Systemic Manifestations, Tooth Eruption and Enamel Defects in Children with Congenital Zika Virus Syndrome: 36-Month Follow-up Case Series

Abstract Objective: To describe systemic manifestations, the characteristics related to tooth eruption, and the occurrence of enamel defects in children with Congenital Zika Virus Syndrome (CZS). Material and Methods: Prospective case series based on nine children with confirmed CZS diagnosis assisted at a reference center in a municipality in the Northeast Region of Brazil. Through a structured interview directed to mothers, information related to prenatal, delivery, and postpartum periods was collected. Tooth eruption was monitored through clinical examinations for 36 months. The modified developmental defect of enamel index (DDE) was used to identify opacities and hypoplasia. Data were presented using descriptive statistics. Results: A high proportion (77.8%) had microcephaly, and 55.5% had low birth weight. Musculoskeletal disorders, swallowing difficulty, and self-injury practices were present in all children. Among the systemic findings, visual impairment (77.8%) and seizures (77.8%) were widely reported. Concerning disorders related to the stomatognathic system, bruxism (66.7%) and difficulty in sucking (33.3%) were present. For most children (77.8%), the deciduous right lower central incisor was the first tooth to erupt (minimum 8 months and maximum 17 months). Enamel defects were diagnosed in only two children (22.2%). Conclusion: A wide range of systemic manifestations was observed in children with CZS, including visual impairment and musculoskeletal disorders. Delayed eruption of the first deciduous tooth was also observed. Enamel defects were present in a small proportion of children.

Correlation between cephalic circumference at birth and ocular alterations in patients with microcephaly potentially associated with Zika Virus infection

SUMMARY INTRODUCTION: The Zika virus (ZIKV) is an arbovirus isolated for the first time in 1947 and transmitted to humans by the Aedes aegypti mosquito. In Brasil, it was first detected in May 2015. Since then, ZIKV has been identified as the etiological agent of acute exanthematous disease in Brasil, and Neuropediatricians of the Recife warned about an epidemic of microcephaly, and the Brazilian Ministry of Health confirmed the association between ZIKV and Congenital malformations and neurological syndromes. The eye, as an extension of the developing brain, has been examined in patients with microcephaly and maternal history of ZIKV infection. METHODS: Twenty newborn patients with microcephaly, whose mothers had presumed Zika virus during pregnancy, were analyzed through medical records. The nonparametric chi-square statistic was used to verify the association between head circumference and ocular alteration at a significance level of 0.0001. RESULTS: The significance of P = 0.000 in the value of non-parametric chi-square statistics was lower than the value of α = 0.0001, demonstrating that, at a level of 0.0001, there is an association between head circumference and ocular alteration. CONCLUSION: Although the knowledge of the natural evolution of the disease is still scarce, the current evidence is strong enough to establish a causal relationship between ZIKV infection during pregnancy and the increased incidence of the microcephaly and serious eye alterations that lead to the severe lower vision of these children.

RESUMO: INTRODUÇÃO: O vírus Zika (ZIKV) é um arbovírus isolado pela primeira vez no ano de 1947, sendo transmitido para o homem pelo mosquito Aedes aegypti. No Brasil foi detectado pela primeira vez em maio de 2015. Desde então, ZIKV foi identificado como o agente etiológico da doença exantemática aguda no Brasil, e neuropediatras do Recife deram o sinal de alerta sobre uma epidemia de microcefalia, tendo o Ministério da Saúde do Brasil confirmado a associação entre ZIKV e malformações congênitas e síndromes neurológicas. O olho, sendo uma extensão do cérebro em desenvolvimento, tem sido examinado em pacientes com microcefalia e história materna de infecção por ZIKV. MÉTODO: Foram analisados, por meio de prontuário médico, 20 pacientes recém-nascidos, portadores de microcefalia, cujas mães tiveram presumidamente Zika vírus durante a gestação. A estatística não paramétrica Qui-Quadrado foi utilizada para verificar a associação entre perímetro cefálico e alteração ocular, no nível de significância de 0,0001. RESULTADOS: A significância de P=0,000 no valor da estatística não paramétrica de Qui-Quadrado foi menor do que o valor de α = 0,0001, demonstrando que podemos afirmar que, em um nível de 0,0001, há uma associação entre o perímetro cefálico e a alteração ocular. CONCLUSÃO: Apesar de ainda serem escassos os conhecimentos sobre a evolução natural da doença, as evidências atuais são fortes o suficiente para estabelecermos a relação causal entre a infecção pelo ZIKV durante a gravidez e o aumento da frequência da microcefalia e alterações oculares graves que levam à baixa severa da visão dessas crianças.

