paper is the second in a two-part three-centre series. It investigates the prevalence of medical conditions affecting cleft lip and/or palate patients attending three cleft units within the UK.Method Retrospective review was undertaken within three cleft units: South Wales (SW), Cleft NET East (CNE) and West Midlands (WM). This was completed via assessment of the 10-year audit record appointment clinical notes for the year 2016/2017.Results In total, 144 cases were reviewed (SW = 42; CNE = 52; WM = 50). Of these, 38.9% of patients (n = 56) had associated medical conditions recorded.Discussion The review highlights the variety and impact of medical conditions affecting UK cleft patients providing insight into the consequent complexity of their dental care.Conclusion An awareness of cleft lip and/or palate patients' associated medical conditions is important for all health care professionals involved in their care. Indeed, understanding of the patient's medical needs by multidisciplinary cleft teams is essential for effective planning and completion of holistic care. Involvement of specialists in paediatric dentistry sharing care with general dental practitioners is vital in providing appropriate oral health care and preventive support.

Advantages of using toothpaste containing propolis and plant oils for gingivitis prevention and oral cavity hygiene in cleft lip/palate patients.

BACKGROUND: The anti-inflammatory and antibacterial action of preparations used during oral hygiene procedures is particularly important in patients with oral cleft. Few reports have been published assessing the influence of natural products on the state of the oral cavity in patients with oral cleft. The aim of this study was to assess the effect of toothpaste containing Polish propolis and plant oils on oral cavity health in patients with oral cleft treated orthodontically. MATERIALS AND METHODS: A total of 50 patients aged 9-16 years old (20 females, 23 males) were selected and randomly assigned into two groups. Group (A) received toothpaste with Polish propolis, tea tree oil, menthol, and rosemary oil. Group (B) received toothpaste without active ingredients (placebo). A baseline assessment was followed by an oral hygiene index (OHI, debris OHI-D, and calculus OHI-C component) and gingival bleeding index (GBI) after 35 days. The methodology of the oral condition assessment included the presence of cleft malformation as a dysmorphic of the anterior maxilla segment. RESULTS: In group A, improvement in oral cavity hygiene assessed for incisors and molars was found (OHI-T p = 0.011). For the gingival condition, a decrease in the gingival bleeding index - total (GBI-T p = 0.002), as well as for the incisors (GBI-I p = 0.007) and molars (GBI-M p = 0.017) was found. CONCLUSIONS: This research confirms the biological effectiveness of toothpaste with Polish propolis and plant oils. These results may be clinically useful for improving preventative oral care and for control of oral infectious diseases during orthodontic treatment in patients with oral cleft.

[Evaluation of advantages of Contractubex gel usage for postsurgical scars treatment in comparison with absence of systematized topical scars treatment of kids with congenital cleft lip and palate]. / Otsenka preimushchestv primeneniya gelya Kontraktubeks pri posleoperatsionnykh rubtsakh v sravnenii s otsutstviem sistematizirovannogo mestnogo lecheniya u detei s vrozhdennymi rasshchelinami verkhnei guby i neba.

OBJECTIVE: Is to evaluate the advantage of Contractubex gel with regards to influence on vascularisation, pigmentation, thickness, surface size, configuration, and elisticity of postsurgical scars of children (after cheilorinoplasty) in comparison to absence of systematized topical treatment. MATERIAL AND METHODS: Into the prospective, non-interventional, observational, multi-centered, in parallel groups, open, controlled study were included 60 patients aged 2,5 months and older with postsurgical scars after first cheilorinoplasty after 7-14 day after operation. Patients were randomized into 2 groups of 30 patients in each. I group - patients get applications of Contractubex gel 3 times a day (in the morning, in the afternoon, in the evening) in accordance with patient information leaflet. II group - control group with no regular therapy of of postsurgical scars (without treatment or without application of oils and gels with anticsarring action). The period of medicine usage - 9 months and more for each patient, the each patient observation duration is 18 months. RESULTS: After analysis of the primary as well as secondary efficacy criteria (total grade based on POSAS scale, reported by investigator/parent) after 3, 6, 12, 18 months of observation in both groups a positive statistically significant dynamics was registered. At the same time in the Contractubex group results were statistically significantly better than in the control group. Positive dynamics was achieved quickier in the main group than in the contol group and was to observe already after 3 months of therapy, during the whole treatment and observation phase, and after 18 months of therapy. Additionally conducted photodocumentation of postsurgical scar development dynamics in terms of the study confirms positive effect of surgery and absence of visual data regarding keloids or hyperthrophic scars formation in patients in both groups. Adverse events, i. a. pain, itch, burning, long-run hyperemia were not registered during the whole period os study. CONCLUSION: The conducted study has shown high efficacy and safety of Contractubex usage for the treatment of postsurgical scars of children with with congenital cleft lip and palate (from 2,5 months old). The statistically significant advantage of the therapy with Contractubex was demonstrated in comparison with the control group (with no regular topical treatment). The obtained results allow to recommend Contractubex gel as an effective and safe medicine for the treatment of scarring after surgeries for kids directly after sutures removal.

