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  • Rare Presentation of an Intracranial Arteriovenous Malformation with Orbital Venous Congestion

    Final Number:

    Dale Ding MD; Kenneth C. Liu MD

    Study Design:

    Subject Category:

    Meeting: AANS/CNS Cerebrovascular Section 2014 Annual Meeting

    Introduction: Intracranial arteriovenous malformations (AVMs) are uncommon vascular lesions which typically present with hemorrhage or, in the case of unruptured lesions, with seizure, headache, or focal neurological deficit. The importance of venous drainage pattern in AVM natural history and treatment outcomes is well documented. It is exceptionally rare for AVMs to drain into the orbital venous system.

    Methods: We present a case of a 42 year-old male with a, large, complex AVM of the midbrain, basal ganglia, and thalamus which partially drained into the superior ophthalmic vein.

    Results: The patient was referred for treatment with radiosurgery. The resolution of orbital venous congestion symptoms accompanies AVM obliteration. An attempt to relieve orbital venous congestion by endovascular obliteration or surgical location without successful and complete cure of the AVM will most likely destabilize the nidus and predispose it to rupture.

    Conclusions: Serial ophthalmologic monitoring by an experience neuro-ophthalmologist is crucial to monitoring the ophthalmologic effects of AVM progression and treatment.

    Patient Care: AVMs are complex cerebrovascular lesions with may cause symptomatology by arterial steal or venous congestion. Correctly associating the clinical symptoms with angioarchitectural features is crucial to optimizing management of AVM patients.

    Learning Objectives: By the conclusion of this session, participants should be able to 1) Describe the importance of venous drainage patterns of intracranial arteriovenous malformations (AVM), 2) Discuss, in small groups the potential for AVM presentation with orbital venous congestion, and 3) Identify an effective treatment for complex AVMs.

    References: 1. Ding D, Yen CP, Xu Z, et al. Radiosurgery for patients with unruptured intracranial arteriovenous malformations. J Neurosurg. 2013 May;118(5):958-66. 2. Volpe NJ, Sharma MC, Galetta SL, et al. Orbital drainage from cerebral arteriovenous malformations. Neurosurgery. 2000 Apr;46(4):820-4. 3. Mohd-Tahir F, Siti-Raihan I, Wan Hazabbah WH. Arteriovenous malformation in temporal lobe presenting as contralateral ocular symptoms mimicking carotid-cavernous fistula. Case Rep Ophthalmol Med. 2013;2013:158961. 4. Kano H, Kondziolka D, Flickinger JC, et al. Stereotactic radiosurgery for arteriovenous malformations, Part 6: multistaged volumetric management of large arteriovenous malformations. J Neurosurg. 2012 Jan;116(1):54-65.

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