Robin Sequence Facial Profile After Conservative Treatment: A Long-Term Follow-Up.

INTRODUCTION: Robin sequence (RS) is a congenital clinical condition characterized by micrognathia, glossoptosis, and respiratory distress. Conservative methods could be responsible for releasing feeding and respiratory impairment but little information about mandibular growth is known in long-term follow-up. OBJECTIVE: Assessing the longitudinal behavior of the facial profile of individuals with isolated RS who underwent conservative micrognathia treatment using photographs during the whole craniofacial growth. METHODS: Photographs of the right facial profile of 100 patients were used (50 individuals with isolated RS and 50 individuals without craniofacial anomaly). The individuals with RS were evaluated at 3 different times (T1: infant, T2: mixed dentition, T3: permanent dentition) by measuring the facial convexity angle (FCA; G.Sn.Pog´). A comparison between T3 and control group (C), individuals without craniofacial anomalies and in permanent dentition, was also performed, checking the FCA, nasolabial angle (Ls.Sn.Cm), mentolabial fold (Li.Si.Pog´), facial inferior third (Sn.Gn´.C) angles and the ratio between middle anterior facial height and lower anterior facial height. RESULTS: The T3 group showed an increased angle of facial convexity and increased facial inferior third angle and middle anterior facial height/lower anterior facial height ratio compared with the control group. In the longitudinal evaluation of individuals with isolated RS, significant differences were identified between T1 and T2 groups and T1 and T3 groups showing that the increased facial convexity was higher in the infants and that did not change significantly between the phases of mixed and permanent dentition. CONCLUSIONS: RS showed increased facial convexity in all phases evaluated, but their convexity decreased with growth. When compared with individuals without craniofacial anomalies, the individuals continue to exhibit retrognathism in the permanent dentition. The lack of a mandible projection has led to a considerable number of orthognathic surgeries for the correction of discrepancies.

Child feeding after palatoplasty: role of nursing staff in the training and evaluation of caregivers / Alimentação da criança após palatoplastia: o papel da equipe de enfermagem na formação e avaliação dos cuidadores

Objective ­ To promote and evaluate the ability of caregivers in child feeding during the postoperative period after palatoplasty. Methods ­ The sample comprised 44 caregivers of children with cleft lip and palate, trained during hospitalization and evaluated on hospital discharge concerning the care related to child feeding after palatoplasty. The study was conducted in 2010, using a constructed and validated instrument. The ability of caregivers was evaluated using a quantitative instrument and associated with socioeconomic factors. The Fisher exact test was used to analyze the significance of differences between paired samples. Results ­ Among the caregivers, 86% were able for feeding after palatoplasty, and the socioeconomic factors and type of cleft did not influence the acquisition of abilities. Conclusions ­ Most caregivers were able for the care; the socioeconomic factors analyzed did not influence the ability for self-care; and 24 hours of hospitalization were sufficient for achievement of abilities related to care.

Objetivo ­ Promover e avaliar a capacidade dos cuidadores na alimentação da criança durante o período pós-operatório após palatoplastia. Métodos ­ A amostra foi composta por 44 cuidadores de crianças com fissura labiopalatina, treinados e avaliados durante a internação na alta hospitalar sobre os cuidados relacionados com a alimentação da criança após palatoplastia. O estudo foi realizado em 2010, utilizando um instrumento construído e validado. A capacidade dos cuidadores foi avaliada utilizando um instrumento quantitativo e associado aos fatores socioeconômicos. O teste exato de Fisher foi utilizado para analisar a significância das diferenças entre amostras pareadas. Resultados ­ Entre os cuidadores, 86% foram capazes de alimentação após a palatoplastia, e os fatores socioeconômicos e tipo de fissura não influenciou na aquisição de habilidades. Conclusões ­ A maioria dos cuidadores eram capazes para o atendimento; os fatores socioeconômicos analisados não influenciaram a capacidade de auto-atendimento; e 24 horas de internação foram suficientes para a realização de habilidades relacionadas ao cuidado.

