Ophthalmic Manifestations of Congenital Zika Syndrome in Colombia and Venezuela

Abstract

Importance: The ocular manifestations and sequelae of Zika virus infection are not well known. Recently, the World Health Organization changed the declaration of Zika as a public health emergency and designated the viral outbreak and related microcephaly clusters as a long-term program of work. This change indicates the urgent need to evaluate and document ophthalmic manifestations in patients for timely management of this disease. In addition, confirmation whether the public health problem in Brazil extends to other regions in South America is needed.

Objective: To report the ocular manifestations of congenital Zika syndrome with microcephaly in Colombia and Venezuela.

Design, setting, and participants: This prospective case series included 43 patients from 2 ophthalmic centers in Colombia and Venezuela who underwent evaluation from October 1, 2015, through June 30, 2016, and were clinically diagnosed with congenital Zika syndrome. Twenty patients were Hispanic; 13, African; 8, white; and 2, Native American.

Interventions: Ophthalmic and systemic evaluations and serologic testing were performed on all infants. Patients underwent external ocular examination and dilated ophthalmoscopy. Serologic testing ruled out toxoplasmosis, rubella, cytomegalovirus, syphilis, and human immunodeficiency virus.

Main outcomes and measures: Ophthalmic manifestations of congenital Zika syndrome.

Results: Of the 43 patients included in this series (28 female and 15 male), the mean (SD) age at examination was 2.1 (1.5) months. The mothers of all the children had no ophthalmic findings and did not report ocular symptoms during pregnancy. All patients had bilateral ophthalmic manifestations. Optic nerve findings included hypoplasia with the double-ring sign, pallor, and increased cup-disc ratio in 5 patients (11.6%). Macular abnormalities included mild to severe pigment mottling in 27 patients (63%) and lacunar maculopathy in 3 (6.9%). Chorioretinal scarring was present in 3 patients (7%). Eleven patients (26%) had a combination of lesions in the posterior pole. Five patients (12%) were diagnosed with congenital glaucoma, characterized by the clinical triad of epiphora, photophobia, and blepharospasm; increased intraocular pressure; corneal clouding at birth; and buphthalmos. These data reveal that 12% (95% CI, 5%-24%) of cases of congenital Zika with microcephaly had anterior segment abnormalities and 88% (95% CI, 76%-94%) had important macular and optic nerve abnormalities. The visual sequelae of these ophthalmic manifestations remain unknown.

Conclusions and relevance: Congenital Zika syndrome in the current study had severe ocular abnormalities, and all patients had bilateral involvement. Ocular findings were focal macular pigment mottling, chorioretinal atrophy with a predilection for the macular area, congenital glaucoma and optical nerve hypoplasia, and optic disc abnormalities. Ophthalmic examination is recommended in patients with congenital Zika syndrome.

Figure 1.. Optic Nerve Hypoplasia

Representative ophthalmoscopy in the left eye of a patient with congenital Zika syndrome demonstrates optic nerve hypoplasia with the double-ring sign (open triangle) and disc pallor.

Figure 2.. Severe Pigment Mottling

Representative ophthalmoscopy in the left eyes of 2 patients with congenital Zika syndrome demonstrates macular chorioretinal atrophy (open triangles) and focal pigment mottling (arrowheads).

Figure 3.. Chorioretinal Scarring

Representative ophthalmoscopy in the left eye of a patient with congenital Zika syndrome demonstrates a chorioretinal scar with macular retinal pigment epithelium hypopigmentation and scarce pigment surrounding 2 sharp areas of hypopigmentation (lacunae) with pigmentation in their borders and 250 µm and 500 µm in size.

Figure 4.. Bilateral Congenital Glaucoma

A patient with bilateral congenital glaucoma had the clinical triad of epiphora, photophobia, and blepharospasm; increased intraocular pressure; corneal clouding at birth; and buphthalmos in both eyes.