Título

CHOROIDAL NEOVASCULARIZATION IN PRESUMED HELICOID PERIPAPILLARY CHORIORETINAL DEGENERATION

Introdução / Purpose

To describe the case of presumed helicoid peripapillary chorioretinal degeneration (HPCD) which atypically evolved with choroidal neovascularization (CNV).

Material e Método / Methods

This is a descriptive study (case report) of one subject. Functional and anatomical recovery was assessed by Snellen visual acuity (VA), fundoscopy, optical coherence tomography (OCT) and fluorescein angiography (FA).

Resultados / Results

A 58-year old woman presented with vision loss in right eye (OD) for 2 months. Her past medical history included hypertension, presbyopia and serpiginous choroiditis, which remained stable and required no treatment for 10 years. On examination, the best corrected VA was 20/40 in OD and 20/20 in left eye (OS). Fundoscopy showed strips of chorioretinal atrophy arising from the optic disc towards the periphery in both eyes. In the right fovea, an irregular area raised the suspicion of CNV, confirmed by FA and OCT. The patient was treated with nine monthly injections of 0.5mg intravitreal ranibizumab along two years. In the third year of follow-up, VA was 20/25 in OD and the lesions remained stable with no residual activity.

Discussão e Conclusões / Discussion

Helicoid peripapillary chorioretinal degeneration (HPCD) is a rare inherited chorioretinal dystrophy first described by Sveinsson in 1939. The disease features symmetrical fundus radial tongue-shaped scars that emerge from the optic nerve, without inflammation. The progression of these lesions leads to visual impairment mainly due to foveal compromise. Not much is known about HPCD’s physiology. Studies have shown extensive choriocapillary damage and secondary CNV. Our patient showed a neovascular membrane which responded to anti-VEGF therapy. Classic neovascular triggering factors, such as inflammation and hypoxia, are considered absent in HCPD and could not be detected in our case. We hypothesize that secondary CNV would be explained by vascular growth factors release following progression of scars and chorioretinal integrity loss at the junction between viable and atrophic retina.

Palavras Chave

Helicoid peripapillary chorioretinal degeneration; Sveinsson chorioretinal atrophy; choroidal neovascularization