Clinical and x-ray oral evaluation in patients with congenital Zika Virus

Abstract Objective: The aim of this study was to investigate possible malformations in the soft, bone and/or dental tissues in patients with congenital Zika Virus (ZIKV) by clinical and x-ray evaluation. Methodology: Thirty children born with ZIKV and 30 children born without ZIKV (control group) were included in the study. Patients were evaluated over 24 consecutive months according to the variables: sex, age, cleft palates, soft tissue lesions, alveolar ridge hyperplasia, short labial and lingual frenums, inadequate posture of the lingual and perioral muscles at rest, micrognathia, narrow palatine vaults, changes in the teeth shape and/or number, sequence eruption, spasms, seizures and eruption delay were evaluated. Chi-square test, Student's t-test and nominal logistic regression were used (p<0.05). Results: Among the 30 babies examined, the mean age of the first dental eruption was 10.8±3.8 with almost two-thirds of the children (n=18, 60%) experiencing eruptions of their first tooth after 9 months of age, nine children (30%) had inadequate lingual posture at rest, more than half of the children (n=18, 60%) had short labial or lingual frenums. ZIKV babies showed a high prevalence of clef palate (p<0.001), inadequate lingual posture at rest (p=0.004), micrognathia (p=0.002), changes in the shape and/or number of teeth (p=0.006), alteration in sequence of dental eruption (p<0.001) and muscles spasms (p=0.002). The delay eruption was associated with inadequate lingual posture at rest (p=0.047), micrognathia (p=0.002) and changes in the shape and/or number of teeth (p=0.021). The delayed eruption (p=0.006) and narrow palatine vaults (p=0.008) were independently associated with ZIKV. Moreover, female patients showed the most narrow palatine vaults (p=0.010). Conclusions: The children with ZIKV showed a greater tendency to have delayed eruption of the first deciduous tooth, inadequate lingual posture and short labial and lingual frenums.

Achados clínicos faciais em bebês com microcefalia / Facial clinical findings in babies with microcephal

A microcefalia pode ser definida como uma redução significante na circunferência ocipitofrontal da cabeça. Desta forma, ela é um achado clínico e não deve ser descrita como uma doença. Suas consequências são dependentes da área cerebral afetada. Sabendo que o crescimento facial é dependente do crescimento do crânio, supõem-se que a microcefalia também possa causar deformações faciais e distúrbios em funções como fonação, mastigação e deglutição. O Hospital Universitário da Universidade Federal de Sergipe desenvolve um trabalho com bebês microcefálicos e proporciona atendimento multiprofissional para acompanhamento desses pacientes, a Odontologia foi incluída nesse grupo de trabalho a partir da iniciativa desse estudo. O presente trabalho teve como objetivo analisar as funções orais de bebês diagnosticados com microcefalia em acompanhamento no Hospital Universitário de Sergipe. Achados clínicos de importante relevância foram descritos baseados no acompanhamento destes bebês. Os resultados obtidos mostraram que as funções mais afetadas foram da deglutição, respiração, tonicidade muscular e erupção dentária. Conclui-se que devido à variabilidade e à imprevisibilidade das consequências da microcefalia, é imprescindível o acompanhamento preventivo odontológico destes casos.(AU)