Neurological Development of Children With Isolated Robin Sequence Treated With Nasopharyngeal Intubation in Early Infancy.

The study assessed the neurodevelopment of children with isolated Robin sequence (IRS) and evaluated if children treated exclusively with nasopharyngeal intubation (NPI) present delay in neurological development. The prospective and cross-sectional study was conducted at the Hospital for Rehabilitation of Craniofacial Anomalies, Brazil. Children with IRS were divided into two groups according to the type of treatment in early infancy: 38 were treated with NPI (more severe cases) and 24 with postural treatment (less severe cases). Regarding interventions, children were assessed at 2 to 6 years of age using the Denver II Developmental Screening Test (Denver II) and Neurological Evolutionary Examination (NEE). According to Denver II, 73.7% in the NPI group and 79.2% in the postural group presented normal development. This result was similar to the results of different studies in the literature with typical population. Considering all areas of development, there were no significant differences in Denver II between the NPI and postural groups (P = .854). In the NPI group, 89.5% of children and 87.5% in the postural group presented normal development in NEE. Language was the most affected area, as 18.4% and 20.8% of children in NPI and postural group, respectively, presented risk for delay in the Denver II. The increased risk for delay in language area was probably due to anatomical conditions of the muscles involved in speech, and to hearing oscillations, as 47.4% in NPI group and 58.3% in postural group underwent myringotomy. IRS treated with NPI had neurological development similar to those in less severe cases. Children treated exclusively with NPI did not present delay in neurological development.

Evaluation of Delayed Puberty in Adolescents With Cleft Lip/Palate.

OBJECTIVE: To assess the frequency of delayed puberty in adolescents with cleft lip and/or cleft palate (CL/P). DESIGN: This was a cross-sectional study of 203 patients with CL/P and no associated syndromes treated at the Hospital for Rehabilitation of Craniofacial Anomalies, University of São Paulo, Bauru, Brazil. We evaluated boys aged 14-19 years and girls aged 13-18 years. The patients were classified according to Tanner stages of sexual development. The age of menarche was recorded. Patients were assigned to three groups according to cleft type: isolated cleft lip (CL), cleft lip and palate (CLP), and isolated cleft palate (CP). The results were expressed as frequencies and averages and compared with pubertal changes described for typically developing adolescents as reported in the literature. RESULTS: Subjects were 115 boys and 88 girls. All boys in the CL group and the CP group had already started puberty, and two boys in the CLP group (2.3%) had delayed puberty. All girls had started puberty. The average age at menarche was 12.3 years in the CL group, 12.1 years in the CLP group, and 12.5 years in the CP group. CONCLUSIONS: The frequency of delayed puberty and the average age at menarche in adolescents with CL/P and no associated genetic syndromes or anomalies were within the expected range for typically developing adolescents (i.e., those without CL/P) in the same age group.

Weight, Length, and Body Mass Index Growth of Children Under 2 Years of Age With Cleft Lip and Palate.

OBJECTIVE: To study the growth of length-for-age (L/A), weight-for-age (W/A), and body mass index (BMI) of children with cleft lip and palate receiving a normal diet; to establish specific growth curves for children with cleft palate with or without cleft lip (CLP/ICP) who had not undergone palatoplasty and for children with isolated cleft lip (ICL); and to assess if CLP/ICP growth differed from ICL growth and if CLP/ICP and ICL growth differed from growth for typical children. DESIGN: Prospective and cross-sectional study. SETTING: Hospital for Rehabilitation of Craniofacial Anomalies, Bauru, São Paulo, Brazil. MAIN OUTCOME MEASURES: Weight and length of 381 children with cleft lip and palate and who were younger than 2 years were recorded and used to calculate W/A, L/A, and BMI growth curves. The 2006 World Health Organization growth charts were used as a reference for typical children. All children received a normal diet for age. RESULTS: Children with CLP/ICP had median W/A and BMI growth curves below growth curves for typical children but showed spontaneous recovery starting at approximately 5 months of age, even with nonoperated cleft palate. Children with ICL had growth similar to that of typical children. Children with CLP/ICP, who initially had W/A and BMI values less than those of the ICL group, had W/A and BMI equal to or higher than the ICL group after 9 months of age. CONCLUSION: Children with CLP/ICP had impaired W/A and BMI growth with spontaneous recovery starting early in childhood. This study established specific W/A, BMI, and L/A growth curves for children with cleft lip and palate.

