José Alberto de Souza Freitas

Oral Clefts and Vitamin Supplementation

OBJECTIVES The evidence linking low levels of folic acid and orofacial clefting (OFC) is presently equivocal. There is stronger evidence for the role of folic acid supplementation in protection against the occurrence and recurrence of neural tube defects. The present investigation tested the hypotheses that cleft lip, cleft palate, or both are inversely associated with maternal intake of dietary and supplemental vitamins during the periconceptional period and first 4 months of pregnancy in a Brazilian population. DESIGN A population-based, case-control study of cleft lip with or without cleft palate (CL(P)) and isolated cleft palate (CP) in a Brazilian population. In structured interviews, case histories were taken from the mothers of a consecutive sample of 450 infants born with nonsyndromic OFC. RESULTS Mothers who had children with CL(P) were less likely to have been supplemented during the periconceptional period. The statistical significance of the difference in prevalence of the use of supplements between mothers of patients and of controls was greater for the CL(P) group: p < .05 for CP and p < .001 for CL(P). Multivariate analysis confirmed this finding of a protective effect for both types of orofacial cleft. CONCLUSIONS The use of vitamin supplements in the first 4 months of pregnancy was suggestive of a protective effect against the occurrence of CP and CL(P) in this population. The significance of an association between multivitamin supplementation and OFC and the possible role of gene/environment interaction are discussed.

Rehabilitative treatment of cleft lip and palate: experience of the Hospital for Rehabilitation of Craniofacial Anomalies/USP (HRAC/USP) Part 4: Oral Rehabilitation

Treatment of patients with cleft lip and palate is completed with fixed prostheses, removable, total, implants and aims to restore aesthetics, phonetics and function and should be guided by the basic principles of oral rehabilitation, such as physiology, stability, aesthetics, hygiene and the expectations of the patient. In order to obtain longevity of a prosthetic rehabilitation, the periodontal and dental tissue as well as the biomechanics of the prosthesis are to be respected. The purpose of this article is to describe the types of prosthetics treatment, which are performed at HRAC/USP for the rehabilitation of cleft area in adult patients.

Rehabilitative treatment of cleft lip and palate: experience of the Hospital for Rehabilitation of Craniofacial Anomalies - USP (HRAC-USP) - Part 2: Pediatric Dentistry and Orthodontics

The aim of this article is to present the pediatric dentistry and orthodontic treatment protocol of rehabilitation of cleft lip and palate patients performed at the Hospital for Rehabilitation of Craniofacial Anomalies - University of São Paulo (HRAC-USP). Pediatric dentistry provides oral health information and should be able to follow the child with cleft lip and palate since the first months of life until establishment of the mixed dentition, craniofacial growth and dentition development. Orthodontic intervention starts in the mixed dentition, at 8-9 years of age, for preparing the maxillary arch for secondary bone graft procedure (SBGP). At this stage, rapid maxillary expansion is performed and a fixed palatal retainer is delivered before SBGP. When the permanent dentition is completed, comprehensive orthodontic treatment is initiated aiming tooth alignment and space closure. Maxillary permanent canines are commonly moved mesially in order to substitute absent maxillary lateral incisors. Patients with complete cleft lip and palate and poor midface growth will require orthognatic surgery for reaching adequate anteroposterior interarch relationship and good facial esthetics.

Prevalência de fissuras orais de 1975 a 1994

OBJECTIVE: To estimate the prevalence of oral clefts in Brazil categorized by etiology and geographical region. METHODS: Case reports of oral cleft in newborns in the period 1975¾1994 were included in the study. Data was collected using the morbidity certificates of the Hospital de Reabilitacao de Anomalias Craniofaciais (Craniofacial Abnormalities Rehabilitation Hospital), Ministry of Health data (Datasus), and Fundacao Instituto Brasileiro de Geografia e Estatistica (Brazilian Census Bureau). RESULTS/CONCLUSIONS: There were 16,853 new cases of oral clefts, with a total prevalence of 0.19 per 1,000 births, and there was an increased prevalence every five years. The highest prevalence were observed in the Midwest, southeast, and south regions. As for the studied categories, cleft lip (or the cleft of lip and palate) was seen in 74% of the cases and isolated cleft palate was seen in 26% of them.

Current data on the characterization of oral clefts in Brazil

This study aimed at investigating the current distribution of the several types of clefts among the patients receiving treatment at the Hospital for Rehabilitation of Craniofacial Anomalies (HRAC-USP), Bauru, Brazil, for the first time during the year 2000. A total of 803 unoperated patients with cleft lip and/or palate, with or without additional malformations, with no recognizable syndromes, who came to the HRAC-USP for enrollment for treatment during the year 2000. A predominance of complete cleft lip and palate, either unilateral or bilateral, was observed (37.1%), followed by isolated cleft palate (31.7%) and isolated cleft lip (28.4%). A discrete relationship between cleft palate and the female gender was noticed (53%), and males were more affected by the other types of clefts (around 60%). The findings revealed a predominance of complete clefts of the primary and secondary palate, the treatment of which is more complex, and whose frequency is greater in males.

