[Ophthalmological manifestations in Fabry's disease. Four clinical cases showing deficient alpha-galactosidase-A activity]. / Manifestaciones oftalmológicas en la enfermedad de Fabry. A propósito de 4 casos con actividad deficiente de a-galactosidasa A.

CASE REPORT: Fabry's disease is an illness produced by an alteration in the catabolism of the glycosphingolipids. We report ophthalmologic findings in 4 people, detected after 113 patient evaluations from an analytical, cardiological and genetic point of view. DISCUSSION: Fabry's disease is uncommon and shows variable ophthalmologic affectation. Some patients with Fabry's disease do not present ocular affectation, while, on the other hand, healthy carriers with important ocular alterations have been described. The deposit of glycosphingolipids produces affectation at the corneal, crystalline, vascular and retinal levels. The vascular alterations affect not only the veins but also the arteries, as we report in our patients.

[Circumscribed choroidal hemangioma treated with photodynamic therapy]. / Hemangioma circunscrito de coroides tratado con terapia fotodinámica.

CLINICAL CASE: A 45-year-old male referred with a scotoma in the left eye of 2 months' evolution. A peripapillary tumor was found, with a basal diameter of 7 mm and thickness 3.4 mm. It was diagnosed as a circumscribed choroidal hemangioma. It was decided to treat the patient using photodynamic therapy. After 4 sessions and 17 months follow-up, the patient remained asymptomatic and visual acuity was stable. There was evidence of subretinal fibrosis and no subretinal fluid was found. DISCUSSION: Photodynamic therapy is a good option for the treatment of circumscribed choroidal hemangioma given the minimal damage is causes to the adjacent retina.