Assessment of speech nasality in children with Robin Sequence.

PURPOSE: To report the outcomes of primary palatoplasty in Robin Sequence (RS); to verify the relationship between modalities of assessment of nasality; to compare nasality between techniques at palatoplasty. METHODS: This study involved the identification of hypernasality in four modalities: live assessment with 4-point scale; live assessment with cul-de-sac test; multiple listeners' ratings of recorded phrase; nasometric assessment. Live ratings of speech nasality and nasalance scores were retrieved from charts, while a recorded phrase was rated by listeners for occurrence of hypernasality. Agreement between the modalities was established as well as association between nasality, nasal turbulence and age at surgery and at assessment. Fisher's exact test was used to compare findings between surgical techniques. RESULTS: Agreement between nasalance, live assessment with 4-point scale, live assessment with cul-de-sac, and multiple listeners' ratings of recorded samples ranged between reasonable (0.32) and perfect (1.00). Percentage occurrence of hypernasality varied largely between assessment modalities. Mean occurrence of hypernasality was lower for the group submitted to Furlow technique (26%) than the group that received von Langenbeck technique (53%). Only findings obtained live were statistically significant (scale: p=0.012; cul-de-sac: p<0.001). Listeners identified nasal turbulence for 22 (32%) samples out of the 69 recordings, and an association was found between hypernasality and nasal turbulence. CONCLUSION: Lower occurrence of hypernasality was identified for patients with RS in Furlow group. Identification of hypernasality varied largely among the four assessment modalities.

Assessment of speech nasality in children with Robin Sequence / Avaliação da nasalidade de fala em crianças com Sequência de Robin

PURPOSE: To report the outcomes of primary palatoplasty in Robin Sequence (RS); to verify the relationship between modalities of assessment of nasality; to compare nasality between techniques at palatoplasty. METHODS: This study involved the identification of hypernasality in four modalities: live assessment with 4-point scale; live assessment with cul-de-sac test; multiple listeners' ratings of recorded phrase; nasometric assessment. Live ratings of speech nasality and nasalance scores were retrieved from charts, while a recorded phrase was rated by listeners for occurrence of hypernasality. Agreement between the modalities was established as well as association between nasality, nasal turbulence and age at surgery and at assessment. Fisher's exact test was used to compare findings between surgical techniques. RESULTS: Agreement between nasalance, live assessment with 4-point scale, live assessment with cul-de-sac, and multiple listeners' ratings of recorded samples ranged between reasonable (0.32) and perfect (1.00). Percentage occurrence of hypernasality varied largely between assessment modalities. Mean occurrence of hypernasality was lower for the group submitted to Furlow technique (26%) than the group that received von Langenbeck technique (53%). Only findings obtained live were statistically significant (scale: p=0.012; cul-de-sac: p<0.001). Listeners identified nasal turbulence for 22 (32%) samples out of the 69 recordings, and an association was found between hypernasality and nasal turbulence. CONCLUSION: Lower occurrence of hypernasality was identified for patients with RS in Furlow group. Identification of hypernasality varied largely among the four assessment modalities. .