Reading in subjects with an oral cleft: speech, hearing and neuropsychological skills.

OBJECTIVE: To evaluate speech, hearing, and neuropsychological correlates to reading among children, adolescents, and young adults with nonsyndromic cleft of the lip and/or palate (NSCL/P). METHOD: All testing was completed in a single visit at a Midwestern university hospital. Subjects in both the NSCL/P (n = 80) and the control groups (n = 62) ranged in age from 7-26 years (average age = 17.60 and 17.66, respectively). Subjects completed a battery of standardized tests evaluating intelligence, neuropsychological skills, and word reading. Subjects with NSCL/P also underwent speech assessment, and past audiology records were evaluated. RESULTS: After controlling for age and socioeconomic status, subjects with cleft performed significantly worse on a test of word reading. For subjects with cleft, word reading deficits were not associated with measures of speech or hearing, but were correlated with impairments in auditory memory. CONCLUSION: These findings show poorer reading among subjects with NSCL/P compared with those without. Further work needs to focus on correlates of reading among subjects with cleft to allow early identification and appropriate intervention/accommodation for those at risk.

Folate pathway and nonsyndromic cleft lip and palate.

BACKGROUND: Nonsyndromic cleft lip with or without cleft palate (NSCLP) is a common complex birth defect. Periconceptional supplementation with folic acid, a key component in DNA synthesis and cell division, has reduced the birth prevalence of neural tube defects and may similarly reduce the birth prevalence of other complex birth defects including NSCLP. Past studies investigating the role of two common methylenetetrahydrofolate reductase (MTHFR) single-nucleotide polymorphisms (SNPs), C677T (rs1801133) and A1298C (rs1801131), in NSCLP have produced conflicting results. Most studies of folate pathway genes have been limited in scope, as few genes/SNPs have been interrogated. Here, we asked whether variations in a more comprehensive group of folate pathway genes were associated with NSCLP, and were there detectable interactions between these genes and environmental exposures? METHODS: Fourteen folate metabolism-related genes were interrogated using 89 SNPs in multiplex and simplex non-Hispanic white and Hispanic NSCLP families. RESULTS: Evidence for a risk association between NSCLP and SNPs in NOS3 and TYMS was detected in the non-Hispanic white group, whereas associations with MTR, BHMT2, MTHFS, and SLC19A1 were detected in the Hispanic group. Evidence for over-transmission of haplotypes and gene interactions in the methionine arm was detected. CONCLUSIONS: These results suggest that perturbations of the genes in the folate pathway may contribute to NSCLP. There was evidence for an interaction between several SNPs and maternal smoking, and for one SNP with gender of the offspring. These results provide support for other studies that suggest that high maternal homocysteine levels may contribute to NSCLP and should be further investigated.

Community-based network system and interdisciplinary management for children with cleft-lip/palate.

OBJECTIVE: To propose a community-based network system and interdisciplinary management for children with cleft lip/ palate, applicable to Thailand and other developing countries. MATERIAL AND METHOD: A network was developed for the care of patients with cleft lip/palate by combining primary health care, community-based rehabilitation and institutional expertise (Tawanchai Center), Five workshops were conducted, including: 1) network development for cleft lip-palate care in the new millennium; 2) a multi-center study on the incidence and etiology of oral clefts and associated abnormalities in Northeast Thailand; 3) establishment of a Network for Children with Cleft Lip/Palate care in Northeast Thailand, including: 3.1) Skill development in cleft lip/palate care for parents and family; 3.2) a community-based model for speech disorders for children with cleft lip/palate in developing countries and for holistic nursing care for children with cleft lip/palate in developing countries; and, 4) development of interdisciplinary team system and network for holistic care for community-based quality of life, health promotion, speech and language intervention for Thai cleft lip/palate. RESULTS: A community-based network system model with interdisciplinary care was developed. CONCLUSION: A community-based network system model with interdisciplinary care can be applied for children with cleft lip/ palate in Thailand and developing countries where there exists a lack of coordinated multidisciplinary services. We plan to implement such a system in the near future.

