RESUMO A síndrome Shprintzen-Goldberg é uma entidade muito rara descrita em 75 pessoas, caracterizada por craniossinostose e habitus marfanóide. O objetivo deste estudo é apresentar o relato de um caso clínico de uma criança brasileira de 6 anos com síndrome da craniossinostose de Shprintzen-Goldberg, apresentando as características craniofaciais e orais e seu manejo clínico, comparando com a literatura. O paciente apresentava deficiência intelectual, craniossinostose, proptose ocular, orelhas anômalas de implantação baixa e outros defeitos esqueléticos e do tecido conjuntivo. As características orais incluíram má oclusão, micrognatia, pseudo fenda palatina, atividade de cárie e controle ineficiente do biofilme. O tratamento foi iniciado com orientações sobre alimentos cariogênicos, higiene oral e indicação de suspensão do uso de mamadeira. Em seguida, as lesões cariosas foram restauradas com resina composta e o paciente encaminhado para cirurgia ortopédica, tratamento ortodôntico, fonoaudiologia e nutricionista. O paciente foi acompanhado periodicamente por 5 anos. Este relato de caso enfatiza a importância do conhecimento das características craniofaciais e orais para o diagnóstico e manejo clínico de uma criança do sexo feminino com síndrome de craniossinostose de Shprintzen-Goldberg rara, destacando a necessidade de cuidados com a saúde bucal em indivíduos com deficiência intelectual.

ABSTRACT Shprintzen-Goldberg craniosynostosis syndrome, characterized by craniosynostosis and marfanoid habitus, is a very rare entity described in 75 individuals worldwide. This study aimed to present a case report of a 6-year-old female Brazilian child with Shprintzen-Goldberg’s craniosynostosis syndrome. We described the craniofacial and oral characteristics and its clinical management, comparing to the existing literature. The patient presented with intellectual disability, craniosynostosis, ocular proptosis, low-set anomalous ears, and other skeletal and connective tissue defects. Oral features included malocclusion, micrognathia, pseudo-cleft palate, dental caries, and inefficient biofilm control. The treatment started with guidance on cariogenic foods, oral hygiene, and an indication to discontinue bottle feeding. Subsequently, the carious lesions were restored with composite resins, and the patient was referred for orthopedic surgery, orthodontic treatment, speech therapy, and nutritional counseling. The patient was followed for 5 years. This case report emphasizes the importance of knowing the craniofacial and oral characteristics for the diagnosis and clinical management of a female child with a rare Shprintzen-Goldberg’s craniosynostosis syndrome. The case also highlights the need for oral health care in individuals with intellectual disabilities.

Resumo Introdução Lesões dentárias por trauma constituem experiências angustiantes em crianças, que promovem alterações tanto físicas quanto emocionais e psicológicas. Objetivo Avaliar a prevalência de traumatismos dentários em pacientes com distúrbio neuropsicomotor e comparar a indivíduos normorreativos. Material e método Avaliaram-se 120 indivíduos, sendo 60 com alterações neuropsicomotoras (grupo de estudo) e 60 normorreativos (grupo controle), de ambos os sexos, de 2 a 15 anos de idade, assistidos na Fundação de Apoio ao Deficiente do Governo do Estado da Paraíba. Os dados foram coletados por meio de questionário estruturado e exame clínico. Foi realizada análise descritiva e inferencial (teste t-student; teste Exato de Fisher), adotando-se um nível de significância de 5%. Resultado A prevalência de traumatismos dentários observada em pacientes com distúrbio neuropsicomotor foi de 20,0%, enquanto que no grupo controle foi de 16,6% (p>0,05); no grupo controle a ocorrência foi maior no sexo masculino. Os grupos diferiram quanto ao tipo de atividade no momento do trauma (p<0,05) em relação à etiologia (p<0,05) e em relação ao local de ocorrência (p<0,05). Para ambos os grupos, os dentes mais afetados foram os incisivos centrais superiores. As fraturas de esmalte, seguidas pelas de esmalte e dentina sem exposição pulpar foram as lesões mais comuns nos dois grupos. Conclusão A prevalência de traumatismos dentários em indivíduos com alteração neuropsicomotora é similar à de indivíduos normorreativos, com maior ocorrência no sexo feminino, em fase anterior à adolescência, durante atividades de rotina.