Nasopharyngoscopy in Robin sequence: clinical and predictive value.

Objective To correlate nasopharyngoscopic findings with clinical manifestations during the first month of life and study the course of respiratory obstruction during the first year in infants with Robin sequence (RS). Design A longitudinal prospective study of children with RS. Setting Hospital de Reabilitação de Anomalias Craniofaciais, University of São Paulo, Bauru-SP, Brazil, 1998 to 2000. Patients Fifty-six children were studied from the age of 1 month to 12 months. Interventions The type of respiratory obstruction was defined by nasopharyngoscopy. Patients for whom glossoptosis was the only mechanism of respiratory obstruction were classified as having mild, moderate, or severe glossoptosis by nasopharyngoscopy and as mild, moderate, or severe cases with respect to the clinical manifestations. Results Forty-two (75%) patients showed respiratory obstruction caused by glossoptosis; seven (43.7%) of these infants with mild clinical manifestations showed moderate glossoptosis during the first month of life and five (31.3%) presented severe glossoptosis; 10 (45.5%) of the infants with severe clinical manifestations showed moderate and 11 (50.0%) severe glossoptosis. At 12 months of age, glossoptosis was mild or absent in 83.3% of the patients, moderate in 14.3% and severe in 2.4%. Conclusions A poor correlation between the severity of glossoptosis and the severity of clinical manifestations was observed for patients with respiratory obstruction caused by glossoptosis during the first month of life, but the correlation between glossoptosis and respiratory distress according to age was statistically significant. Nasopharyngoscopy is not a good method for predicting the severity of the clinical course of respiratory obstruction caused by glossoptosis.

Fissuras lábio-palatais: estudo caso-controle

This study relates to a case-control analysis for the purpose of verifying the association between oral clefts and possible risk factors. The analysed variables were: place of mothers residence (urban/rural), pollution, parental diseases, mothers diseases during the first four months of pregnancy, intake of drugs related to this period, heredity, smoking habits, alcohol consumption and X-ray examinations during pregnancy or X-ray examinations prior to pregnancy. There were 450 cases of clefts of whom 354 had a cleft lip with or without cleft palate and 96 had a cleft palate. The relative risk (RR) for each variable by was estimated points and at a 95% of confidence interval and multivariate analysis was applied. As regards cleft lip with or without cleft palate, the risk factors are heredity (RR = 4.96), epilepsy in the mother (RR = 2.39) and the intake of drugs such as anti-inflammatory substance in the first four months of pregnancy (RR = 2.59). Related to cleft palate, the risk factors are heredity (RR = 2.82) and pollution (RR = 2.58).Realizou-se um estudo caso-controle com o proposito de se detectar possiveis fatores de risco para o aparecimento de fissuras orais. Foram objeto de analise: local de moradia da mae nos quatro primeiros meses de gestacao, poluicao, aplicacao de pesticida/herbicida na lavoura, doencas dos pais, doencas da mae nos quatro primeiros meses de gestacao, ingestao medicamentosa nesse periodo, hereditariedade, tabagismo, consumo de bebida alcoolica e exposicao a raio-X. Foram aplicados formularios as maes referentes aos 450 casos, sendo 354 portadores de fissuras labiais ou labio-palatais e 96 de fissuras palatinas, e as maes referentes aos 450 controles. Empregou-se analise multivariada e as variaveis hereditariedade (RR=4,96), epilepsia na mae (RR=2,39) e ingestao de anti-inflamatorio (RR=2,59) foram consideradas fatores de risco para fissuras labiais ou labio-palatais. As variaveis hereditariedade (RR=2,82) e poluicao (RR=2,58) foram consideradas fatores de risco para fissuras palatinas.This study relates to a case-control analysis for the purpose of verifying the association between oral clefts and possible risk factors. The analysed variables were: place of mothers residence (urban/ rural), pollution, parental diseases, mothers diseases during the first four months of pregnancy, intake of drugs related to this period, heredity, smoking habits, alcohol consumption and X-ray examinations during pregnancy or X-ray examinations prior to preganacy. There were 450 cases of clefts of whom 354 had a cleft lip with or without cleft palate and 96 had a cleft palate. The relative risk (RR) for each variable by was estimated points and at a 95% of confidence interval and multivariate analysis was applied. As regards cleft lip with or without cleft palate, the risk factors are heredity (RR = 4.96), epilepsy in the mother (RR = 2.39) and the intake of drugs such as anti-inflammatory substance in the first four months of pregnancy (RR = 2.59). Related to cleft palate, the risk factors are heredity (RR = 2.82) and pollution (RR = 2.58).

