Multimodal Imaging Characteristics in Unilateral Occlusive Macular Telangiectasia with Atypical X-Shaped Lesion

TL;DR

This paper describes a rare case of a unique X-shaped lesion in a type of retinal disease called MacTel, using multiple imaging techniques to better understand its characteristics.

Contribution

The paper introduces a novel X-shaped lesion pattern in Type 3a MacTel, expanding the known imaging spectrum of the disease.

Findings

Multimodal imaging revealed an X-shaped hypopigmented lesion with central pigmentation and ischemic features.

OCTA confirmed right-angled venules and localized ischemia in the deep capillary plexus.

The case supports the diagnosis of Type 3a MacTel with distinct vascular and structural abnormalities.

Abstract

Macular Telangiectasia (MacTel) is a rare retinal vascular disorder, with Type 3a MacTel being a distinct form characterized by retinal ischemia with the classical findings of MacTel, such as juxtafoveal telangiectasis, right-angled venules, and deep capillary plexus involvement without central nervous system findings. This case presents a novel X-shaped lesion pattern and ischemic features, expanding the known imaging spectrum of MacTel. A 53-year-old male with diabetes and a history of aripiprazole use presented with persistent blurred vision, a black curtain sensation, and metamorphopsia in the right eye. Visual acuity was 0.8 in the right eye and 1.0 in the left. A multimodal imaging approach, including fundus photography, fundus autofluorescence (FAF), fluorescein angiography (FFA), optical coherence tomography (OCT), and optical coherence tomography angiography (OCTA), was used to evaluate structural and vascular abnormalities. Fundus examination revealed an X-shaped hypopigmented lesion with central pigmentation. FAF showed hypoautofluorescence, indicating chronic RPE loss, and no loss of foveal autofluorescence was observed. FFA demonstrated progressive hyperfluorescence with perifoveal aneurysmal and telangiectatic vessels, along with a slightly enlarged foveal avascular zone (FAZ), suggesting ischemic involvement. OCT revealed intraretinal cysts, a disruption of the ellipsoid zone and external limiting membrane, pigment epithelial detachment, and increased choroidal backscattering. OCTA confirmed right-angled venules, aneurysmal telangiectatic vessels, and localized ischemia predominantly affecting the deep capillary plexus. This case highlights a rare variant of Type 3a MacTel with a unique X-shaped lesion. The presence of juxtafoveal telangiectasis, vascular occlusion, right-angled venules, and deep capillary plexus changes supports the diagnosis. Multimodal imaging played a critical role in characterizing the disease and differentiating it from other macular disorders, contributing to an expanded understanding of the clinical and imaging spectrum of MacTel.

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