Angioid Streaks and Pseudoxanthoma Elasticum

Diseases

Angioid streaks (AS) and pseudoxanthoma elasticum (PXE) are rare diseases but can cause sever visual impairment.
PXE damages several organs: the eyes (AS), skin, and the cardiovascular system. Angioid streaks exist without being associated with PXE.

Angioid streaks are deep, pigmented orange lines located radially in the macula around the peripapillary area. They correspond to breaks in fragile zones of Bruch’s membrane, which allow the passage of choroidal neovascularization (CNV) into the subretinal space.

Even though this is a rare disease, unfortunatly neovascularization is a very common complication (72%-86% of cases), it is often bilateral (85% of cases) and the average time to bilateralization is very short (18 months).
Evolution of neovascular complications was extremely serious .The spontaneous evolution led to visual acuity lower than 1/10, legal blindness before 50 years of age. This disease has serious socio economic impact and produces severe repercusions in the family context because of the young age of affected patients.

Management

Recently, the hope of an effective treatment has been confirmed by a publication in the American Journal of Ophthalmology by the Créteil team.
We used anti-VEGF intraocular injection, which is the first treatment proven to stabilize visual acuity in 86% of patients with choroidal neovascularization complicating angioid streaks and in 93% of patients with PXE.
The results of this large series with a long follow-up (two years) were confirmed by two smaller studies, one American and the other Italian.

To improve the quality of diagnosis and therapeutic management, a specialized consultation was established in January 2011 by Dr Gerard Mimoun and Pr Eric Souied.