Nested case-control study of one-carbon metabolites in mid-pregnancy and risks of cleft lip with and without cleft palate.

Evidence exists for an association between use of vitamin supplements with folic acid in early pregnancy and reduced risk for offspring with cleft lip with/without cleft palate (CLP). A few observations have been made about nutrients related to one-carbon metabolism other than folate. Our prospective study attempted to extend information on nutrition and CLP by measuring nutrient analytes in mid-pregnancy sera. This study included data from a repository of women's mid-pregnancy serum specimens collected in California from 2003-04. Each woman's specimen was linked with delivery information to determine whether her fetus had CLP or another structural malformation, or was nonmalformed. We identified 89 CLP cases. We randomly selected 409 specimens as controls. Specimens were tested for homocysteine, methylmalonic acid, folate, vitamin B12, pyridoxal phosphate, pyridoxal, pyridoxic acid, riboflavin, choline, betaine, methionine, methionine sulfoxide, cysteine, cystathionine, arginine, and asymmetric and symmetric dimethylarginine. We observed three analytes with odds ratios unlikely to be explained by random variation, i.e., elevated CLP risks were observed for low levels and for high levels of pyridoxal phosphate (vitamin B6), higher levels of choline, and low levels of symmetric dimethylarginine. These data did not show meaningful differences between cases and controls for any other analytes.

The effect of tongue appliance on the nasomaxillary complex in growing cleft lip and palate patients.

Midfacial deficiency is a common feature of cleft lip and palate patients due to scar tissue of the lip and palate closure procedure. The aim of this study was to evaluate the effectiveness of the physiological force of the tongue to move the maxilla in forward position. This research has been done experimentally by, before and after treatment following up in private practice. Ten patients (6 female, 4 male) with complete bilateral cleft lip and palate were selected. All of them had Cl III malocclusion with maxillary deficiency due to scar tissue of lip and palate surgery. Their age ranged from 7.6 to 9.8 years. All the patients were delivered tongue appliance to transfer the force of the tongue to maxillary complex. The mean observation time was 13+/-2 months to achieve positive overjet. Pre- and post-lateral cephalograms were compared to evaluate the skeletal changes with paired t-test. The results showed that after the application of tongue appliance, normal sagittal maxillomandibular relationship was achieved. SN-ANS angle was increased 1.9+/-1.8 - P < 0.03. This study showed that the tongue appliance could transfer considerable force during rest and swallowing period to the maxilla. This method might be considered to improve the deficient maxilla by means of growth modification and redirect concept.

Case report: surgeon and orthodontist work together from patient's birth.

This case report presents one of the initial participants of a new treatment protocol started in 1965 for children born with complete clefts of the lip, alveolus, and palate at Children's Memorial Hospital, Chicago, USA. The surgeon and orthodontist worked together and in tandem from the time of the patient's birth. The protocol involves lip and palate closure, along with the placement of a passive maxillary prosthesis and minimal primary osteoplasty to the alveolus to help stabilize the maxillary segments. This case is noteworthy in that the patient had a number of congenitally missing teeth, and treatment required moving a tooth into and through an area originally cleft. The patient is now 40 years of age. Intra- and extraoral photographs, cephalometric radiographs, occlusal dental radiographs, and dental casts through the treatment stages are presented.

Socio-economic status and risk of conotruncal heart defects and orofacial clefts.

Studies of socio-economic status (SES) have figured prominently in research related to a variety of health outcomes, although the question remains as to whether SES contributes to the aetiologies of congenital anomalies. This study examines the association of SES with risks of conotruncal heart defects and orofacial clefts, using interview data from 696 case mothers (86% of eligible) and 734 (78%) control mothers from a population-based case-control study. Socio-economic measures from maternal interview included mother's education and employment. Reported addresses were linked with the US census to characterise six measures of neighbourhood SES (education, poverty, unemployment, occupation, crowding and rental occupancy). Results were adjusted for race-ethnicity, multivitamin/mineral supplement intake, cigarette smoking and binge drinking. Results for individual and neighbourhood measures suggested that low SES was associated with increased risk of d-transposition of the great arteries (dTGA), reduced risk of tetralogy of Fallot (TOF), but was not associated with risk of orofacial clefts. For example, when examining odds ratios (OR) that compared risks among women whose neighbourhoods were in the lowest vs. highest quartile of the census-based SES measures, ORs for five of the six measures were> 1.4 for dTGA, and ORs for all six measures were < 0.7 for TOF. ORs for clefts tended to be closer to 1. This study suggests that SES risks are birth defect specific.