Abstract Introduction Traumatic dental injuries are distressing experiences in children, which promote both physical, emotional and psychological changes. Objective To evaluate the prevalence of dental trauma in patients with neuropsychomotor disorder and to compare to normoreactive individuals. Material and method 120 individuals, 60 neuropsychomotor changes (study group) and 60 normorreatives (control group), of both sexes, from 2 to 15 years old, assisted in the Foundation of Support to the Disabled of the Government of the State of Paraíba. Data were collected through a structured questionnaire and clinical examination. Descriptive and inferential analysis (t-student test; Fisher's exact test) was performed, adopting a significance level of 5%. Result The prevalence of dental trauma observed in patients with neuropsychomotor disorder was 20.0%, whereas in the control group it was 16.6% (p>0.05); in the control group the occurrence was higher in males. The groups differed according to the type of activity at the moment of the trauma (p<0.05) in relation to the etiology (p<0.05), and in relation to the place of occurrence (p<0.05). For both groups, the most affected teeth were the maxillary central incisors. Enamel fractures, followed by enamel and dentin fractures without pulp exposure were the most common lesions in both groups. Conclusion The prevalence of dental trauma in individuals with neuropsychomotor alterations is similar to that of normorreative individuals, with a higher occurrence in females, in preteen phase, during routine activities.

AIM: To evaluate the influence of the classification of cerebral palsy CP on the caries experience in children and adolescents, and determine the types of teeth most affected by dental caries. METHODS: A total of 181 individuals with CP, aged 8.30±4.10 years were examined for dental caries diagnosis in primary, mixed and permanent dentitions. The sample consisted of 96 quadriplegic individuals, 56 diplegic, 18 hemiplegic and 11 with choreoathetosis. The groups were compared using Kruskal-Wallis and Chi-square tests. Univariate logistic regression models were used to identify whether age was a predictor of caries risk α=5%. RESULTS: The sample was homogeneous as regards sex and age, among patients with quadriplegia, diplegia, hemiplegia and choreoathetosis. Out of the total sample, 51.4% of the individuals were caries free. The overall mean for the dmft and DMFT indices were 1.70±3.30 and 0.70±1.60, respectively. The mean caries experience in primary, mixed and permanent dentitions did not vary significantly according to the CP classification p>0.05. Child´s age was a predictor for dental caries experience p<0.05. CONCLUSIONS: The CP classification did not influence on caries experience, and the teeth most affected by dental caries were the primary and first permanent molars.

INTRODUÇÃO: Em indivíduos com paralisia cerebral, ocorre redução do fluxo salivar, o que pode predispor à hipomineralização do esmalte e à cárie dentária. OBJETIVO: Comparar a microdureza do esmalte de dentes decíduos anteriores e posteriores de crianças com paralisia cerebral (PC) e crianças normorreativas (NR). MATERIAL E MÉTODO: Foram utilizados 58 dentes decíduos esfoliados/extraídos, que foram distribuídos em quatro grupos: (A) anteriores de PC (n=14); (B) anteriores de NR (n=16); (C) posteriores de PC (n=14), e (D) posteriores de NR (n=14). Os dentes foram seccionados, incluídos em resina acrílica, lixados e polidos. A análise da microdureza longitudinal foi realizada em microdurômetro com endentador do tipo Knoop, com carga estática de 25 g/f e tempo de 5 segundos. Foram realizadas três fileiras de 12 endentações, distanciadas entre si em 100 mm, com profundidade entre 10 e 180 mm. Foram obtidas as médias de cada profundidade para cada dente e, posteriormente, as médias de cada profundidade para cada grupo avaliado. O teste estatístico empregado foi o de Mann-Whitney (α=5%). RESULTADO: As médias de microdureza foram 253,5 ± 55,3 (A), 247,6 ± 45,7 (B), 284,4 ± 68,7 (C) e 252,2 ± 53,8 (D). Não houve diferença estatisticamente significante entre os grupos A e B e os grupos C e D, para cada profundidade e para a média global (p>0,05). CONCLUSÃO: Pôde-se concluir que a microdureza de esmalte de dentes decíduos anteriores e posteriores de crianças com PC foi semelhante à microdureza de esmalte de dentes decíduos de crianças normorreativas.

INTRODUCTION: Cerebral palsy subjects present reduced salivary flow, which may predispose to enamel hypomineralization and dental caries. AIM: To compare the enamel microhardness of anterior and posterior primary teeth of normorreactive children (NR) and children with cerebral palsy (CP). MATERIAL AND METHOD: The sample was consisted of 58 exfoliated/extracted primary teeth distributed into 4 groups: (A) anterior teeth of CP (n= 14), (B) anterior teeth of NR (n=16),(C) posterior teeth of CP (n=14) and (D) posterior teeth of NR (n=14). The teeth were hemisectioned, embedded in acrylic resin, grounded and polished. The longitudinal microhardness analysis was carried out in a microdurometer with a Knoop indenter with a 25-gram load for 5 seconds. Three rows of 12 indentations were made from the outer surface from 10 to 180 mm. The rows were spaced 100 mm from each other. The values of the three measurements at each distance were averaged for each tooth and, afterwards, for each group. The non-parametric statistical test used was the Mann-Whitney U (α=5%). RESULT: The microhardness means data were 253.5 ± 55.3 (A), 247.6 ± 45.7 (B), 284.4 ± 68.7 (C) and 252.2 ± 53.8 (D).There was no significant difference between the groups A and B, as well as groups C and D for each distance and global average (p>0.05). CONCLUSION: It can be concluded that the enamel microhardness of anterior and posterior primary teeth of cerebral palsy children was similar to the normorreactive children.