A methodology report of a randomized prospective clinical trial to assess velopharyngeal function for speech following palatal surgery

Cleft lip and palate occurs in approximately 1 in every 750 live human births, making it one of the most common congenital malformations. Surgical closure of the palatal cleft does not always result in a velopharyngeal port capable of supporting normal speech. The University of Florida (UF), in collaboration with the University of São Paulo (USP), is engaging in a 5-year prospective, randomized controlled study to compare velopharyngeal function for speech outcomes between patients undergoing palatoplasty for complete unilateral cleft lip and palate performed using the von Langenbeck procedure with intravelar velarplasty and those receiving the Furlow double-reversing Z-plasty palatoplasty. The von Langenbeck procedure was selected as the time-tested standard against which the Furlow procedure could be judged. The Furlow procedure, a relatively new operation, has been reported to yield substantially higher rates of velopharyngeal competency for speech than have most other reported series and theoretically should result in less disturbance to midfacial growth. A total of 608 patients will be entered into one of two age categories. Inclusion of two age groups will allow a comparison of results between patients having surgery before 1 year of age (9-12 months) and patients undergoing surgery at approximately 1.5 years of age (15-18 months). Speech data will be collected and will be available for definitive analysis throughout the last 3 years of the study. Collection of preliminary growth data will require more than 5 years; growth analysis is anticipated to continue until all patients have reached maturity. The Hospital for Research and Rehabilitation of Patients with Cleft Lip and Palate at the University of São Paulo (USP-HPRLLP) in Bauru, Brazil, is uniquely situated for conducting this study. The well-equipped and modern facilities are staffed by well-trained specialists representing all disciplines in cleft-palate management. In addition, an already existing social services network throughout Brazil will ensure excellent follow-up of study cases. The clinical caseload at this institution currently exceeds 22,000, and more than 1200 new cases are added annually. This project represents a unique opportunity to obtain prospective data from a large number of subjects while controlling the variables that have traditionally plagued cleft-palate studies. This study is designed to determine which of the two proposed surgical procedures is superior in constructing a velum capable of affecting velopharyngeal competency for the development of normal speech.

Speech outcomes and velopharyngeal function after surgical treatment of velopharyngeal insufficiency in individuals with signs of velocardiofacial syndrome.

Objective: The objective of the study was to analyze if individuals with velocardiofacial syndrome (VCFS) present the same characteristics of speech and velopharyngeal function (VPF) compared with patients with nonsyndromic submucous cleft palate, as well as to compare the effectiveness of palate surgery on the speech function and VPF between groups. Methods: This was a prospective study performed at the Speech Therapy Sector and Physiology Laboratory, Hospital for Rehabilitation of Craniofacial Anomalies/University of São Paulo. The procedure performed was primary palatoplasty associated or not to superiorly based pharyngeal flap surgery. There were 50 patients with velopharyngeal insufficiency: 25 with signals of VCFS (VCFS group) and 25 without syndrome with submucous cleft palate (SMCP group). The hypernasality was scored by 3 examiners; nasalance was evaluated by nasometry, and VPF was assessed by the size of the velopharyngeal gap on the nasoendoscopy. The evaluations were conducted before and, in average, 18 months after surgery. Results: Before surgery, the VCFS and SMCP groups presented similar speech function and VPF characteristics in all parameters, with no statistically significant differences. After surgery, there was reduction in the hypernasality, nasalance, and VPF in, respectively, 20%, 31%, and 36% of patients in the VCFS group and in 24%, 30%, and 30% in the SMCP group. Elimination/normalization of variables was obtained in 28%, 19%, and 8% of patients in the VCFS group and 20%, 40%, and 25% in the SMCP group, respectively, for hypernasality, nasalance, and VPF. There was no statistically significant difference between groups. Conclusions: Patients with VCFS presented similar speech function and VPF characteristics as patients with nonsyndromic SMCP. The surgery for velopharyngeal insufficiency correction was equally effective for the improvement and resolution of speech symptoms and VPF in patients with VCFS compared with the SMCP group.

Rehabilitative treatment of cleft lip and palate: experience of the Hospital for Rehabilitation of Craniofacial Anomalies/USP (HRAC/USP) - Part 5: Institutional outcomes assessment and the role of the Laboratory of Physiology

The Laboratory of Physiology provides support for the diagnosis of functional disorders associated with cleft lip and palate and also conducts studies to assess, objectively, the institutional outcomes, as recommended by the World Health Organization. The Laboratory is conceptually divided into three units, namely the Unit for Upper Airway Studies, Unit for Stomatognathic System Studies and the Unit for Sleep Studies, which aims at analyzing the impact of different surgical and dental procedures on the upper airways, stomatognathic system and the quality of sleep of individuals with cleft lip and palate. This paper describes the main goals of the Laboratory in the assessment of procedures which constitute the basis of the rehabilitation of cleft lip and palate, i.e., Plastic Surgery, Orthodontics and Maxillofacial Surgery and Speech Pathology.