Association of Severe Hydrocephalus With Congenital Zika Syndrome.

Importance: Hydrocephalus is a treatable but potentially fatal complication that has not been previously described in congenital Zika syndrome (CZS). Objective: To describe the clinical features and imaging findings in 24 patients with congenital Zika syndrome (CZS) who developed hydrocephalus. Design, Setting, and Participants: This case series included patients with hydrocephalus who were born in October and November 2015 and followed up until mid-2017 in the 2 largest national referral centers for CZS in Brazil. The participants included consecutively enrolled children with a clinical and laboratorial diagnosis of CZS who developed clinical and/or image findings suggestive of hydrocephalus and who were confirmed to experience increased intracranial hypertension during ventriculoperitoneal shunt procedures. Main Outcomes and Measures: To retrospectively describe clinical and image findings in these 24 patients. Results: This multicenter cohort included 308 patients with CZS; 24 consecutive children were enrolled in this study. These children were aged between 3 to 18 months, and 13 of 24 (54%) were female. All patients presented with at least 1 positive test result for anti-Zika antibodies in cerebrospinal fluid or serum and had classic signs of CZS. At the time of hydrocephalus diagnosis, only 14 of 24 patients (58%) had symptoms and signs suggestive of hydrocephalus (mainly worsening seizures, vomiting, irritability, and/or sudden increase of head circumference percentile). Two of 24 patients (8%) had no symptoms suggestive of hydrocephalus but were found to have reduced brain volume on repeated imaging. Cerebellar or brainstem hypoplasia on baseline imaging were found in 18 of 23 patients (78%). At the second computed tomographic scan, all patients showed a marked increase of ventricular volume, compatible with communicating hydrocephalus, and reduction of brain tissue that was visibly worse than on baseline imaging for the 23 patients with repeated scans. Conclusions and Relevance: We present evidence that hydrocephalus is a complication of CZS in at least a proportion of patients. 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, we provisionally recommend that high suspicion and appropriate monitoring for hydrocephalus should be part of the standard care of patients with CZS.

Common findings on head computed tomography in neonates with confirmed congenital Zika syndrome.

OBJECTIVE: To describe head computed tomography (CT) findings in neonates with congenital Zika virus infection confirmed in cerebrospinal fluid. MATERIALS AND METHODS: This was a study of 16 newborn infants who exhibited abnormal head CT findings during an outbreak of Zika virus infection. Those infants had the following features: brain imaging suggestive of congenital infection; brain calcifications and negative results on tests for other main infectious causes of primary microcephaly, namely toxoplasmosis, cytomegalovirus, rubella, and HIV; positivity for Zika virus on IgM antibody capture enzyme-linked immunosorbent assay in cerebrospinal fluid. RESULTS: Decreased brain volume was observed in 13 (81.2%) of the infants. All of the infants showed cortico-subcortical calcifications, mainly located in the frontal lobe. In 15 neonates (93.7%), ventriculomegaly was observed. Colpocephaly was a common finding, being observed in 10 patients (62.5%). A prominent occipital bone was identified in 9 patients (56.2%). CONCLUSION: Our study proves that Zika virus infection can cause congenital brain damage, with or without microcephaly. Some predominant head CT findings in neonates with congenital Zika virus infection, although not pathognomonic, are strongly suggestive of a pattern.

Common findings on head computed tomography in neonates with confirmed congenital Zika syndrome / Achados frequentes na tomografia computadorizada do crânio em neonatos com síndrome congênita pelo vírus Zika confirmada

Abstract Objective: To describe head computed tomography (CT) findings in neonates with congenital Zika virus infection confirmed in cerebrospinal fluid. Materials and Methods: This was a study of 16 newborn infants who exhibited abnormal head CT findings during an outbreak of Zika virus infection. Those infants had the following features: brain imaging suggestive of congenital infection; brain calcifications and negative results on tests for other main infectious causes of primary microcephaly, namely toxoplasmosis, cytomegalovirus, rubella, and HIV; positivity for Zika virus on IgM antibody capture enzyme-linked immunosorbent assay in cerebrospinal fluid. Results: Decreased brain volume was observed in 13 (81.2%) of the infants. All of the infants showed cortico-subcortical calcifications, mainly located in the frontal lobe. In 15 neonates (93.7%), ventriculomegaly was observed. Colpocephaly was a common finding, being observed in 10 patients (62.5%). A prominent occipital bone was identified in 9 patients (56.2%). Conclusion: Our study proves that Zika virus infection can cause congenital brain damage, with or without microcephaly. Some predominant head CT findings in neonates with congenital Zika virus infection, although not pathognomonic, are strongly suggestive of a pattern.

Resumo Objetivo: Descrever os achados da tomografia computadorizada (TC) de crânio em neonatos com infecção congênita pelo vírus Zika confirmada no líquido cefalorraquiano. Materiais e Métodos: Série de 16 recém-nascidos que apresentaram achados anormais na TC de crânio durante o surto de infecção por vírus Zika. Essas crianças apresentavam: imagens cerebrais sugestivas de infecção congênita; calcificações do parênquima cerebral e sorologias negativas para as outras principais causas infecciosas de microcefalia primária, como toxoplasmose, citomegalovírus, rubéola; IgM pelo método ELISA para vírus Zika positivo no líquido cefalorraquiano. Resultados: Redução volumétrica do parênquima encefálico foi observada em 13 (81,2%) pacientes. Todos os recém-nascidos apresentaram calcificações na junção corticossubcortical, localizadas principalmente no lobo frontal (100%). Em 15 neonatos (93,7%), ventriculomegalia esteve presente. Colpocefalia foi um achado comum, ocorrendo em 10 pacientes (62,5%). Proeminência do osso occipital foi identificada em 9 pacientes (56,2%). Conclusão: Nosso estudo comprova que a infecção pelo vírus Zika pode causar lesões encefálicas congênitas, com e sem microcefalia. Há um conjunto de achados tomográficos predominantes na TC de crânio em recém-nascidos com infecção congênita pelo vírus Zika, não patognomônicos, mas fortemente sugestivos de um padrão.

Follow-up brain imaging of 37 children with congenital Zika syndrome: case series study.

Objective To compare initial brain computed tomography (CT) scans with follow-up CT scans at one year in children with congenital Zika syndrome, focusing on cerebral calcifications.Design Case series study.Setting Barão de Lucena Hospital, Pernambuco state, Brazil.Participants 37 children with probable or confirmed congenital Zika syndrome during the microcephaly outbreak in 2015 who underwent brain CT shortly after birth and at one year follow-up.Main outcome measure Differences in cerebral calcification patterns between initial and follow-up scans.Results 37 children were evaluated. All presented cerebral calcifications on the initial scan, predominantly at cortical-white matter junction. At follow-up the calcifications had diminished in number, size, or density, or a combination in 34 of the children (92%, 95% confidence interval 79% to 97%), were no longer visible in one child, and remained unchanged in two children. No child showed an increase in calcifications. The calcifications at the cortical-white matter junction which were no longer visible at follow-up occurred predominately in the parietal and occipital lobes. These imaging changes were not associated with any clear clinical improvements.Conclusion The detection of cerebral calcifications should not be considered a major criterion for late diagnosis of congenital Zika syndrome, nor should the absence of calcifications be used to exclude the diagnosis.