Orbital exenteration after transarterial embolization in a patient with Wyburn-Mason syndrome: pathological findings.
BACKGROUND
We present the pathological findings at orbital exenteration in a patient with Wyburn-Mason syndrome who underwent transarterial embolization.


METHODS
A 31-year-old man with a 10-year history of gradual exacerbation of left exophthalmos and left cheek swelling was found to have facial and orbital arteriovenous malformations on the left side. There was no vascular malformation in the brain. The feeding arteries derived from the left internal maxillary artery, facial artery, and ophthalmic artery. He underwent several courses of transarterial embolization of the feeding arteries from the left internal maxillary artery and then from the facial artery, resulting in no reduction of the arteriovenous malformation. He finally elected to undergo ophthalmic artery embolization in the expectation of a reduction and with the understanding that he would lose sight in his left eye. Two years later, he requested lid-sparing orbital exenteration and reconstruction with cutaneous flap transfer and prosthesis for cosmetic reasons.


METHODS
Pathologically, orbital vascular channels of varying sizes were filled with embolizing glue and had degenerating vascular wall cells surrounded by inflammatory cell infiltration. The central retinal artery in the optic nerve was also filled with the embolizing glue, and the retina lost the ganglion cell layer and inner nuclear layer but maintained the outer nuclear layer and outer segments.


CONCLUSIONS
Marked anastomoses and hence incomplete embolization among the feeding arteries of facial and orbital vascular malformations in Wyburn-Mason syndrome do not respond well to attempts at feeding vessel embolization, which result in unsuccessful closure of the malformation.
Matsuo
Toshihiko
T
Department of Ophthalmology, Okayama University Graduate School of Medicine Dentistry, and Pharmaceutical Sciences, Okayama, Japan. matsuot@cc.okayama-u.ac.jp.
Yanai
Hiroyuki
H
Pathology, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, Okayama, Japan.
Sugiu
Kenji
K
Neurological Surgery, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, Okayama, Japan.
Tominaga
Susumu
S
Otolaryngology/Head & Neck Surgery, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, Okayama, Japan.
Kimata
Yoshihiro
Y
Plastic/Reconstructive Surgery, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, Okayama, Japan.
D000328:Adult; D001165:Arteriovenous Malformations / Q000473:pathology* / Q000601:surgery / Q000628:therapy*; D002342:Carotid Artery, External / Q000000981:diagnostic imaging / Q000503:physiopathology; D002343:Carotid Artery, Internal / Q000000981:diagnostic imaging / Q000503:physiopathology; D004621:Embolization, Therapeutic; D004659:Enbucrilate / Q000627:therapeutic use; D005094:Exophthalmos / Q000209:etiology / Q000601:surgery; D005145:Face / Q000098:blood supply*; D005451:Fluorescein Angiography; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008438:Maxillary Artery / Q000002:abnormalities*; D009880:Ophthalmic Artery / Q000002:abnormalities*; D009915:Orbit / Q000601:surgery; D017233:Orbit Evisceration; D011859:Radiography; D016634:Radiosurgery; D013524:Surgical Flaps; D013577:Syndrome
D004659:Enbucrilate
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