Título

X-LINKED JUVENILE RETINOSCHISIS (XLJR)

Introdução / Purpose

To present a case report of a patient with XLJR treated with topical dorzolamide.

Material e Método / Methods

A 9-year-old boy was referred to Hilton Rocha Foundation with progressive visual loss in both eyes and retinal detachment suspicion. There was no history of ophthalmic disease and his medical history was unremarkable. The best-corrected visual acuity (BCVA) was 20/70 in the right eye (OD) and 20/40 in the left eye (OS). Anterior segment biomicroscopy and Goldmann applanation tonometry were normal. The binocular indirect ophthalmoscopy exam evidenced the presence of retinoschisis in the inferior temporal quadrant in both eyes (figure 1). Complementary exams were requested to investigate the suspicion of retinoschisis.

Resultados / Results

Spectral-domain optical coherence tomography (SD-OCT) revealed a classic pattern with loss of the foveolar contour and the presence of foveal "cysts", mainly located in the external retinal layers (figure 2). Treatment with topical dorzolamide hydrochloride was initiated and after three months the BCVA improved to 20/30 in both eyes.

Discussão e Conclusões / Discussion

Retinoschisis is characterized by the abnormal splitting of the retina’s neurosensory layers. This ocular disease may mimic retinal detachment condition during the ophthalmic examination. Classic diagnostic feature of this disease is the foveal schisis that appears as small "cystic" spaces associated with fine radial striations in the central macular region. Peripheral retinoschisis can also occur in more than 50% of the affected cases. SD-OCT was fundamental for the diagnosis in this case and treatment with topical dorzolamide increased intraretinal fluid reabsorption.

Palavras Chave

Retinoschisis; Dorzolamide; Intraretinal Fluid; Foveal "Cysts"