[Wyburn-Mason syndrome].
BACKGROUND
There are various malformations of retinal vessels. Some of them are associated with cerebral vascular anomalies.


METHODS
This report is given on a girl 6 years of age with a retinal racemose angioma.


RESULTS
The clinical examination showed a unilateral racemose angioma combined with retinachorioidal anastomosis and a hemifacial vascular malformation. We found teleangiectasia in this area. The ophthalmoscopy demonstrated a blurred border of optic disc on the right eye with forward protrusion of the disc about + 2.0 dioptres. Large racemose lesions may be localized to the optic nerve and may involve segmental areas of the macula. The ultrasound showed a papillary stasis with highly reflective structures. The ultrasonic patterns are interpreted as a cholesterosis or fibrovascular tissue. This findings may belong to the rare Wyburn-Mason syndrome. Acute neurological symptoms by similar lesions the midbrain and hemorrhages during extraction of teeth are reported in literature.
Tost
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F
Klinik und Poliklinik, Augenkrankheiten der Martin-Luther-Universität Halle-Wittenberg.
Weidlich
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Tost
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D002648:Child; D003937:Diagnosis, Differential; D005134:Eye Neoplasms / Q000175:diagnosis / Q000235:genetics*; D005260:Female; D006391:Hemangioma / Q000175:diagnosis / Q000235:genetics*; D006801:Humans; D002538:Intracranial Arteriovenous Malformations / Q000175:diagnosis / Q000235:genetics*; D009887:Ophthalmoscopy; D012171:Retinal Vessels / Q000002:abnormalities*; D013577:Syndrome
Zum Wyburn-Mason-Syndrom.