Congenital retinal macrovessel: atypical presentation using optical coherence tomography

Abstract

To describe a congenital retinal macrovessel with macular thickening. This case was investigated using fundus photography, fluorescein angiography, Spectralis optical coherence tomography and a 10-2 visual field test. A 23-year-old man was referred to our clinic with decreased vision in the right eye. Fundus examination of the right eye revealed a congenital retinal macrovessel that originated inferior to the superotemporal branch of the central retinal vein. Using fluorescein angiography, early filling and delayed emptying of the aberrant vein were observed. Spectralis optical coherence tomography demonstrated macular thickening and was supported by a 10-2 visual field test that revealed a relative scotoma corresponding to the same location. At the 18-month follow-up, visual acuity remained stable. Although rare, this case demonstrated that macular thickening can cause decreased visual acuity in the presence of a congenital retinal macrovessel.

Retinal Microvascular Abnormalities in Neurofibromatosis Type 1 Associated with Congenital Retinal Macrovessels

“…A reduction of vision in such cases is rare. When it has occurred, it has been attributed to Valsalva retinopathy, the rupture of capillary alterations, foveal cysts, or the mere presence of an abnormal vessel in the foveal area [1–5]. …”

“…In patients with this condition, a vein in 1 eye is seen to cross the horizontal raphe and the macula. The associated visual disturbance is mild, and such cases may be detected only incidentally [1–5]. …”

Congenital Retinal Macrovessel: a Case Report

“…Most congenital retinal macrovessel cases are unilateral and stable with excellent visual prognosis and are detected on routine examination. In rare cases, foveolar cysts, macular hemorrhages, pigmentary changes, serous macular detachment and a displaced fovea may also be seen [1,2,[4][5][6]. These findings can deteriorate VA.…”

“…Its origin often derives from the vein without a significant clinical symptom and decreased visual acuity [3,5]. In rare cases, a congenital retinal macrovessel can be an artery or an artery and vein together [1][2][3]. In this article, we report a patient with a congenital retinal macrovessel crossing the foveal avascular region and touching the fovea, with a minimal visual defect and no macular thickening.…”

“…In 1982, Brown et al [1][2][3][4] formally termed such abnormal vessel as "congenital retinal macrovessel", which presents as unilateral, abnormally large, aberrant retinal vessel and transverses through the foveal avascular region in the posterior region. Such vessel supplies or drains the retina both inferiorly and superiorly to the horizontal raphe.…”