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. CASE REPORT: 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. CONCLUSION: 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.
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. CASE REPORT: 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. CONCLUSION: 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.