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Backgroud  Retinal racemose hemangioma (RRH) is a rare congenital disorder that often co-occurs with other ocular complications. In this study, we present a case of RRH complicated with retinal vein obstruction in three branches and provide a review of ocular complications and associations with RRH.    Case presentation  One case of RRH is presented. Fundus examination, fluorescein angiography (FFA) and optical coherence tomography (OCT) of the patient identified Group 3 RRH complicated with retinal vein occlusions in the superotemporal, inferotemporal, and inferonasal branches. Macular edema, which causes visual impairment, was detected. A brief literature review was also presented. The PubMed database was searched for RRH or related keywords to find reports of ocular complications or associations published on or before Dec. 31, 2013. A total of 140 papers describing167 RRH cases were found. The mean age of diagnosis was 22.97 years. Ocular complications were mentioned in 32 (19.16%) cases. Retinal vein occlusion (46.88%) was the major ocular complication in RRH, followed by hemorrhage (34.38%). Eight (4.79%) cases were associated with other ocular diseases such as Sturge–Weber syndrome , Morning glory disc anomaly and macroaneurysm.    Conclusions  Although RRH is a relatively non-progressive condition, its complications may lead to vision loss and should be treated in time.

bmc ophthalmology

Retinal vein occlusion in retinal racemose hemangioma: a case report and literature review of ocular complications in this rare retinal vascular disorder