Congenital Zika Syndrome in Puerto Rico, Beyond Microcephaly, A Multiorgan Approach.

OBJECTIVE: Zika virus (ZIKV) infection was identified in Puerto Rico on December 2015, and the outbreak encouraged us to characterize clinical manifestations and laboratory findings of intrauterine exposed infants. METHODS: Retrospective medical record review of infants born to mothers with confirmed ZIKV infection during pregnancy was performed from January 2016-June 2017. We included patients admitted to UPH Neonatal Intensive Care Unit or referred for follow-up at UPH High Risk Clinics. The database was approved by the University of Puerto Rico, Medical Sciences Campus, IRB. RESULTS: 191 infants born to ZIKV positive mothers during pregnancy were identified. Normal head sonogram was found in 93% of the normo cephalic infants. Ocular findings were reported in 50% of the patients with microcephaly and 31% of the normo-cephalics. Fifteen newborns (7.8%) presented with microcephaly, of which 73% showed calcifications in head sonogram, and had severe anomalies on brain MRI. Auditory brainstem response test was performed on all newborns, 80% were within normal limits. CONCLUSION: Among the group of infants born to mothers with Zika positive test 4% had microcephaly. Of concern to us is the fact that 31% of normo cephalic infants had ocular manifestations and 7% of them had findings on head sonogram. While microcephaly is the severest form of Congenital Zika Syndrome, ocular manifestations might characterize the spectrum of disease. These findings reiterate the importance of detailed neonatal evaluations of exposed infants.

Association of Zika Virus Exposure in Utero with Ocular Phenotypes in a Group of Newborns in Puerto Rico Exposure with ocular phenotypes in newborns in Puerto Rico.

OBJECTIVE: After the outbreak in January 2016, researchers in Brazil reported the first cases of ophthalmic abnormalities in infants with microcephaly and presumed Zika virus (ZIKV) intrauterine infection. Screening for ocular lesions in all newborns exposed to ZIKV prenatally has been emphasized because of a chorioretinal macular scar found in a number normocephalic patient exposed to ZIKV. METHODS: A retrospective review of the medical records of infants born to mothers found to be ZIKV-positive during their pregnancies (January 2016-July 2017) was performed. We included all newborns and infants admitted to the NICU and/or receiving ambulatory care at the high-risk clinics at the University Pediatric Hospital, San Juan, Puerto Rico. The creation of this database was approved by the IRB of the University of Puerto Rico, Medical Sciences Campus. RESULTS: Records from 234 newborns born to Zika-positive mothers were identified. The ophthalmic evaluations of 95 patients were available. Sixty-three of them had normal findings (66%). Twelve of the 95 patients had microcephaly (12.6% of the cohort). Of the microcephalic group, half had normal ophthalmological findings. Of the normocephalic group (83/95), almost 31% had ocular findings: a small optic nerve in 3%, a double-ring sign in 10%, macular stippling in 8%, mottling in 4%, an oval optic nerve in 2%, a tilted nerve in 1%, and blunted foveal reflex in 1%. CONCLUSION: We report herein ZIKV associated ophthalmologic findings, similar to those published, in 34% of the infants with suspected/confirmed ZIKV fetal exposure. These data underline the importance of ocular examination in patients with suspected congenital Zika but without other findings on physical exam.