Natural course and multimodal imaging of macular oedema associated with congenital retinal macrovessel: a case report

Author: Ioannis Markopoulos (Greece)

Co-authors: Spyros Atzamoglou, Vasilios Peponis, Ioannis Halkiadakis

Purpose

To present a case of unilateral congenital retinal macrovessel (CRM) complicated with cystoid macular oedema and decreased visual acuity, which showed spontaneous resolution and visual recovery within a few days from presentation.

Setting/Venue

Ophthalmiatrio Athinon Hospital, Athens, Greece

Methods

We report a case of a 54 years old female who presented with sudden unilateral visual impairment of 3-days onset in her left eye. Her past medical history included Hashimoto thyroiditis.

Results

Visual acuity in the left eye was 6/15. Fundoscopy revealed a retinal macrovessel crossing the macula. Optical coherence tomography (OCT) showed significant cystoid macular oedema and subretinal fluid in the fovea. OCT angiography (OCTA) revealed the retinal macrovessel located superiorly to the fovea, an inferior foveal branch of the macrovessel with stenosed lumen at its origin, an irregular foveal avascular zone (FAZ) and an aberrant small vessel crossing the FAZ. The patient was re-examined two days later for further imaging. Surprisingly, visual acuity had recovered to 6/7.5 and OCT showed almost complete resolution of the macular oedema. Color fundus photography illustrated a large aberrant dilated retinal vein originating from the optic disc and crossing the macular area superiorly to the fovea. An inferior foveal branch of this macrovessel showed lumen stenosis at its branching point, surrounded by a white cuff of perivascular sheathing which extended to the inferior wall of the macrovessel. Capillary dilatation was noted in the broader macular area. Fluorescein angiography (FA) confirmed the venous nature of the macrovessel in the early frames. The macrovessel showed delayed venous filling. Microaneurysmal abnormalities and capillary dilatation were noted in the macula. There were no signs of dye leakage.

Conlusions

CRM is a rarely reported malformation of the retina which is usually an incidental finding without ophthalmic complications. However, as shown in our case, CRM can be complicated with macular oedema and result in visual impairment. CRM has also been associated with venous malformations of the brain, so our patient was referred for MRI of the brain, which disclosed no abnormal findings. In addition, in this case CRM was shown to be associated with telangiectatic vascular changes in the surrounding macular area, highlighting the various clinical features and complications, as well as the unpredictable natural course of this retinal malformation, which in our opinion warrants regular follow-up.

Financial Disclosure

NONE