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Congenital heart diseases consist of structural defects of the heart and represent serious malformations, which manifest in newborns, contributing significantly to perinatal mortality. The dental management of patients with this condition may require caution by professionals, owing to the possibility of bleeding and the risk of causing an infectious endocarditis due to the variety of microorganisms residing in the oral cavity. The present case report aimed to detail dental care under general anesthesia in a hospital for a child with congenital heart disease. Female patient, 4 years old, was diagnosed with atrial and interventricular communication and hemodynamic repercussion 21 days after birth. The patient received dental treatment according to the proposed plan, which included dental restorations, conservative pulp therapy and extraction of lower anterior teeth. Posteriorly, a removable partial prosthesis was made for the patient for the purpose of functional aesthetic rehabilitation. The use of general anesthesia may reduce the risk of infective endocarditis and allows the performance of several dental procedures with a single antibiotic prophylaxis, since the cardiac pediatric patient needs a correct planning of the procedures to be performed. Las cardiopatías congénitas consisten en defectos estructurales del corazón y representan graves malformaciones, que se manifiestan en los recién nacidos, contribuyendo significativamente a la mortalidad perinatal. El manejo odontológico de pacientes en esta condición puede requerir precaución por parte de los profesionales, debido a la posibilidad de sangrado y al riesgo de causar una endocarditis infecciosa debido a la variedad de microorganismos que residen en la cavidad bucal. El presente reporte de caso tuvo como objetivo detallar la atención dental bajo anestesia general en un hospital para un niño con cardiopatía congénita. Paciente de sexo femenino, 4 años, diagnosticada de comunicación auricular e interventricular y repercusión hemodinámica a los 21 días del nacimiento. Recibió tratamiento dental de acuerdo con el plan propuesto, que incluyó restauraciones dentales, terapia pulpar conservadora y extracción de dientes anteriores inferiores. Luego, se realizó una prótesis parcial removible para el paciente con el propósito de rehabilitación estética funcional. El uso de anestesia general puede reducir el riesgo de endocarditis infecciosa y permitió la realización de varios procedimientos dentales con una única profilaxis antibiótica, ya que el paciente pediátrico cardíaco necesita una correcta planificación de los procedimientos a realizar. As cardiopatias congênitas consistem em defeitos estruturais do coração e representam malformações graves, que se manifestam nos recém-nascidos, contribuindo significativamente para a mortalidade perinatal. O manejo odontológico de pacientes nessa condição pode exigir cautela dos profissionais, devido à chance de sangramento e ao risco de causar uma endocardite infecciosa devido à variedade de microrganismos que residem na cavidade oral. O presente relato de caso teve como objetivo detalhar o atendimento odontológico sob anestesia geral em um hospital para uma criança com cardiopatia congênita. Paciente do sexo feminino, 4 anos, com diagnóstico de comunicação atrial e interventricular e repercussão hemodinâmica 21 dias após o nascimento. Recebeu tratamento odontológico de acordo com o plano proposto, que incluía restaurações dentárias, terapia conservadora pulpar e exodontia de dentes anteriores inferiores. Em seguida, foi confeccionada uma prótese parcial removível para o paciente com a finalidade de reabilitação estética funcional. O uso da anestesia geral pode reduzir o risco de endocardite infecciosa e possibilitar a realização de diversos procedimentos odontológicos com uma única profilaxia antibiótica, uma vez que o paciente cardíaco pediátrico necessita de um planejamento correto dos procedimentos a serem realizados.

Esclerose tuberosa é uma condição rara e autossômica dominante. Miofibroblastos são células que exibem fenótipo híbrido entre fibroblastos e células musculares lisas. O objetivo deste caso clínico é mostrar as características clínicas e histopatológicas da esclerose tuberosa e avaliar miofibroblastos nos angiofibromas cutâneos dessa condição. Lesões removidas foram coradas em HE e tricrômico de Masson. Para determinar a presença de miofibroblastos foi usada imunoistoquímica para α-SMA. Essa reação mostrou-se negativa para miofibroblastos. Como α-SMA é um marcador específico para essas células, esse resultado sugere que miofibroblastos não estejam envolvidos com os angiofibromas da esclerose tuberosa descrita. Tuberous sclerosis is a rare autosomal dominant disorder. Myofibroblasts are cells with a hybrid phenotype between fibroblasts and smooth muscle cells. The objective of this study is to describe clinical and histopathological characteristics of tuberous sclerosis and to conduct an immunohistochemical evaluation of myofibroblasts in cutaneous angiofibromas present in this condition. Lesion sections removed were stained with hematoxylin-eosin and Masson's trichrome. Immunohistochemistry against α-SMA was done to determine the presence of myofibroblasts, and the reaction was negative. Since α-SMA is a specific marker for myofibroblasts, this result suggests that myofibroblasts are not involved in cutaneous angiofibromas present in the tuberous sclerosis case reported.

