Retinal Racemose Hemangioma With Optic Neuropathy in a Child

The aim of this study is to report an unusual case of retinal racemose haemangioma (RRH) in a child resulting in optic neuropathy and its optical coherence tomography angiography (OCT-A) findings. This is a retrospective case report. For almost a year, a 13-year-old girl experienced gradual, painless, generalized blurred vision in her right eye. Visual acuity was 6/60 with a positive relative afferent pupillary defect (RAPD) in her right eye. The right-eye fundus showed enlarged and tortuous retinal vessels extending from the optic disc to all four quadrants, including the juxta foveal region. OCT analysis revealed distortion in the region of enlarged vessels with minimal retinal fluid while OCT-A of the macula area demonstrated dilated and tortuous vessels in the superficial layers of the retina. Right intra-orbital vascular channels surrounding the optic nerve with optic nerve atrophy and gliosis were detected on magnetic resonance imaging angiography/venography (MRA/MRV). The cerebral angiogram reported an abnormal tangle of small vessels within the right orbit that received supply from a dilated right ophthalmic artery indicating the presence of retro-orbital arteriovenous malformation (AVM). She was then referred to the neurosurgeon and a decision was made not to embolize or resect the dilated vessel as this might lead to occlusion of the ophthalmic artery and thus worsen her vision. RRH may present in the paediatric age group, and optic nerve atrophy is one of the disease manifestations. OCT-A is a less invasive diagnostic option compared to fundus fluorescein angiography (FFA) for diagnosis and monitoring of disease progression.