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We report a rare case of congenital retinal macrovessel with decreased visual acuity, with a 14-year follow-up. Both the clinical findings and the visual acuity remained unchanged throughout the follow-up period.

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.

AN 18- YEAR-OLD healthy white man reported a sudden decrease in central visual acuity in his right eye after physical exertion, probably combined with a Valsalva maneuver, while repairing his car. He did not report other symptoms and was not taking systemic medications. Best-corrected visual acuity was 20/600 OD and 20/20 OS. Anterior segment examination and intraocular pressures were normal. Ophthalmoscopic examination of the right eye disclosed the presence of an anomalous retinal vein extending along the papillomacular bundle through the foveal avascular zone and temporally. A preretinal hemorrhage overlaid the fovea and partially obscured the underlying a nomalous r etinal v ein (Figure 1). Fluorescein angiography of the right eye demonstrated blocked fluorescence from this preretinal hemorrhage (Figure 2). No evidence of choroidal neovascularization was observed. On follow-up examination 5 weeks later, the visual acuity had improved to 20/20 OD. The preretinal hemorrhage had completely resorbed, allowing examination of the underlying retinal vasculature. Fluorescein angiography at this time disclosed the anomalous vein crossing over the foveal avascular zone, and the vein appeared normal on angiographic examination (Figure 3 and Figure 4). There is no evidence of an arteriovenous anastomosis or late leakage.

Four cases of macular ectopia are described. Pseudostrabismus, caused by an increased angle alpha, is only one of the signs common to macular ectopia. A correct diagnosis is of fundamental importance because the treatment of macular ectopia is different from that of strabismus.

The authors describe a case of congenital retinal macrovessel with decreased visual acuity. Ophthalmoscopy and fluorescein angiography showed the malformation to be congenital and benign.