Does the interaction between maternal folate intake and the methylenetetrahydrofolate reductase polymorphisms affect the risk of cleft lip with or without cleft palate?

Periconceptional folic acid supplementation may reduce the risk of cleft lip with or without cleft palate (CL(P)). Polymorphisms in the methylenetetrahydrofolate reductase (MTHFR) gene reduce availability of 5-methyltetrahydrofolate, the predominant circulating form of folate. To determine the effect of MTHFR C677T and MTHFR A1298C genotypes and haplotypes on CL(P) risk and the interaction with maternal periconceptional dietary folate and folic acid supplement intake, the authors conducted a case-control triad study in the Netherlands (1998-2000) among 179 CL(P) and 204 control families. Infant and parental MTHFR C677T and MTHFR A1298C genotypes and haplotypes were not associated with CL(P) risk in the case-control and transmission disequilibrium test analyses. Mothers carrying the MTHFR 677TT genotype and who either did not use folic acid supplements periconceptionally or had a low dietary folate intake, or both, had an increased risk of delivering a CL(P) child (odds ratio (OR) = 5.9, 95% confidence interval (CI): 1.1, 30.9; OR = 2.8, 95% CI: 0.7, 10.5; OR = 10.0, 95% CI: 1.3, 79.1, respectively). No supplement use, low dietary folate intake, and maternal MTHFR 1298CC genotype increased the risk of CL(P) offspring almost sevenfold (OR = 6.5, 95% CI: 1.4, 30.2). Thus, the detrimental effect of low periconceptional folate intake on the risk of giving birth to a CL(P) child was more pronounced in mothers with the MTHFR 677TT or MTHFR 1298CC genotype.

Dental experience of cleft affected children in the west of Ireland.

AIM: To investigate caries experience and initial access to dental services in a group of children with cleft lip/palate in the west of Ireland. DESIGN AND METHOD: Cross sectional study with prospective data capture and matched control. Details of children born with a cleft were obtained from all health professionals likely to be involved in delivering care to these children. Existing databases were cross-referenced to eliminate duplication or missed patients. A matched control sample was recruited from 14 schools in the region. RESULTS: A sample of 90 cleft affected children (48 male, 42 female) with any category of cleft born between 1980-1996 (i.e. 16 years) was compared with a control group of 100 non cleft children (60 male, 40 female). The DMF index was determined by a trained and calibrated clinician. Twenty-two percent (n=20) of the cleft group were caries free compared to 41% (n=41) in the control group. The combined dmf/DMF for the cleft group was 2.09 compared to 1.50 for the control (P<0.05). Separate analysis of the dmf and DMF between the two groups indicated that the difference lay in the caries found in the deciduous dentition of the cleft group. The first dental visit was at 4 years of age for the cleft group. CONCLUSIONS: Cleft affected children in the region did not receive adequate and regular dental care at the appropriate time. In view of the significantly greater risk of dental disease in clefting, particularly in the deciduous dentition, all cleft affected children should be referred for comprehensive and continued preventive dental care from the first year of life.

Comparative anti-caries effects of tablet and liquid fluorides in cleft children.

Children with cleft lip and/or palate are at a higher risk for developing caries of the primary incisors compared with non-cleft children. To determine whether fluoride in tablet or liquid form would be more efficacious with children with cleft lip and/or palate, a two-year clinical investigation was conducted to test the anti-caries effects. One-hundred and fifteen cleft children (59 boys and 56 girls) between 22 and 26 months old were randomly selected into control, tablet and liquid fluoride groups. The amount of administered fluoride was 0.25 mg F daily in non-fluoridated Taiwan. Dental examinations were conducted using mirrors and #23 explorers. Caries were assessed using the DMF index in the baseline, first year and second year. The results showed that children in the tablet and liquid groups had a significantly lower DMFT increment than in the control group (p < 0.05). In the DMFS index, children in the liquid group showed a significantly lower caries increment than in the control group (p < 0.01), and children in the tablet group presented a borderline, but non-significant statistical difference when compared with the control group (p = 0.065). No significant statistical difference was found in either DMFT or DMFS between tablet and liquid fluoride administrations (p = 0.521 and p = 0.383, respectively). It is concluded that dietary fluoride supplements in liquid form show efficacy in reducing early childhood caries in the cleft children. Liquid fluoride showed slightly better numerical anti-caries effect than tablet fluoride, which is possibly due to its ease of administration with small children.