Fissuras do lábio e/ou palato (FL/P) representam as anomalias congênitas mais comuns da face, correspondendo a aproximadamente 65% de todas as malformações da região craniofacial. OBJETIVO: Descrever casos clínicos incomuns de FL/P não-sindrômicas (FL/PNS), diagnosticadas em um Serviço de referência em Minas Gerais, Brasil, e correlacionar tais alterações com possíveis fatores de risco. CASUÍSTICA E MÉTODOS: Realizou-se estudo retrospectivo, entre os anos de 1992 e 1º semestre de 2009, a partir dos prontuários clínicos. RESULTADOS: Entre 778 casos de FL/PNS diagnosticadas no período de 17 anos, 5 (0,64%) foram de FL/PNS incomuns, sendo todos os pacientes do gênero masculino. Verificou-se que, dos 5 pacientes, 2 apresentaram fissura labial direita incompleta associada à fissura palatina incompleta; 2 eram afetados por fissura labial esquerda incompleta e fissura palatina incompleta e 1 com fissura lábio-palatina esquerda completa e fissura palatina direita completa. Fatores de risco como consanguinidade, tabagismo e etilismo materno, uso de medicamento na gestação, histórico de aborto e/ou natimorto e doenças maternas não foram associados às FL/ PNS incomuns CONCLUSÕES: Este estudo descreveu 5 casos raros de FL/PNS em uma população brasileira, não apresentando correlação com os fatores de risco analisados. Confirmou-se ainda a raridade na prevalência de tais alterações. Cleft lip and/or palate (CL/P) represent the most common congenital anomalies of the face, corresponding to approximately 65% of all malformations of the craniofacial region. AIM: to describe unusual clinical cases of non-syndromic CL/P (CL/PNS), diagnosed in a reference service in Minas Gerais, Brazil, and correlate these alterations with possible risk factors. MATERIALS AND METHODS: we carried out a retrospective study, between the years of 1992 and the 1st half of 2009, from medical records. RESULTS: Among the 778 cases of CL/PNS diagnosed in the period of 17 years, 5 (0.64%) were unusual CL/PNS, and all patients were male. It was found that among the 5 patients, 2 had incomplete right cleft lip with incomplete cleft palate, 2 were affected by left incomplete cleft lip and incomplete cleft palate, and 1 had a cleft lip and palate associated with complete right cleft palate. Risk factors such as consanguinity, maternal smoking and alcohol consumption, medication usage during pregnancy, history of abortion and/or stillbirths and maternal diseases were not associated with unusual CL/PNS. CONCLUSIONS: This study described 5 unusual cases of CL/PNS in a Brazilian population; no associations with the risk factors analyzed were seen. It also confirmed the unusualness of the prevalence of such alterations.

Cleft lip and/or palate (CL/P) represent the most common congenital anomalies of the face, corresponding to approximately 65% of all malformations of the craniofacial region.to describe unusual clinical cases of non-syndromic CL/P (CL/PNS), diagnosed in a reference service in Minas Gerais, Brazil, and correlate these alterations with possible risk factors.we carried out a retrospective study, between the years of 1992 and the 1st half of 2009, from medical records.Among the 778 cases of CL/PNS diagnosed in the period of 17 years, 5 (0.64%) were unusual CL/PNS, and all patients were male. It was found that among the 5 patients, 2 had incomplete right cleft lip with incomplete cleft palate, 2 were affected by left incomplete cleft lip and incomplete cleft palate, and 1 had a cleft lip and palate associated with complete right cleft palate. Risk factors such as consanguinity, maternal smoking and alcohol consumption, medication usage during pregnancy, history of abortion and/or stillbirths and maternal diseases were not associated with unusual CL/PNS.This study described 5 unusual cases of CL/PNS in a Brazilian population; no associations with the risk factors analyzed were seen. It also confirmed the unusualness of the prevalence of such alterations.

Tuberous sclerosis is a rare autosomal dominant disorder. Myofibroblasts are cells with a hybrid phenotype between fibroblasts and smooth muscle cells. The objective of this study is to describe clinical and histopathological characteristics of tuberous sclerosis and to conduct an immunohistochemical evaluation of myofibroblasts in cutaneous angiofibromas present in this condition. Lesion sections removed were stained with hematoxylin-eosin and Masson's trichrome. Immunohistochemistry against alpha-SMA was done to determine the presence of myofibroblasts, and the reaction was negative. Since alpha-SMA is a specific marker for myofibroblasts, this result suggests that myofibroblasts are not involved in cutaneous angiofibromas present in the tuberous sclerosis case reported.