J Cerebrovasc Endovasc Neurosurg.  2013 Sep;15(3):251-254. 10.7461/jcen.2013.15.3.251.

Intraosseous Arteriovenous Malformation of the Sphenoid Bone Presenting with Orbital Symptoms Mimicking Cavernous Sinus Dural Arteriovenous Fistula: A Case Report

• Park ES
• Jung YJ
• Yun JH
• Ahn JS
• Lee DH

• KMID: 1491448
• DOI: http://doi.org/10.7461/jcen.2013.15.3.251

Abstract

Intraosseous arteriovenous malformation (AVM) in the craniofacial region is rare. When it occurs, it is predominantly located in the mandible and maxilla. We encountered a 43-year-old woman with Klippel-Trenaunay syndrome affecting the right lower extremity who presented with a left orbital chemosis and proptosis mimicking the cavernous sinus dural arteriovenous fistula. Computed tomography angiography revealed an intraosseous AVM of the sphenoid bone. The patient's symptoms were completely relieved after embolization with Onyx. We report an extremely rare case of intraosseous AVM involving the sphenoid bone, associated with Klippel-Trenaunay syndrome.

Keyword

Arteriovenous malformations; Hemangioma; Primary intraosseous vascular malformation; Klippel-Trenaunay-Weber syndrome

MeSH Terms

Adult
Angiography
Arteriovenous Malformations
Cavernous Sinus
Caves
Central Nervous System Vascular Malformations
Exophthalmos
Female
Hemangioma
Humans
Klippel-Trenaunay-Weber Syndrome
Lower Extremity
Mandible
Maxilla
Orbit
Sphenoid Bone

Figure

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  Fig. 1 Photography shows the hypertrophy of our patient's right lower extremity, with bluish cutaneous stains.

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  Fig. 2 (A, B) Left external carotid angiogram shows the arteriovenous malformation (AVM) fed by the internal maxillary and accessory meningeal arteries. (C) Left accessory meningeal artery injection demonstrates a high flow AVM. (D) The nidus is drained via the ipsilateral cavernous sinus with significant reflux into the ipsilateral superior and inferior ophthalmic veins while the inferior petrosal sinus is patent.

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  Fig. 3 (A) Brain magnetic resonance image with T2-weighted coronal image shows that the vascular mass corresponded with the dark-signaled mass (Double arrowheads) in the left side of the sphenoid body and the medial aspect of the greater wing. (B, C) Contrast-enhanced brain computed tomography shows a vascular abnormality localized in the sphenoid bone near the left paracavernous region. The abnormal vascular lesion (Arrow) is mainly located in sphenoid bone. The draining vein (Arrowhead) is located in the paracavernous region.

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  Fig. 4 Three-month follow-up angiogram shows complete obliteration of the AVM. (A) Scout image. (B) Left external carotid angiogram, lateral view of arterial phase. (C) Left external carotid angiogram, anteroposterior view of arterial phase.

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