Evidence that hydrocephalus is a complication in at least a proportion of patients with congenital Zika syndrome has been published in JAMA Neurology.

In an effort to describe the clinical features and imaging findings of this treatable but potentially fatal condition, 24 patients with congenital Zika syndrome who developed hydrocephalus were enrolled in this case series study. All 308 patients were born in October and November 2015 and followed up until mid-2017 in Brazil’s 2 largest national referral centers for congenital Zika syndrome.

The children were aged 3 to 18 months, 54% were female, and all presented with at least 1 positive test result for anti-Zika antibodies in cerebrospinal fluid, along with classic congenital Zika syndrome symptoms. When diagnosed with hydrocephalus, 58% of patients had signs or symptoms of the condition, such as worsening seizures, vomiting, irritability, and/or sudden increase of head circumference percentile. 

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Only 2 of 24 patients had no signs of hydrocephalus, but repeated imaging showed reduced brain volume. Cerebellar or brainstem hypoplasia was found in 18 of 23 patients during baseline imaging. All 23 patients with multiple scans showed marked increase of ventricular volume compatible with communicating hydrocephalus, as well as reduction of brain tissue that was visibly worse than on baseline imaging at the second computed tomographic scan.

The observations from this report were curated via clinical access to the largest group of oldest surviving patients with congenital Zika syndrome in Brazil, and according to investigators, “the association of severe microcephaly with hydrocephalus in some infants with [congenital Zika syndrome] is surprising.” Findings suggested that clinicians caring for children with congenital Zika syndrome need to be aware of this potential complication, and that routine central nervous system reimaging after a few months of life or in the presence of clinical worsening be done because of the finding that clear clinical symptoms were not always present.

The investigators concluded that further studies are needed to determine the overall risk, age of maximal risk, and methods of detection of hydrocephalus in patients with congenital Zika syndrome, as well as long term follow-up to determine the benefits of surgical intervention.

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van der Linden V, de Lima Petribu NC, Pessoa A, et al. Association of severe yydrocephalus with congenital Zika syndrome [published online November 19 2018]. JAMA Neurol. doi: 10.1001/jamaneurol.2018.3553