Barbara Delas, Eva Villota, Alvaro Fernandez-Vega, Jeroni Nadal (Barcelona, Spain)


Racemose hemangiomas of the retina are rare developmental anomalies. Although visual prognosis is usually good because of their clinical stability, the development of retinal vascular complications should be taken into account when planning follow-up, especially in children.


We describe two cases of venous thrombosis secondary to unilateral racemose angioma. Case 1. A 6-year-old healthy boy consulted for a decrease in vision of the left eye. Best-corrected visual acuities (BCVA) were 20/30. The left fundus revealed massive dilation and tortuosity of retinal vessels in a random pattern. Retinal arteries could not be differentiated from the retinal veins. 24 months later, BCVA decreased to 20/200 and exploration evidenced an iris rubeosis with central retinal vein occlusion. Pan-retinal photocoagulation was performed and iris neovascularization regressed without neovascular glaucoma. When last seen, visual acuity was 20/200, the fundus examination revealed a cystoid macular edema and the intraocular pressure was normal. Case 2. A 12-year-old girl consulted for progressive loss of visual acuity in the left eye. The BCVA was 20/200, and the examination revealed venous thrombosis in the superior temporal vein area in the context of a massive arteriovenous malformation. Treatment with intravitreal triamcinolone (4mg) resulted in anatomic and functional improvement.

Effectiveness / Safety:

Because of risk of vascular complications we should follow congenital arteriovenous communications closely. Standard care of central vein obstruction and branch retinal vein obstruction could keep these patients free of secondary complications but requires close monitoring to detect any retinal or iris neovascularization.

Take home message:

To increase our awareness of venous obstruction associated with arteriovenous communication. Immediate recognition is crucial in order to avoid potential serious complications, such as retinal and iris neovascularization.