AIM: To investigate whether oral motor performance is determinant for the hydration status and the effect of a supplemental oral fluid supply on salivary osmolality.METHODS: The sample consisted of 99 children with cerebral palsy aged 6 to 13 years old. In this study, children participated in 2-day evaluations: 1st day - baseline: saliva collection, caries experience and oral motor performance evaluations; and 2nd day: saliva collection after supplemental fluid supply. Prior to each evaluation, the participants were trained for saliva collection. Unstimulated whole saliva was collected using cotton roll at baseline, with the amount of fluid usually offered by caregivers, and 48 h after baseline, with as much as twice the normal daily fluid intake previously offered. Salivary osmolality was measured using a freezing point depression osmometer. Caries experience index for decayed, missed and filled teeth (DMFT) was evaluated. According to the Oral Motor Assessment Scale, the children were classified into subfunctional or functional groups. Chi-square, Student's t test and Pearson's correlation coefficient were used.RESULTS:The subfunctional group presented a higher percentage of quadriplegic children (p<0.001), with significantly higher values for caries experience (p<0.001) and salivary osmolality (p<0.001), which did not diminish when supplemental fluid supply was offered, compared with the functional group (p=0.001).CONCLUSIONS: The effectiveness of oral motor performance plays an important role in the hydration status of children with cerebral palsy and those with worse oral motor performance may be at higher risk of oral diseases.

INTRODUÇÃO: Estudos têm demonstrado que quanto maior a severidade do dano neurológico em crianças com paralisia cerebral (PC), maior é o risco de doenças orais. OBJETIVO: Correlacionar a experiência de cárie com a osmolaridade salivar em crianças com PC. MATERIAL E MÉTODO: Participaram do estudo 99 crianças (9,2±2,3 anos) com PC, em tratamento reabilitacional. A saliva de repouso foi coletada no período matutino, utilizando rolos absorventes (Salivette®) por cinco minutos. A osmolaridade salivar foi medida por depressão do ponto de congelamento em osmômetro. As avaliações incluíram a experiência de cárie pelo índice de dentes cariados, perdidos e obturados (CPOD), e o motor oral durante o processo de alimentação da criança. Foram utilizados os testes Qui-quadrado, t de Student, Correlação de Spearman e razão de chances (OR), com nível de significância de 5%. RESULTADO: O grupo 1 (G1) foi composto por 41 crianças livres de cárie e o grupo 2 (G2), por 58 crianças com cárie (CPOD=3,5±2,7). Os grupos foram homogêneos para gênero (p=0,884) e idade (p=0,174). Entretanto, diferiram significantemente com relação à motricidade oral, apresentando G2 maiores porcentagens de crianças com motor oral subfuncional (p<0,001) e tetraparéticos (p=0,001). O G2 apresentou valores significantemente maiores (p<0,001) para osmolaridade (99,6±36,7mOsml) quando comparado ao G1 (76,7±15,9mOsml). Observou-se uma correlação significante entre osmolaridade e experiência de cárie (p<0,001). Apresentar osmolaridade superior a 76,7 (OR=5,18; 1,85 a 14,83) foi determinante individual de maior probabilidade de apresentar risco de cárie (CPOD>0). CONCLUSÃO: Maiores valores de osmolaridade salivar aumentam o risco de cárie em crianças com PC.

INTRODUCTION: Studies have shown that the greater the severity of neurological damage in children with cerebral palsy (CP), the greater risk of oral diseases. OBJECTIVE: To correlate the caries experience with salivary osmolality in children with CP. MATERIAL AND METHOD: 99 CP children (9.2±2.3 years) in rehabilitation treatment participated of this study. Unstimulated whole saliva was collected in the morning using cotton roll (Salivette®) for 5 minutes. The salivar osmolality was measured using a freezing point depression osmometer. Caries experience index for decayed, missing and filled teeth (DMFT) and oral motor were assessed during the feeding process. It was used the chi-square test, t Student test, Spearman correlation and odds ratio (OR) with a significance level of 5%. RESULT: Group 1 (G1) consisted of 41 caries-free children, and group 2 (G2) of 58 children with caries (DMFT=3.5±2.7). The groups were homogeneous for gender (p=0.884) and age (p=0.174). However, they differed significantly with respect to the oral motor, with G2 showing higher percentages of children with sub-functional (p<0.001) and tetraparetic (p=0.001) oral motor. Group 2 showed significantly higher values (p<0.001) for salivar osmolarity (99.6±36.7 mOsml) compared to G1 (76.7±15.9 mOsml). There was a significant correlation between osmolality and caries experience (p<0.001). Presenting osmolality greater than 76.7 (OR=5.18, 1.85 to 14.83) was a individual determinant of higher probability for caries risk (DMFT>0). CONCLUSION: Higher values of salivary osmolality increase the risk of caries in CP children.