Congenital macular macrovessels

Background: Congenital aberrant macular vessels are rare and may cause visual impairment when crossing the fovea, when causing the formation of foveolar cysts, or when haemorrhage occurs. Methods: From the records of patients with vascular anomalies seen at the Retina Department of the University Federico II in Naples from 1980 to 2005, we reviewed all cases presenting an abnormal, large, retinal vessel crossing the macular region. Results: An anomalous macular macrovessel was present in 13 cases. Follow-up ranged from 3 months to 21 years (mean 14 years). In all cases the abnormal vessel was a vein, presenting a fluoroangiographic early filling and delayed emptying. In some cases there were microvascular bed anomalies, such as enlargement of the foveal avascular zone, focal capillary dilation, or microaneurysmal abnormalities. In one case late, mild, intraretinal staining along the anomalous vessel indicated retinal oedema. Visual impairment occurred in five eyes, being caused by a preretinal haemorrhage in one case and by the mere presence of the macrovessel in the foveal area in four cases, and had not improved at following controls. Conclusion: In the presence of an aberrant vessel crossing the macular region, visual acuity and ophthalmoscopic and fluoroangiographic findings tend to have remained stable at long-term follow-up.