Microcephaly can be defined as a significant reduction in the ocipitofrontal circumference of the head. In this way, it is a clinical finding and should not be described as a disease. Its consequences are dependent on the affected brain area. Knowing that facial growth is dependent on skull growth, it is assumed that microcephaly can also cause facial deformities and disturbances in functions such as phonation, chewing and swallowing. The University Hospital of the Federal University of Sergipe develops a work with microcephalic babies and sends multiprofessional care to follow up the patients, a dentistry was included in the work group from the initiative of the study. The present study had as objective to analyze as oral functions of babies diagnosed with microcephalus in follow-up, not Hospital Universitário de Sergipe. Clinical findings of major relevance have been reported in the follow-up of these infants. The results obtained are demonstrated as more affected functions such as swallowing, breathing, muscular tone and dental eruption. It is concluded that due to the variability and the unpredictability of the consequences of microcephaly, it is essential the dental preventive follow-up of these cases.(AU)

Seckel syndrome with chromosomal 18 deletion.

Four case records of patients with Seckel Syndrome (SS) were retrieved. Typical of bird headed dwarfism was seen in all. Chromosome 18 deletion was seen in one child with SS. MRI abnormalities were detected in 3 patients. Cytogenetic studies and neuroimaging is likely to provide important diagnostic and prognostic information.

Galloway-Mowat syndrome

Galloway-Mowat syndrome is a rare multisystem genetic disorder with constellation of neurological, skeletal, growth, facial, gastrointestinal and renal abnormalities. This case report describes Galloway-Mowat syndrome in a young boy suffering from congenital microcephaly, developmental delay, seizures and various dysmorphic features in whom nephritic syndrome became apparent at 5 years of age

First Turkish Patient with Floating Harbor Syndrome with Additional Findings: Cryptorchidim and Microcephaly

We report the first Turkish patient with Floating Harbor Syndrome (FHS). The 12-year old male patient exhibited classical dysmorphic features of FHS, mental retardation, celiac disease and additional undescribed findings: microcephaly and cryptorchidism.

Circunferencia cefálica en niños desnutridos menores de 2 años / Head circunference in children mal nutrition minor of two years

Se estudió el perímetro cefálico en 89 niños con desnutrición moderada y grave en edades comprendidas entre 0 y 2 años; ingresados al Hospital "J.M. de los Ríos" en el período enero-septiembre 1994. Se relacionó la circunferencia cefálica con la edad, sexo, estraficación socioeconómica motivo de ingreso, grado de desnutrición, tipo de desnutrición clínica, talla y formula de Boyd. El perímetro cefálico en niños con desnutrición proteínico energética es mucho menor en comparación con los patrones de referencia para una población normal (p<0.001). El perímetro cefálico se encuentra afectado en niños mayores de 18 meses (P<0.001,odds:2.8). El cálculo del perímetro cefálico según la fómula de Boyd se correlaciona con la medida de la circunferencia cefálica, con un coeficiente de correlación positivo de 0.9(P0.0001)

Chilaiditi's sign in a 12 year old mentally instutionalised blind girl: a new association?

A 12 year old, blind Bedouin female with microcephaly, profound mental retardation, proportionate short stature and associated hepato diaphragmatic interposition of the colon is reported. It seems that this association represents a new syndrome

Diagnóstico diferencial entre microcefalia primaria, craneoestenosis y cierre precoz de fontanela / Differential diagnosis between primary microcefalia craneostenosis and early consolidation of fontanela

En el presente estudio se reporta la incidencia y el diagnóstico diferencial entre microcefalia primaria, craneostenosis y cierre precoz de fontanela, en el Hospital Universitario de Caracas y en el Hospital de Niños J.M. de Los Ríos. Se encontró un aumento notable de un buen volumen de cierre precoz de fontanela anterior, los niños totalmente normales; se encontró también hipertensión endocraneana en 42% en craneoestenosis con más de una sutura estenosada y 13% en craneoestenosis con más de una sola sutura involucrada. nosotros concluimos que la presencia de un cierre prematuro de la fontanela anterior en un craneo que tiene forma, medidas y armonía simétrica en su constitución, puede corresponder a una variante normal del desarrollo