Approach Considerations
Options for treatment of unruptured intracranial AVMs include observation alone, radiation therapy with radiosurgery, endovascular embolization, surgical resection, or, in many cases, some combination of therapies. The risk for hemorrhage or other potentially life-threatening events from the AVM should be balanced against the risk of the specific treatments, the size, feeder, and draining pattern; the location of the lesion; and the expertise available at the individual institution.
Medical Care
Unlike intracranial AVMs, retinal AVMs do not usually bleed but can produce vitreous or retinal hemorrhages. Some patients are at a higher risk for loss of visual function through various mechanisms (eg, retinal vascular occlusions, retinal ischemia, retinal detachment, neovascular glaucoma).
Because of the stability of the retinal lesions, management by an ophthalmologist often is unnecessary in patients with Wyburn-Mason syndrome (WMS) beyond diagnosing the condition, obtaining intracranial imaging, organizing the appropriate systemic referrals, and performing routine and periodic ophthalmic examinations. Symptomatic medical treatment can be offered for neovascular glaucoma. [24, 25] However, recent reports have described success with intravitreal ranibizumab in the treatment of Wyburn-Mason syndrome with retinal artery macroaneurysm and exudation and intravitreal bevacizumab in the treatment of bilateral racemose hemangioma and retinal detachment. [26, 27]
Likewise, depending on individual findings, size, location, and course, many patients with cerebral AVM have been safely observed for long periods. Spontaneous resolution of these vascular lesions has been reported.
Surgical Care
Scatter retinal photocoagulation may be indicated in instances of retinal ischemia following venous occlusive events. Likewise, pars plana vitrectomy for nonclearing vitreous hemorrhage and cyclodestructive procedures for neovascular glaucoma may be recommended.
As noted above, some intracranial AVMs may require multimodal therapy, including primary or adjunctive surgery.
Consultations
Referral for neurologic evaluation is indicated when retinal AVMs are diagnosed.
Retinal consultation may be needed for treatment of intraocular lesions.
Neurologic, dermatologic, neurosurgical, radiation therapy, or neurointerventional consultations may be necessary.
Long-Term Monitoring
Patients should be followed for progression clinically for intracranial and intraocular disease (vitreous hemorrhage, serous retinal detachment, cystoid macular edema, venous occlusions, neovascular glaucoma, neovascularization, choroidal infarction, and recently reported peripheral retinal ischemia), as well as radiographically for intracranial AVMs. Continued serial and possibly lifelong observation of patients with Wyburn-Mason syndrome is recommended. [28, 29]
Further Inpatient Care
Patients may require inpatient evaluation and treatment for intracranial lesions.
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The dilated and abnormal retinal vasculature characteristic of a retinal arteriovenous malformation.
