The Wyburn-Mason or Bonnet-Dechaume-Blanc syndrome is a sporadic illness which is a type of phakomatosis, usually present as unilateral arteriovenous malformations(AVM), for the first defined by Magnus in 1874 as retinal AVM later in 1932 another description gave by Yates and Payne; as an AVMs of the retina and cerebral vasculature, while in 1937 Bonnet, Dechaume and Blanc defined as AVM involving facial, retinal and brain blood vessels, then in 1943 all literature revised by Wyburn-Mason to put together to his case reports, therefore, the name of Bonnet-Dechaume-Blanc syndrome given in France, but in English articles entitled as Wyburn-Mason syndrome. Due to a lack of diagnostic imaging techniques at that time, all diagnosis was made by clinical findings, operation or autopsy. It is usually a unilateral disorder that involves three components: the orbit, brain (ipsilaterally to the retina), and the face. The sensory region of trigeminal nerves involved takes the form of naevi, which can be fully formed or absent. However, this latter is often a partial disease, but it rarely causes bilateral involvement with an asymmetrical malformation. The pathology begins in the embryonic period. Vascular dysgenesis can cause a variety of neurocutaneous vascular defects, including those in the cerebrum, ocular, or both. The AVM can cause optic nerve compression, which results in impaired perfusion and eventually optic atrophy. Glaucoma can also be caused by elevated blood pressure or neovascularization due to ischemia. This could lead to vitreous hemorhage(5). Archer et al. Archer et al. Group 2 (Clinically, hemorhage and edema due to direct AVM. There is no capillary network between them. Group 3 (Clinically it is difficult to distinguish veins from arteries due to the severely dilapidated blood vessels all around retina). A 41-year-old male presents with gradual decline in visual acuity in both his eyes.
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