The present study strongly suggests that the increasing trend of hydrocephaly at CAISM might be related to the ultrasonography prenatal diagnosis. The subsequent referral of these cases to CAISM is due to the fact that this center is located in a Reference Hospital. Finally, among the hydrocephaly clinic groups, the increasing trend is specially observed in the etiological heterogeneous group of isolated hydrocephaly.

Summaryobjective To investigate the association of Yellow Fever Vaccination (YFV) during pregnancy with the presence of structural defect in exposed babies.methods An observed/expected frequencies study, before and after the vaccination campaign against YF was designed. 304 babies exposed to YFV during the prenatal period underwent dysmorphological examinations. The expected frequencies of malformations were obtained from a reference population of 10 691 births occurred in the period immediately prior to the vaccination campaign and born in the same region. These frequencies were evaluated using Poisson distribution model.results The major malformation rate found in this study was 3.3% (CI 1.7-6.3%). Minor dysmorphisms, especially naevus, were significantly more frequent (P < 0.001) than in the reference population.conclusions The data here presented provide no indication that immunization with YFV early in pregnancy increases the risk of major malformations. However, the association found between YFV during pregnancy and minor dysmorphisms, especially pigmented naevus, seems to be a bias of evaluation. We suggest, nevertheless, that a reproductive risk hypothesis regarding minor dysmorphisms should be considered in future studies involving YFV.

Artigo submetido em 07.06.02, aceito em 03.12.02. ResumoObjetivo: analisar criticamente o aumento da incidência de hidrocefalia na Maternidade do CAISM -UNICAMP, identificando o(s) fator(es) que podem explicar esse aumento.Casuística e métodos: estudou-se os portadores de hidrocefalia nascidos no CAISM, no período de setembro de 1987 a dezembro de 1998, exceto as hidrocefalias secundárias a defeito de fechamento de tubo neural. Os dados foram coletados a partir dos arquivos do Programa de Genética Perinatal-CAISM, incluindo as fichas ECLAMC e as do Ambulatório de Genética Perinatal, além de prontuários médicos hospitalares dos recém-nascidos e/ou de suas mães. As hidrocefalias foram classificadas em quatro grupos clinicamente distintos. As incidências das hidrocefalias e suas tendênci-as foram estudadas a partir do teste de tendência de CochranArmitage.Resultados: foram registrados 111 recém-nascidos portadores de hidrocefalia (3,16/1.000 nascimentos). A incidência anual das hidrocefalias mostra uma tendência em aumento significativa (p=0,001), que se acentuou após 1992. Dos 4 subgrupos de hidrocefalias (isoladas, associadas a infecção, associadas a síndromes dismórficas e associadas a defeitos múltiplos), apenas entre as isoladas foi observada tendência em aumento significativa (p=0,001). A maioria, 85% (94/111), das hidrocefalias registradas no período tinha diagnóstico pré-natal, e dessas, em 66% identificou-se um encaminhamento para o CAISM devido a tal diagnóstico.Conclusões: o presente estudo sugere fortemente que a tendên-cia em aumento das hidrocefalias no CAISM está associada ao diagnóstico ultra-sonográfico pré-natal e conseqüente encaminhamento preferencial desses casos para o CAISM, dada a sua condição de hospital de referência na região. Dentre os grupos clínicos de hidrocefalia, a tendência em aumento se verifica especialmente no grupo, etiologicamente heterogêneo, das hidrocefalias isoladas.J Pediatr (Rio J) 2003;79(2):135-40: hidrocefalia congênita, diagnóstico pré-natal, incidência, ultra-sonografia. AbstractObjective: to investigate the increasing incidence of hydrocephaly at the Hospital CAISM-UNICAMP and to identify its probable causes.Methods: all children with hydrocephaly delivered at CAISM from September 1987 to December 1998 were studied. Those children presenting hydrocephaly due to neural tube defects were excluded. Data were collected from medical records of the Perinatal Genetics Sector (CAISM). Hydrocephaly incidence and statistical trend were analyzed using the Cochran-Armitage test.Results: during the study, 111 infants with hydrocephaly (3.16/ 1,000 births) were identified. The annual incidence shows a significant increasing trend of hydrocephaly cases (p = 0.001). This phenomenon has been more evident since 1992. After classifying hydrocephaly in four subgroups (i.e., isolated hydrocephaly, hydrocephaly associated with congenital infection, syndromic hydrocephaly, and hydrocephaly associated with multiple defects), only isolated hydrocephaly cases show a significant increasing t...

We describe an infant with Dandy-Walker malformation and tetramelic postaxial polydactyly type 1A. Parental consanguinity reinforces previous suggestions for autosomal recessive inheritance.

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