< back

Macular telangiectasias type 2 (MacTel2)

Description

A 65-year-old male with a history of type 2 diabetes mellitus on metformin treatment presents with progressive vision loss in both eyes.

Visual acuity is 20/25 in the right eye and 20/30 in the left eye. The fundus has a practically normal appearance. OCT of the right eye shows disorganization of the internal retinal layers, a pseudocyst and a discontinuous external limiting membrane (ELM) in the area temporal to the fovea. OCT of the left eye shows the same disorganization of the internal layers, a pseudocyst, as well as discontinuity in the ELM and the ellipsoid zone (EZ), also in the area temporal to the fovea. Fluorescein angiography shows early hyperfluorescence with contrast leakage at late times in these areas temporal to the fovea. A diagnosis of idiopathic macular telangiectasias type 2 (MacTel2) is made.

Comments

MacTel2 are alterations of the perifoveal capillaries, associated with loss of the outer nuclear layer and ellipsoid zone and which may be accompanied by pseudocysts in all retinal layers. In advanced stages, secondary subretinal neovascularization (SRV) may appear. It is a bilateral pathology that is usually considered to be acquired. Its etiology is unknown at present, although the most accepted hypothesis is that the primary site of involvement is the Müller cells. In the early stages, the fundus is practically normal, although a grayish coloration temporal to the fovea and right-angled venules can be seen. Diagnosis is made by multimodal imaging with characteristic changes in autofluorescence (hyperAF due to loss of xanthophyll pigment), OCT (loss of MLE and EZ, pseudocysts and, in advanced stages, SRV) and fluorescein angiography. There are 5 progressive stages, with only treatment currently available for stage 5 (NVSR) with anti-VEGF drugs.

Indication