Wyburn-Mason syndrome: a case report.
OBJECTIVE
Wyburn-Mason syndrome is a rare disease associated with multiple arteriovenous malformations of the brain, orbit, and face resulting from an insult occurring during embryonic development.


METHODS
We present the clinical and radiological features of a 47-year-old-man with Wyburn-Mason syndrome who suffered from recurrent bleeding episodes primarily at the nasal corner of the left orbit. After radiotherapy and several angioembolisations, surgical reduction with exenteration of the left orbit was performed and resulted in reduced bleeding. Two years later the patient presented with maxillary sinus empyema. Due to massive endonasal bleeding, endoscopy could not be performed and the maxillary empyema was treated via a transorbital approach.


CONCLUSIONS
Although close observation represents the standard of care in Wyburn-Mason syndrome, patient-specific management decisions are required in the presence of symptoms or complications. In the presented case, surgical intervention proved to be successful.
Wiegand
S
S
Department of Otolaryngology, Head and Neck Surgery, University Hospital Giessen, & Marburg, Campus Marburg, Germany. swiegand@med.uni-marburg.de
Eivazi
B
B
Bien
S
S
Sesterhenn
A M
AM
Teymoortash
A
A
Werner
J A
JA
D001165:Arteriovenous Malformations / Q000150:complications* / Q000628:therapy; D004621:Embolization, Therapeutic / Q000379:methods; D004653:Empyema / Q000000981:diagnostic imaging / Q000601:surgery; D017809:Fatal Outcome; D006470:Hemorrhage / Q000532:radiotherapy; D006801:Humans; D002538:Intracranial Arteriovenous Malformations / Q000150:complications / Q000175:diagnosis / Q000628:therapy; D008279:Magnetic Resonance Imaging; D008297:Male; D008443:Maxillary Sinus / Q000000981:diagnostic imaging / Q000601:surgery; D008875:Middle Aged; D017233:Orbit Evisceration; D009916:Orbital Diseases; D010254:Paranasal Sinus Diseases / Q000000981:diagnostic imaging / Q000601:surgery; D011859:Radiography; D012008:Recurrence; D013577:Syndrome