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

Abstract

BackgroudRetinal 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 presentationOne 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.ConclusionsAlthough RRH is a relatively non-progressive condition, its complications may lead to vision loss and should be treated in time.

Retinal racemose hemangioma with retinal artery macroaneurysm: Optical coherence tomography angiography (OCTA) findings

“…The cause for loss of vision is due to late ocular complications like retinal vein occlusion (45%), hemorrage (33%), macular edema or rare congenital vascular malformations like morning glory disc anomaly, Sturge-Weber syndrome and macroaneurysm. 1 …”

“…Congenital arteriovenous communications or retinal racemose hemangioma (RRH) is a rare, unilateral, non hereditary and sporadic phacomatosis characterized by the appearance of dilated and tortuous retinal vessels frequently extending from the optic disc to the retinal periphery. 1 Archer et al 2 have divided these AV communications into 3 groups depending on the size and location of vascular malformation. The first group is characterized by abnormal capillary plexus between the major vessels of the AV malformations and is usually asymptomatic.…”

Binarization of enhanced depth imaging optical coherence tomographic images of an eye with Wyburn-Mason syndrome: a case report

“…As in our patient, the large retinal AVMs in Wyburn-Mason syndrome usually cause cystic retinal degeneration [ 4 , 7 - 9 ]. Onder et al [ 10 ] reported a patient with Wyburn-Mason syndrome who developed serous retinal detachment and cystoid macular edema.…”

Optical coherence tomography angiography of fleeting macroaneurysm in retinal racemose angioma

“…[ 4 ] This has been attributed to hyperdynamic flow through small caliber vessels and steal phenomenon caused by increased venous pressure and decreased arterial pressure. [ 5 ]…”

“…They reviewed 167 cases of racemose angioma, among which only one case had MA. [ 5 ] There is a single case report by Tilanus et al . demonstrating fleeting MA in a case of racemose angioma over a course of 4 years.…”