Título / Title

MULTIMODAL ANALYSIS AND TREATMENT IN A PATIENT WITH PSEUDOXANTHOMA ELASTICUM: A CASE REPORT.

Introdução / Purpose

To do a multimodal analysis of choroidal neovascularization and therapeutic response in patients with Pseudoxanthoma elasticum and angioid streaks. A 57-year-old female patient referred to ophthalmology care with acutely reduced left central vision for 4 days. The best corrected visual acuity was 20/20 in the right eye and 20/200 in the left eye (BCVA). On funduscopic biomicroscopy, sub retinal hemorrhage was found with macular elevation of left eye. AS were noticed in the peripapillary area of both eyes, radiating to the periphery of the posterior pole and towards the fovea with slight foveal mottling. The patient had yellow coalescent papules on the neck which are primary skin lesions, and typical redundant skin folds developed.

Material e Método / Methods

We compared the images of the OCTA with OCT and FA of a patient treated with ranibizumab after three applications and your response to it. Three monthly ranibizumab injections were indicated as a proposed treatment. Unfortunately, the patient got conjunctivitis and she had a delay in the second dose. Therefore, the complete treatment was after the fourth month.

Resultados / Results

At baseline, fluorescein angiography confirmed the presence of a classic foveal CNV in the left eye and AS radiatings. Indocyanine green angiography did not evidence any alteration in both eyes. While the left eye showed subfoveal CNV. Seven days after the first injection, the patient had been evaluated and she had an increased in the BCVA (20/80 in the left eye), as well as in imaging OCT and OCTA. However, after the follow-up the patient has developed sub retinal fibrosis in the left eye.

Discussão e Conclusões / Conclusion

The treatment with anti-vgef is the gold standard today for CNV. It has been shown to be effective in the sub retinal neovascular membrane. Nevertheless, some cases progress to sub retinal fibrosis. In this case, it can not be concluded if the delay of the second drug application may have caused the unfavorable outcome.

Palavras Chave

Pseudoxanthoma elasticum; Angioids streaks; Treatment; sub retinal neovascular membrane.