Details

Autor(en) / Beteiligte

• Handa, Swati
• Chew, Rebecca Y K
• Eong, Kah-Guan A U

Titel

Photo Quiz: Convoluted Vessels in the Eye

Ist Teil von

• American family physician, 2008-10, Vol.78 (8), p.981

Ort / Verlag

Leawood: American Academy of Family Physicians

Erscheinungsjahr

2008

Quelle

EZB Electronic Journals Library

Beschreibungen/Notizen

• A congenital vascular abnormality in which AVMs of the visual pathway, face, and CNS coexist is called Wyburn-Mason syndrome; however, involvement of all three systems is not required for the diagnosis.5,6 Visceral and skeletal anomalies have also been reported in patients with Wyburn-Mason syndrome.1,2 Ophthalmic manifestations such as the characteristic retinal picture of dilated, tortuous, and convoluted AVMs; visual impairment; and proptosis may suggest the presence of the syndrome. Visual impairment in patients with retinal AVMs is usually caused by cystic degeneration in the retina, macular edema, macular hemorrhage, retinal venous occlusive disease, retinal ischemia, neovascular glaucoma from the isolated retinal AVM,2,7 or AVMs in the visual pathway.5 AVMs typically occur in the midbrain, and neurologic manifestations (e.g., cerebral or subarachnoid hemorrhage) depend on the size and location of the malformations.