OBJETIVO: Reportar os resultados da palatoplastia primária na Sequência de Robin (SR); verificar a relação entre modalidades de avaliação da nasalidade; comparar nasalidade entre técnicas na palatoplastia. MÉTODOS: Este estudo envolveu a identificação da hipernasalidade em quatro modalidades: avaliação ao vivo com escala de quatro pontos; avaliação ao vivo com teste cul-de-sac; julgamento de gravações por juízes e avaliação nasométrica. Julgamentos ao vivo da nasalidade e escores de nasalância foram obtidos em prontuários, enquanto uma frase gravada foi julgada por juízes para ocorrência de nasalidade. Concordância entre as quatro modalidades foi estabelecida assim como associação entre nasalidade, turbulência nasal e idades na cirurgia e na avaliação. Teste exato de Fisher foi usado para comparar achados entre as técnicas cirúrgicas. RESULTADOS: A concordância entre nasalância, avaliação ao vivo com escala de quatro pontos e com teste cul-de-sac e julgamentos de gravações por juízes variou entre razoável (0,32) e perfeita (1,00). Porcentagem de ocorrência de hipernasalidade variou muito entre as diferentes modalidades. Ocorrência média de hipernsalidade no grupo operado com técnica de Furlow foi menor (26%) do que no grupo que recebeu a técnica de von Langenbeck (53%). Somente os resultados avaliados ao vivo foram estatisticamente significantes (escala: p=0,012; cul-de-sac: p<0,001). Juízes ouviram turbulência nasal em 22 (32%) das 69 gravações e uma associação entre hipernasalidade e turbulência nasal foi encontrada. CONCLUSÃO: Ocorrência de hipernasalidade foi menor para os pacientes com SR que receberam a técnica de Furlow. Identificação da hipernasalidade variou grandemente entre as quatro modalidades de avaliação. .

Longitudinal study of growth of children with unilateral cleft lip and palate: 2 to 10 years of age.

Objective : To study the growth of children with complete unilateral cleft lip and palate (UCLP) from 2 to 10 years of age and to assess whether growth varied from that of children without UCLP (typical children). Design : Physical growth was one of the outcome measures of a National Institutes of Health-sponsored longitudinal, prospective clinical trial conducted by the University of Florida and the University of São Paulo. Setting : Hospital of Rehabilitation of Craniofacial Anomalies, University of São Paulo (HRAC-USP), Bauru, Brazil. Main Outcome Measures : Height and weight were prospectively measured for 360 healthy children with UCLP who were nonsyndromic, belonged to median socioeconomic status, and received health care at HRAC-USP. To compare growth of children with UCLP to that of typical children, growth curves for UCLP were developed and compared with World Health Organization curves for 2006 and 2007, which were used as reference for typical children. Third-degree polynomials were used to explain the relationship of length and weight with age. Confidence limits of 95% were used for the mean curve using the statistic Z ~ N (0,1). Results : Children with UCLP from 2 to 10 years old presented height and weight growth curves similar to those of typical children for both genders. Conclusion : Children with UCLP from 2 to 10 years old presented physical growth similar to that of typical children.

Alimentação da criança no pós-operatório imediato de palatoplastia: comparação entre as técnicas utilizando copo e colher / Alimentacion del nino en el post operatorio inmediato de palatoplastia: una comparacion entre las tecnicas utilizando vaso y cuchara / Feeding children in the immediate perioperative period after palatoplasty: a comparison between techniques using a cup and a spoon

Estudo prospectivo realizado no Hospital de Reabilitação de Anomalias Craniofaciais, com o objetivo de analisar comparativamente a melhor técnica para alimentar a criança no pós-operatório imediato de palatoplastia: utilizando copo ou colher. Foram acompanhados 44 crianças e seus cuidadores durante a alimentação, em 4 horários consecutivos, gerando 176 avaliações, 88 utilizando copo e 88 a colher. Os testes Exato de Fisher e de Mann-Whitney foram usados para análise estatística, com nível de significância de 5% (p<0,05). O escape de alimento pela comissura labial foi menor (p=0,024; 17%), o volume administrado foi maior (p=0,029; 12%) e a tosse foi menos frequente (p=0,026; 13%) com a técnica que utiliza a colher. Conclui-se que a técnica de administração que utiliza colher para a alimentação pós-palatoplastia é melhor que a que utiliza o copo.

