Medscape is available in 5 Language Editions – Choose your Edition here.


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.


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.


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.



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.

Contributor Information and Disclosures

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.


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.


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.

  1. Bonnet P, Dechaume J, Blanc E. L'aneurysme cirsoide de la retine (aneurysme vasemeaux). J Med Lyon. 1937. 18:165-178.

  2. Wyburn-Mason R. Arteriovenous aneurysm of midbrain and retina, facial naevi and mental changes. Brain. 1943. 66:163-203.

  3. Ponce FA, Han PP, Spetzler RF, Canady A, Feiz-Erfan I. Associated arteriovenous malformation of the orbit and brain: a case of Wyburn-Mason syndrome without retinal involvement. Case report. J Neurosurg. 2001 Aug. 95(2):346-9. [Medline].

  4. Liu A, Chen YW, Chang S, Liao YJ. Junctional visual field loss in a case of Wyburn-Mason syndrome. J Neuroophthalmol. 2012 Mar. 32(1):42-4. [Medline].

  5. Schmidt D. [Congenital retinocephalic facial vascular malformation syndrome. Bonnet-Dechaume-Blanc syndrome or Wyburn-Mason syndrome]. Ophthalmologe. 2009 Jan. 106(1):61-8; quiz 69. [Medline].

  6. Matsuo T, Yanai H, Sugiu K, Tominaga S, Kimata Y. Orbital exenteration after transarterial embolization in a patient with Wyburn-Mason syndrome: pathological findings. Jpn J Ophthalmol. 2008 Jul-Aug. 52(4):308-13. [Medline].

  7. Medina FM, Maia OO Jr, Takahashi WY. Rhegmatogenous retinal detachment in Wyburn-Mason syndrome: case report. Arq Bras Oftalmol. 2010 Jan-Feb. 73(1):88-91. [Medline].

  8. Cortnum SO, Sorensen P, Andresen J. Bilateral Wyburn-Mason syndrome presenting as acute subarachnoid haemorrhage - a very rare congenital neurocutaneous [corrected] disorder. Acta Neurochir (Wien). 2008 Jul. 150(7):725-7. [Medline].

  9. Tlucek PS, Moreau A, Siatkowski RM, Repka MX. A twisted mess. Surv Ophthalmol. 2012 Jan-Feb. 57(1):77-82. [Medline].

  10. Achrol AS, Guzman R, Varga M, Adler JR, Steinberg GK, Chang SD. Pathogenesis and radiobiology of brain arteriovenous malformations: implications for risk stratification in natural history and posttreatment course. Neurosurg Focus. 2009 May. 26(5):E9. [Medline].

  11. Vucic D, Kalezic T, Kostic A, Stojkovic M, Risimic D, Stankovic B. Duane type I retraction syndrome associated with Wyburn-Mason syndrome. Ophthalmic Genet. 2012 Jun 14. [Medline].

  12. Archer DB, Deutman A, Ernest JT, Krill AE. Arteriovenous communications of the retina. Am J Ophthalmol. 1973 Feb. 75(2):224-41. [Medline].

  13. Theron J, Newton TH, Hoyt WF. Unilateral retinocephalic vascular malformations. Neuroradiology. 1974. 7(4):185-96. [Medline].

  14. Bech K, Jensen OA. On the frequency of co-existing racemose haemangiomata of the retina and brain. Acta Psychiatr Scand. 1961. 36:47-56. [Medline].

  15. Telander DG, Choi SS, Zawadzki RJ, Berger N, Keltner JL, Werner JS. Microstructural Abnormalities Revealed by High Resolution Imaging Systems in Central Macular Arteriovenous Malformation. Ophthalmic Surg Lasers Imaging. 2010 Mar 9. 1-4. [Medline]. [Full Text].

  16. Turell ME, Singh AD. Vascular tumors of the retina and choroid: diagnosis and treatment. Middle East Afr J Ophthalmol. 2010 Jul. 17(3):191-200. [Medline]. [Full Text].

  17. Rizzo R, Pavone L, Pero G, Chiaromonte I, Curatolo P. A neurocutaneous disorder with a severe course: Wyburn-Mason's syndrome. J Child Neurol. 2004 Nov. 19(11):908-11. [Medline].

  18. Ruggieri M, Konez O, Di Rocco C. Neurocutaneous Disorders Phakomatoses and Hamartoneoplastic Syndromes. New York, NY: Springer; 2008. 345--52.

  19. Shah GK, Shields JA, Lanning RC. Branch retinal vein obstruction secondary to retinal arteriovenous communication. Am J Ophthalmol. 1998 Sep. 126(3):446-8. [Medline].

  20. Effron L, Zakov ZN, Tomsak RL. Neovascular glaucoma as a complication of the Wyburn-Mason syndrome. J Clin Neuroophthalmol. 1985 Jun. 5(2):95-8. [Medline].

  21. Panagiotidis D, Karagiannis D, Tsoumpris I. Spontaneous development of macular ischemia in a case of racemose hemangioma. Clin Ophthalmol. 2011. 5:931-2. [Medline]. [Full Text].

  22. Bloom PA, Laidlaw A, Easty DL. Spontaneous development of retinal ischaemia and rubeosis in eyes with retinal racemose angioma. Br J Ophthalmol. 1993 Feb. 77(2):124-5. [Medline]. [Full Text].

  23. Brodsky MC, Hoyt WF. Spontaneous involution of retinal and intracranial arteriovenous malformation in Bonnet-Dechaume-Blanc syndrome. Br J Ophthalmol. 2002 Mar. 86(3):360-1. [Medline]. [Full Text].

  24. Hartmann A, Mast H, Choi JH, Stapf C, Mohr JP. Treatment of arteriovenous malformations of the brain. Curr Neurol Neurosci Rep. 2007 Jan. 7(1):28-34. [Medline].

  25. Lunsford LD, Kondziolka D, Flickinger JC, Bissonette DJ, Jungreis CA, Maitz AH, et al. Stereotactic radiosurgery for arteriovenous malformations of the brain. J Neurosurg. 1991 Oct. 75(4):512-24. [Medline].

  26. Morgan MK, Johnston IH, de Silva M. Treatment of ophthalmofacial-hypothalamic arteriovenous malformation (Bonnet-Dechaume-Blanc syndrome). Case report. J Neurosurg. 1985 Nov. 63(5):794-6. [Medline].

  27. Pan DH, Guo WY, Chung WY, Shiau CY, Chang YC, Wang LW. Gamma knife radiosurgery as a single treatment modality for large cerebral arteriovenous malformations. J Neurosurg. 2000 Dec. 93 Suppl 3:113-9. [Medline].

  28. Pollock BE, Gorman DA, Coffey RJ. Patient outcomes after arteriovenous malformation radiosurgical management: results based on a 5- to 14-year follow-up study. Neurosurgery. 2003 Jun. 52(6):1291-6; discussion 1296-7. [Medline].

  29. Schmidt D, Pache M, Schumacher M. The congenital unilateral retinocephalic vascular malformation syndrome (bonnet-dechaume-blanc syndrome or wyburn-mason syndrome): review of the literature. Surv Ophthalmol. 2008 May-Jun. 53(3):227-49. [Medline].

  30. Wilkins RH. Natural history of intracranial vascular malformations: a review. Neurosurgery. 1985 Mar. 16(3):421-30. [Medline].

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.
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.