OSLI Retina

October 2016

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952 Ophthalmic Surgery, Lasers & Imaging Retina | Healio.com/OSLIRetina ■ C A S E R E P O R T ■ First Travel-Associated Congenital Zika Syndrome in the US: Ocular and Neurological Findings in the Absence of Microcephaly Camila V. Ventura, MD; Maria Paula Fernandez, MD; Ivan A. Gonzalez, MD; Delia M. Rivera-Hernandez, MD; Roberto Lopez-Alberola, MD; Maria Peinado, MD; Angelica A. Floren, MD; Patricia A. Rodriguez, MD; Basil K. Williams Jr., MD; Gabriela de la Vega Muns, MD; Ana J. Rodriguez, RN; Catherin Negron, BA; Brenda Fallas; Audina M. Berrocal, MD ABSTRACT: A 6-day-old female baby with known di- agnosis of congenital Zika infection was referred for ophthalmologic examination. The mother (37 years old) was referred for a pruritic rash, conjunctival hy- peremia, and malaise at 12 weeks of gestation while still living in Venezuela. Upon arrival to Miami, Zika virus (ZIKV) exposure was confirmed during prena- tal screening. At birth, due to the known exposure, a complete congenital ZIKV workup was performed, including brain ultrasound and MRI, which disclosed calcifications in the frontal lobe. Fundus examination revealed a hypopigmented retinal lesion in the left eye that was documented with retinal imaging. [Ophthalmic Surg Lasers Imaging Retina. 2016;47:952-955.] INTRODUCTION Zika virus (ZIKV), a neurotropic flavivirus, is mainly spread to humans by the Aedes aegypti mosquitoes. 1 Despite being discovered more than 60 years ago, only recently ZIKV raised world concern due to an alarming emergence of microcephaly in babies born to mothers infected with the virus in the Northeast of Brazil. 2,3 The ZIKV outbreak that began in Brazil has affected other parts of Latin America and the Caribbean, and ultimately has now reached the United States. 4,5 Although travel-associated ZIKV infection has been identified in the U.S., a case of congenital Zika syndrome (CZS) has not been previously reported. 5 Herein, we describe the first travel-associated CZS case born in the U.S. CASE REPORT A 6-day-old female baby born to a 37-year-old Venezuelan mother at 40 weeks' gestational age via vaginal delivery was referred for ophthalmologic consultation after being diagnosed with brain calcifications and a known intrauterine infection of ZIKV. At 12 weeks of gestation, while living in Venezuela, the mother developed a pruritic rash followed by bilateral nonpurulent conjunctivitis and fever that resolved in a few days. Routine fetal ultrasound performed in Venezuela did not show any abnormalities. After arriving in Miami, the mother underwent her third trimester prenatal screening and was found to be immunoglobulin M (IgM)-positive for ZIKV. At birth, the baby's head circumference measured 34 cm (23rd percentile) and her weight was 3,690 grams (59th percentile). After being admitted to the neonatal intensive care unit, a full congenital infection workup was performed, including ZIKV investigation. Toxoplasmosis, rubella, cytomegalovirus, syphilis, human immunodeficiency virus, and Dengue fever virus were ruled out. Real-time reverse transcription polymerase chain reaction for ZIKV was negative in the cord blood, serum, urine, and cerebrospinal fluid (CSF). IgM antibodies for ZIKV were identified in the CSF, umbilical cord blood, and serum (Table). The encephalogram through the anterior fontanel revealed tubular calcifications at the grey-white matter junction. The brain MRI demonstrated intraparenchymal calcification in the left subcortical white matter of the frontal lobe. A smooth appearance of the right frontal and anterior temporal lobes suggested mild neuronal migration anomaly (Figure 1A and 1B). Ophthalmic examination was performed 6 days after delivery and a circular hypopigmented lesion superior to the optic nerve was observed on fundus From the Department of Ophthalmology, Bascom Palmer Eye Institute, Miller School of Medicine, University of Miami, Miami (CVV, MPF, BKW, CN, BF, AMB); the Altino Ventura Foundation, Recife, Brazil (CVV); the Depart- ment of Ophthalmology and Visual Sciences, Paulista School of Medicine, Federal University of São Paulo, São Paulo, Brazil (CVV); the Department of Pediatrics, Division of Pediatric Infectious Diseases, Jackson Memorial Hos- pital, Miller School of Medicine, University of Miami, Miami (IAG, DR, PAR); the Department of Neurology, Jackson Memorial Hospital, Miller School of Medicine, University of Miami, Miami (RL); the Department of Pediatrics, Jackson Memorial Hospital, Miller School of Medicine, University of Miami, Miami (MP, AAF, AJR); and the Department of Neuroradiology, Jackson Memorial Hospital, Miller School of Medicine, University of Miami, Miami (GD). Originally submitted August 28, 2016. Revision received September 1, 2016. Accepted for publication September 13, 2016. The authors report no relevant financial disclosures. Address correspondence to Audina M Berrocal, MD, Bascom Palmer Eye Institute, 900 NW 17th Avenue, Miami, FL 33136; email: aberrocal@med. miami.edu. doi: 10.3928/23258160-20161004-09 EXPEDITED REVIEW

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