Título

STRAATSMA SYNDROME

Introdução / Purpose

To report a case of unilateral persistent myelinated fibers in the retina causing amblyopia.

Material e Método / Methods

Case report.

Resultados / Results

The association between myopia, strabismus and persistent myelinated fibers is known as Straatsma syndrome. The patient is currently in visual rehabilitation to develop the best visual acuity possible, although the poor prognosis.

Discussão e Conclusões / Discussion

A.R.C., female, 05 years old, caucasian, from Boa Vista – RR, was brought to the office for retinography. Her mother complaint of crossed eyes and low visual acuity in her left eye since birth. She was submitted to full eye examination and it was detected high myopia in both eye, but greater in her left eye. Exotropia of 45 PD with right eye dominant. Biomicroscopy with no abnormalities in both eyes, as well as her right eye fundoscopy. Her left eye exhibits a chalk-whitish lesion that begins in the optic disc and accompanies the retinal fibers and the temporal arcade. All those findings together indicate poor prognosis. Due to her early age and visual development stage, it was instituted spectacles and right eye occlusion 4h daily. She is waiting revaluation. In general, persistent myelinated fibers have no impact in visual acuity. When they are massive or located in macular region, as our patient, they can cause privation amblyopia. This is the worst type of amblyopia. That is the reason of the poor prognosis of Straatsma Syndrome. Although this patient has no familiar history, Straatsma syndrome can be familial. It´s a rare syndrome with a high impact of patient’s quality of life. It must be rolled in as the differentials of leukocoria, so it must be well-known by ophthalmologists.

Palavras Chave

Straatsma syndrome, persistent myelinated fibers, leukocoria

Area

CLINICAL RETINA