Three Cases of Congenital Retinal Macrovessel, One Coexisting with Cilioretinal Artery

Mauthner¹ first reported a large aberrant retinal vessel crossing the macula in 1869. In 1982, Brown et al.² described the clinical and fluorescein angiographic features of congenital retinal macrovessel (CRM) in seven patients. Impairment of vision in the involved eye is uncommon and is characterized by foveal cyst, macular hemorrhage, serous macular detachment, branch retinal artery occlusion or other vascular abnormalities.³ CRM occurs mainly in veins but more rarely may stem from an artery or artery and vein together.⁴ Beatty et al.⁵ in this case report we present a cilioretinal artery connecting with a CRM, suggesting that such patients are at increased risk of retinal vascular decompensation. Herein, we present three cases showing no vision loss in routine ophthalmological examination, and interestingly, one patient had both CRM and cilioretinal artery.

Congenital retinal macrovessel is a rare finding and is usually discovered incidentally. CRM are mesenchymal in origin and develop around the first weeks of the second trimester when differentiation of arteries and veins occurs.⁶ They are generally asymptomatic, and vision is not affected in most cases. Archer et al.⁷ classified congenital retinal arteriovenous communications into three groups. Group 1 arteriovenous communications are the mildest variant, and clinically, can be very subtle. Group 2 are larger than those of group 1. Our case 2 was compatible with group 1 and our cases 1 and 3 were compatible with group 2 of the Archer classification. To our knowledge, a congenital retinal venous macrovessel that communicates with a cilioretinal artery is very rare. This condition was first described by Beatty et al.⁵ Most of the cases of CRM that have been documented to date exhibited normal visual acuity.² When macrovessel is associated with reduced vision, one of the rare conditions should be considered: foveal cyst, macular hemorrhage or serous detachment, macular ischemia, branch retinal artery occlusion, and Valsalva retinopathy.⁸ For this reason, clinicians should be vigilant and follow these patients regularly.