Infratentorial abnormalities were found to be a strong predictor of ophthalmic abnormalities in children with congenital Zika syndrome (CZS), according to study results published in the Journal of the Pediatric Infectious Diseases Society.

In this cross-sectional study conducted in Paraíba, Brazil, researchers investigated whether the presence or absence of infratentorial abnormalities in children born with clinical and radiologic features of CZS was associated with ophthalmic abnormalities.

The study authors defined infratentorial abnormalities as the “presence of severe hypoplasia or dysmorphic cerebellum and vermis cerebelli associated with severe hypoplasia or absent segmentation of the brainstem.”

Continue Reading

Of the 75 infants (49.3% boys) included in the study, all had brain calcifications and 53 (70.7%) presented with microcephaly. Infratentorial abnormalities were present in 17 infants, and 16 (94.1%) had ophthalmic abnormalities. In the remaining 58 infants without infratentorial abnormalities, 16 (27.6%) had ophthalmic abnormalities (odds ratio [OR], 42.0; 95% CI, 5.1-342.9; P <.001). Infants with infratentorial abnormalities were more likely to have macular chorioretinal atrophy (OR, 23.7; 95% CI, 6.0-93.3; P <.001) and optic nerve abnormalities (OR, 11.5; 95% CI, 3.3-40.0; P <.001). Infratentorial abnormalities were associated more with ophthalmic abnormalities than microcephaly (94.0% vs 43.4%; P <.001).

There was a statistically significant difference in head circumference between infants with ophthalmic abnormalities (n=32) compared with infants without ophthalmic abnormalities (n=43): 29.1 cm vs 30.3 cm (P =.016). However, no significant difference in gestational age was noted between the 2 groups (P =.12).

Although infratentorial abnormalities were strongly associated with ophthalmic abnormalities, the absence of infratentorial abnormalities was not associated with a normal ophthalmologic outcome, as 16 of the 32 infants who had an ophthalmic abnormality did not have a radiologic predictor.

Limitations of this study include lack of inclusion of pregnant women with asymptomatic Zika virus infection, as well as a limited number of children with mild symptoms of CZS.

Given that the presence of infratentorial abnormalities was a significant predictor of ophthalmic abnormalities, the researchers advise that “All neonates of mother with possible [Zika virus] exposure at any time during pregnancy should have a complete ophthalmologic evaluation.”


Sampaio VV, Melo ASO, Coleman AL, et al. A novel radiologic finding to predict ophthalmic abnormalities in children with congenital Zika syndrome. J Pediatric Infect Dis Soc. Published online May 20, 2021. doi:10.1093/jpids/piab010