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Wyburn-Mason Syndrome Treatment & Management

  • Author: Andrew G Lee, MD; Chief Editor: Hampton Roy, Sr, MD  more...
 
Updated: Apr 09, 2015
 

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 of 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.

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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 is often 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.[19, 20]

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 also been reported.

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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.

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Consultations

Referral for neurologic evaluation is indicated when retinal AVMs are diagnosed.

Retinal consultation may be needed for treatment of intraocular lesions.

Neurosurgical, radiation therapy, or neurointerventional consultations may be necessary.

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Contributor Information and Disclosures
Author

Andrew G Lee, MD Chair, Department of Ophthalmology, Houston Methodist Hospital; Clinical Professor, Associate Program Director, Department of Ophthalmology and Visual Sciences, The University of Texas Medical Branch; Clinical Professor, Department of Surgery, Division of Head and Neck Surgery, University of Texas MD Anderson Cancer Center; Professor of Ophthalmology, Neurology, and Neurological Surgery, Weill Medical College of Cornell University; Clinical Associate Professor, University of Buffalo, State University of New York School of Medicine

Andrew G Lee, MD is a member of the following medical societies: American Academy of Ophthalmology, Association of University Professors of Ophthalmology, American Geriatrics Society, Houston Neurological Society, Houston Ophthalmological Society, International Council of Ophthalmology, North American Neuro-Ophthalmology Society, Pan-American Association of Ophthalmology, Texas Ophthalmological Association

Disclosure: Received ownership interest from Credential Protection for other.

Coauthor(s)

Nagham Al-Zubidi, MD Fellow in Neuro-ophthalmology, Department of Ophthalmology, The Methodist Hospital, Weill Cornell Medical College

Disclosure: Nothing to disclose.

Specialty Editor Board

Simon K Law, MD, PharmD Clinical Professor of Health Sciences, Department of Ophthalmology, Jules Stein Eye Institute, University of California, Los Angeles, David Geffen School of Medicine

Simon K Law, MD, PharmD is a member of the following medical societies: American Academy of Ophthalmology, Association for Research in Vision and Ophthalmology, American Glaucoma Society

Disclosure: Nothing to disclose.

J James Rowsey, MD Former Director of Corneal Services, St Luke's Cataract and Laser Institute

J James Rowsey, MD is a member of the following medical societies: American Academy of Ophthalmology, American Association for the Advancement of Science, American Medical Association, Association for Research in Vision and Ophthalmology, Florida Medical Association, Sigma Xi, Southern Medical Association, Pan-American Association of Ophthalmology

Disclosure: Nothing to disclose.

Chief Editor

Hampton Roy, Sr, MD Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences

Hampton Roy, Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, Pan-American Association of Ophthalmology

Disclosure: Nothing to disclose.

Additional Contributors

Gerhard W Cibis, MD Clinical Professor, Director of Pediatric Ophthalmology Service, Department of Ophthalmology, University of Kansas School of Medicine

Gerhard W Cibis, MD is a member of the following medical societies: American Academy of Ophthalmology, American Association for Pediatric Ophthalmology and Strabismus, American Ophthalmological Society

Disclosure: Nothing to disclose.

Acknowledgements

Ann E Bidwell, MD Assistant Professor, Department of Ophthalmology, Northwestern University, Feinberg School of Medicine

Ann E Bidwell, MD is a member of the following medical societies: American Academy of Ophthalmology

Disclosure: Nothing to disclose.

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Dilated arteriovenous system representing a well-compensated unilateral retinal arteriovenous malformation. This 12-year-old girl had 20/20 vision and a negative systemic evaluation.
 
 
 
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