-Also known as Bonnet-Bechaume-Blanc syndrome, Congenital Unilateral Retinocephalic Vascular Malformation Syndrome, Racemose Angiomatosis.
-Non-hereditary congenital neurocutaneous disorder leading to arteriovenous malformations of the brain, orbit, retina and skin.
-Caused by an abnormality in the development of blood vessels during embryonic or fetal growth.
DIAGNOSIS:
-Fundus Exam: Unilateral tortuous dilated retinal vessels.
-FFA: Rapid filling of vascular anomalies without significant leakage.
-OCT: enlarged thickened retinal vessels and retinal edema.
-MRI: indicates the location, size, mass effect, edema of intracranial arteriovenous malformations
-Cerebral angiography: displays the characteristics of feeding arteries and draining veins, indicates the exact architecture of the anomaly
OCULAR MANIFESTATIONS:
-Proptosis
-Blepharoptosis
-Abnormally dilated vessels of the conjunctiva
-Nerve palsies
-Nystagmus
-Strabismus
-Decreased visual acuity or total blindness (retinal ischemia, intraocular lesions)
-Vitreous Hemorrhage
-Vein occlusions
-Retinal Detachment
-Neovascular Glaucoma
-Rubeosis Iridis
-Optic Disc Edema
-Optic Atrophy
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