Hydrocephalus: New Complication of Congenital Zika Syndrome

November 23, 2018

Hydrocephalus may be a complication of congenital Zika syndrome, and the presenting signs and symptoms are challenging to recognize, a Brazilian group reports.

Monitoring for hydrocephalus, including assessing the potential harbinger of cerebellar or brainstem hypoplasia, should be part of the standard care of patients with this condition, they conclude.

"We present evidence that hydrocephalus is a complication of congenital Zika syndrome in at least a proportion of patients," the authors, led by Vanessa van der Linden, MD, Association for Assistance of Disabled Children, Recife, Brazil, comment.

"The clinical spectrum of this condition continues to evolve, but given that presenting signs and symptoms of hydrocephalus can be challenging to recognize in CZS [congenital Zika syndrome], we provisionally recommend that high suspicion and appropriate monitoring for hydrocephalus should be part of the standard care of patients with CZS," they conclude.

The report was published online November 19 in JAMA Neurology.

In their article, van der Linden and colleagues describe a case series of 24 patients aged 3 to 18 months who developed hydrocephalus and who had tested positive at least once for anti-Zika antibodies.

Fourteen of the patients had symptoms and signs suggestive of hydrocephalus (mainly worsening seizures, vomiting, irritability, and/or sudden increase of head circumference percentile); 18 had cerebellar or brainstem hypoplasia at baseline; and two who had no such symptoms but were found to have reduced brain volume on repeated imaging. On a second CT scan, all patients showed a marked increase of ventricular volume, compatible with communicating hydrocephalus, and a reduction of brain tissue that was visibly worse than on baseline imaging.

van der Linden and colleagues note that Zika is an arthropod-borne virus that can cause a mild febrile illness with maculopapular rash, joint pain, and conjunctivitis.

Zika infection was first reported in Brazil in 2015, and a dramatic increase in cases of microcephaly subsequently occurred. In 2016, the causative relationship between microcephaly and Zika was established.

However, presentations of congenital Zika syndrome go beyond microcephaly and include other clinical patterns, such as retinal abnormalities with visual impairment, hearing loss, and limb abnormalities, such as arthrogryposis.

Now for the first time, a series of infants with congenital Zika syndrome who developed hydrocephalus is described.

"The association of severe microcephaly with hydrocephalus in some infants with congenital Zika syndrome is surprising, and it is important for clinicians who care for children with congenital Zika syndrome to be aware of this potential complication," the authors write.

"Our observations stem from clinical access to the largest group of oldest surviving patients with congenital Zika syndrome in Brazil. Consequently, we are continually gaining new insights into the evolution and the natural history of this disease, its clinical spectrum, and its pathophysiology," they add.

They stress that because clear clinical signs and symptoms of hydrocephalus were not always present in these patients, routine central nervous system reimaging after a few months of life or in the presence of clinical worsening, even with nonspecific symptoms, is needed.

Further studies are required to determine the overall risk for hydrocephalus in patients with congenital Zika syndrome, as well as whether there is an age at which patients are at maximal risk and methods for early detection, the authors state. Long-term follow-up is also needed to determine the benefits of surgical intervention.

"Our data indicate that the clinical picture of congenital Zika syndrome has not yet been fully defined, and the possibility of hydrocephalus in the setting of severe microcephaly should be appreciated by clinicians caring for these children," they conclude.

JAMA Neurol. Published online November 19, 2018. Full text

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