Cortical auditory dysfunction in children with oral clefts: relation with cleft type.

OBJECTIVE: Up to 46% of individuals with oral clefts suffer from language-learning disabilities. The degree of these disabilities varies according to cleft type. The pathogenesis of cognitive malfunctioning or its relationship with cleft type is not known. We investigated persistence of auditory short-term memory (STM) that is implicitly involved in language-specific perception in children with clefts, grouped using fine-graded cleft classification. METHODS: Cortical evoked potentials were recorded in 78 children with non-syndromic oral clefts and in 32 healthy peers. A mismatch negativity (MMN) potential that indexes preattentive detection of change in auditory input was obtained in response to tone sounds. In order to test durability of short-term memory traces, sounds were presented with three stimulation rates. RESULTS: With slowest stimulation, MMN amplitudes were reduced in cleft children as compared to the healthy peers (P < 0.00065). Only cleft-lip children did not significantly differ from controls. Among isolated palatal clefts, the more posteriorly delimited the cleft was, the smaller was the amplitude of MMN. MMNs of smallest amplitudes were obtained in the subgroup of complete unilateral cleft of lip and palate. CONCLUSIONS: Reduced MMN amplitudes, found in cleft children, imply deficiency in auditory STM trace maintenance. This dysfunction is likely to contribute to their language and learning disabilities. The MMN diminution with shorter/more posterior clefts suggests that differences in auditory cortex function are one of the underlying mechanisms of the cleft type-malcogniton association.

Otoacoustic emissions in screening cleft lip and/or palate children for hearing loss--a feasibility study.

Patients with cleft lip and/or palate abnormalities are likely to suffer permanent conductive (PCHL) or sensorineural hearing loss (SNHL) due in part to the association with syndromes known to include PCHL or SNHL. The presence of otitis media, a nearly universal complication in these patients, makes identifying the hearing impaired in this population a challenge, since the detection of permanent hearing loss is made more difficult. This problem might be overcome by using click-evoked otoacoustic emissions (CEOAE's) shortly after birth. Twenty-one out of 28 newborns presented to the regional cleft palate team were eligible for CEOAE screening shortly after birth. Among these 21 infants, five had anomalies other than cleft lip and/or palate associated with an increased risk for hearing impairment. At the first CEOAE screening (ILO88 Emission Analyser, mean age 3 weeks, range 1-11 weeks) clear CEOAE's were present in all of the 18 ears of nine infants with isolated lip and/or jaw clefts. In 24 ears of 12 infants with palatal clefts, CEOAE's were present in only six ears (three infants), not demonstrable due to noisy registration in another six ears and absent in 12 ears despite a quiet registration. During follow-up of those who failed the first screening (18 ears, nine infants), normal hearing was found in 14 ears (seven infants) and sensorineural hearing loss was documented in four ears (two infants). CEOAE screening in infants with isolated lip and/or jaw clefts seems feasible and effective. In infants with palatal clefts an auditory brain stem screening might be more appropriate.

[Video-feedback therapy with the flexible nasopharyngoscope. The potentials for modifying velopharyngeal closure and phonation deficiencies in cleft patients]. / Video-Feedback-Therapie mit dem flexiblen Nasopharyngoskop. Einflussmöglichkeiten auf den velopharyngealen Verschluss und Lautbildungsfehler bei Spaltpatienten.

Psychological interviews with cleft palate patients have revealed why a number of these patients do not profit, or do so only on a limited basis, from conventional speech therapy. They don't control articulation by means of the auditory canal. To treat these patients the video feedback therapy with the nasopharyngoscopy (Witzel et al.) was employed as the initial step. The next step was to expand on this method by establishing indication criteria, necessary diagnostic techniques, and an efficient therapy plan. In this paper a single case study is employed to discuss this method and show the results attained by its use.

Experience with alternative material for nasal augmentation.

In the last six years, 101 Proplast II implants were used for correcting various nasal deformities. The time of observation ranged from one to six years. During this period, four implants were removed: two because of late postoperative infection and two others because of trauma. Our experience indicates that Proplast II is a good alternative in cases that require alloplastic implants to correct an existing nasal deformity.