Estudio prospectivo desarrollado en el Hospital de Rehabilitación de Anomalías Cráneo-faciales, con el objetivo de evaluar comparativamente la mejor técnica para alimentar al niño en el post-operatorio inmediato de palatoplastía, utilizando un vaso y una cuchara. Se realizó el seguimiento a 44 niños y sus cuidadores durante la alimentación, en cuatro horarios consecutivos, lo que generó 176 evaluaciones, 88 utilizando un vaso y 88 una cuchara. Para el análisis estadístico, se utilizaron las pruebas Exacta de Fisher y de Mann-Whitney, con un nivel de significancia del 5% (p<0,05). El escape de alimento por la comisura labial fue menor (p=0,024; 17%), el volumen administrado fue mayor (p=0,029; 12%) y la tos fue menos frecuente (p=0,026; 13%) con la técnica que utiliza la cuchara. Se concluye que la técnica de alimentación post-palatoplastía con cuchara es mejor que la que utiliza el vaso.

This prospective study conducted at the Hospital for the Rehabilitation of Craniofacial Anomalies aimed to compare the best technique – a cup or a spoon – for feeding children immediately after palatoplasty. We assessed 44 children and their caregivers during feeding every 4 hours; this generated 176 evaluations: 88 using a cup and 88 using a spoon. The Fisher exact test and the Mann-Whitney test were used for statistical analysis, with a significance level of 5% (p<0.05). When the spoon was used, the percentage of patients with food escaping through the labial commissure was lower (17%; p=0.024, the portion administrated was higher (12%; p=0.029), and coughing was less frequent (13%; p=0.026) compared with use of a cup. We conclude that using a spoon to administer food after palatoplasty is better than using a cup.

[Feeding children in the immediate perioperative period after palatoplasty: a comparison between techniques using a cup and a spoon]. / Alimentação da criança no pós-operatório imediato de palatoplastia: comparação entre as técnicas utilizando copo e colher.

This prospective study conducted at the Hospital for the Rehabilitation of Craniofacial Anomalies aimed to compare the best technique - a cup or a spoon - for feeding children immediately after palatoplasty. We assessed 44 children and their caregivers during feeding every 4 hours; this generated 176 evaluations: 88 using a cup and 88 using a spoon. The Fisher exact test and the Mann-Whitney test were used for statistical analysis, with a significance level of 5% (p<0.05). When the spoon was used, the percentage of patients with food escaping through the labial commissure was lower (17%; p=0.024, the portion administrated was higher (12%; p=0.029), and coughing was less frequent (13%; p=0.026) compared with use of a cup. We conclude that using a spoon to administer food after palatoplasty is better than using a cup.

Clinical and fiberoptic endoscopic evaluation of swallowing in Robin sequence treated with nasopharyngeal intubation: the importance of feeding facilitating techniques.

OBJECTIVE: To evaluate oral feeding capacity, the swallowing process, and risk for aspiration, both clinically and during fiberoptic endoscopic evaluation of swallowing, in infants with isolated Robin sequence treated exclusively with nasopharyngeal intubation and feeding facilitating techniques. DESIGN: Longitudinal and prospective study. SETTING: Hospital de Reabilitação de Anomalias Craniofaciais, University of São Paulo, Bauru, Brazil. PATIENTS: Eleven infants with isolated Robin sequence, under 2 months of age, treated with nasopharyngeal intubation. INTERVENTIONS: Feeding facilitating techniques were applied in all infants throughout the study period. The infants were evaluated clinically and through fiberoptic endoscopic evaluation of swallowing at first, second, and, if necessary, third week of hospitalization (T1, T2, T3). The mean volume of ingested milk was registered during clinical evaluation, and events were registered during feeding. RESULTS: The respiratory status of all infants was improved after nasopharyngeal intubation; 72% of them presented risk for aspiration during fiberoptic endoscopic evaluation of swallowing at T1. This risk was less frequent when thickened milk was given to the infants and at subsequent evaluations (T2 and T3). CONCLUSIONS: Nasopharyngeal intubation aids in stabilizing the airway in isolated Robin sequence, but it does not relate directly to feeding. The risk for aspiration was present in most of the infants, mainly during the first week of hospitalization, and improved within a few weeks, after the use of feeding facilitating techniques.

