Arterio-venous communications of the retina are rare congenital anomalies that are direct communications between artery and vein without intervening capillary bed. Retinal AVM has a widely variable spectrum of presentation. Retinal arteriovenous malformation (AVM) is known to be associated with intracranial AVM. Retinal AVM may be associated with central and branch retinal vein occlusion, neovascular glaucoma, optic atrophy, retinal exudation, and vitreous hemorrhage. Racemose aneurysm of the retina is the rarest, which may be associated with similar AVM in the ipsilateral part of the brain. Coexistence of AVM in the retina and the brain is considered to be one of the phakomatosis. A 27 year old male presented with hemiparesis, conjunctival telangiectasia and retinal AVM. Fundus examination showed convoluted, dilated, tortuous retinal vessels extending from the optic disc to the periphery of retina in left eye. Massively dilated vessels were noted in the superotemporal and inferotemporal quadrants. Computed Tomography of brain revealed linear hypodense area with gliosis in the left parietotemporal region. MRI showed evidence of AVM with multiple tiny tortuous feeder vessels from the left middle cerebral artery, forming a sparse nidus within the gliotic area. Hemiplegia occurs secondary to subarachnoid or intracranial hemorrhage, caused by the AVM. It is imperative that a detailed fundus examination should be done in any case of conjunctival telangiectasia. Imaging of the brain is advisable in a case of retinal AVM to rule out cerebral AVM. Retinal AVM may result in visual loss and requires vigilant follow up.
Keywords: Conjunctival telangiectasia, Intracranial AVM, Retinal arteriovenous malformation (AVM)
