Wyburn-Mason Syndrome

• 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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Image from Rajan Eye Care Hospital

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