Gastroesophageal reflux in severe cases of Robin sequence treated with nasopharyngeal intubation.

OBJECTIVE: To study the prevalence of abnormal gastroesophageal reflux in infants with Robin sequence who had severe respiratory obstruction treated with nasopharyngeal intubation and to evaluate the efficacy of nonsurgical treatment. DESIGN: Longitudinal prospective study. SETTING: Hospital de Reabilitação de Anomalias Craniofaciais, University of São Paulo, Brazil. PATIENTS: Twenty infants with severe isolated Robin sequence treated with nasopharyngeal intubation. INTERVENTIONS: We performed 24-hour esophageal pH monitoring on each child at 2, 4, and 6 months of age. Respiratory and feeding status were evaluated. We considered abnormal gastroesophageal reflux as reflux index values above the 95th percentile of the Vandenplas reference for normal children. RESULTS: The prevalence of reflux index above the 95th percentile at the first exam was 6/20, a value significantly higher than the reference (5/103, p < .01). At the second and third exams, reflux index values were decreased. Ninety percent of the infants showed improvement of respiratory difficulty and developed oral feeding capacity. CONCLUSIONS: The prevalence of abnormal gastroesophageal reflux is higher in infants with severe cases of Robin sequence than in normal infants. Nonsurgical procedures improved respiratory and feeding difficulties of most of these infants.

Growth impairment of children with different types of lip and palate clefts in the first 2 years of life: a cross-sectional study.

OBJECTIVE: To analyze the differences in growth impairment according to sex in the 2 first years of life in children with three types of clefts. METHODS: This was a cross-sectional study of 881 children (58.9% boys and 41.1% girls) with cleft lip and palate treated at the Craniofacial Anomaly Rehabilitation Hospital, (University of São Paulo, Bauru, SP), Brazil. Age ranged from 1 to 24 months. Three types of clefts were evaluated: isolated cleft lip (181/20.5%), isolated cleft palate (157/17.8%) and cleft lip + palate (543/61.6%). Weight and length measurements and data regarding breast-feeding and socioeconomic level were obtained. Children with weight and length below the 10th percentile of the NCHS reference were considered to have impaired growth. RESULTS: Sample distribution according to cleft type and sex was similar to that observed in other epidemiological studies. Breast-feeding was more frequent in the isolated cleft lip group (45.9%) then in the isolated cleft palate (12.1%) or cleft lip + palate group (10.5%). Isolated cleft lip children showed less marked impairment of weight (23.8%) and length (19.3%) compared to the cleft lip + palate group (35.7% and 33.1%, respectively). In the latter group, the proportion of children with weight and length below the 10th percentile was very close to that of the isolated cleft palate group (34.4% and 38.9%). CONCLUSIONS: The impairment in weight and length was more severe in cleft lip + palate and isolated cleft palate children and may be attributed to feeding difficulties compared to the isolated cleft lip group.

Prejuízo no crescimento de crianças com diferentes tipos de fissura lábio-palatina nos 2 primeiros anos de idade: um estudo transversal / Growth impairment of children with different types of lip and palate clefts in the first 2 years of life: a cross-sectional study

OBJETIVO: Analisar as diferenças da restrição de crescimento até o segundo ano de vida, com base no gênero, entre crianças com três tipos de fissuras. MÉTODOS: Estudo transversal com 881 crianças (58,9 por cento meninos e 41,1 por cento meninas) com fissura labial e palatina do Hospital de Reabilitação de Anomalias Craniofaciais, Universidade de São Paulo, Bauru (SP), Brasil, com idades entre 1 a 24 meses. Foram avaliados três tipos de fissuras: fissura labial (181/20,5 por cento), fissura palatina (157/17,8 por cento) e fissura labial + palatina (543/61,6 por cento). Foram obtidas as medidas de peso e comprimento e os dados acerca do aleitamento materno e do nível socioeconômico. Crianças com peso e comprimento abaixo do percentil 10 da referência NCHS foram consideradas como tendo restrição do crescimento. RESULTADOS: A distribuição da amostra de acordo com o tipo de fissura e gênero foi semelhante àquela observada em outros estudos epidemiológicos. O aleitamento materno foi mais freqüente no grupo com fissura labial (45,9 por cento) que nos grupos de fissura palatina (12,1 por cento) ou de fissura labial + palatina (10,5 por cento). Os lactentes com fissura labial mostraram menos comprometimento do peso (23,8 por cento) e do comprimento (19,3 por cento) comparados àqueles do grupo com fissura labial + palatina (35,7 por cento e 33,1 por cento, respectivamente), sendo que o último grupo mostrou proporções de crianças com peso e comprimento inferiores ao percentil 10 muito próximas às do grupo com fissura palatina (34,4 por cento e 38,9 por cento). CONCLUSÕES: O comprometimento do peso e comprimento é mais grave nos lactentes com fissura labial + palatina e com fissura palatina e pode ser atribuído principalmente às dificuldades de alimentação, em comparação ao grupo com fissura labial.

Growth of children with isolated Robin sequence treated by nasopharyngeal intubation: importance of a hypercaloric diet.

OBJECTIVE: To compare the growth curves (weight and length) of two groups of infants with isolated Robin sequence (RS) treated with nasopharyngeal intubation (NPI), one group receiving a hypercaloric diet and the other receiving a normal diet for age, and compare the growth rates and duration of NPI between the two groups. DESIGN: Prospective longitudinal study. SETTING: Hospital de Reabilitação de Anomalias Craniofaciais, Universidade de São Paulo, Bauru, Brazil, 1998 to 2000. PATIENTS: Twenty-three children with isolated RS treated by prolonged NPI. INTERVENTIONS: Nine children received a milk formula supplemented with 5% to 7% glucose polymers and 3% to 5% medium chain triglycerides (group 1), and 14 children received a milk formula only (group 2). Weight and length were measured at birth and every month up to 6 months of life for the construction of weight and length growth curves. The time of use of the nasogastric tube, age at the beginning of NPI, and duration of NPI were also determined. RESULTS: No significant difference in length was observed between the two groups. The mean weight curves obtained for the two groups were below the 50th percentile of the National Center for Health Statistics, but weight gain was higher in group 1. The duration of NPI was shorter in this group. CONCLUSIONS: The hypercaloric diet led to an improvement in nutritional status, which in turn contributed to improved respiratory conditions in isolated RS.

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.

Importancia da intervençäo dietética no estado nutricional de lactentes portadores de Seqüencia de Robin / The importance of a dietetic intervention on the nutrition of infants with Robin Sequence

A proposta do presente estudo foi avaliar a influência da intervençäo dietética no estado nutricional dos pacientes com Seqüência de Robin e propor um regime dietético que considerasse suas dificuldades e necessidades nutricionais. Um estudo prospectivo e longitudinal foi realizado com 34 lactentes, avaliados dietética, antropométrica e laboratorialmente, em três diferentes momentos. A dieta hipercalórica foi introduzida na avaliaçäo inicial (A1). NAs avaliaçöes intermediária (A2) e final (A3), 91,2 porcento dos infantes eram alimentados exclusivamente por via oral e o volume médio ingerido durante todo o estudo esteve abaixo dos padronizados para a idade. Em A2 e A3, 85 porcento dos pacientes apresentaram incremento pôndero-estatural percentil maior ou igual a 50. Foram observadas adequaçöes crescentes na massa protéico-somática e reservas adiposas. Näo foram encontradas alteraçöes sangüíneas do colesterol, triglicérides, glicemia e estoques protéico-viscerais. A suplementaçäo dietética foi responsável pela recuperaçäo e manutençäo do estado nutricional dos pacientes com Sequência de Robin, mesmo nos casos mais graves.(au)