Sturge-Weber Syndrome Medication

  • Author: Monte A Del Monte, MD; Chief Editor: Hampton Roy Sr, MD   more...
 
Updated: Mar 20, 2012
 

Medication Summary

Medical therapy in patients with Sturge-Weber syndrome involves many agents, including beta blockers, carbonic anhydrase inhibitors, and prostaglandin analogues, that can be used to lower the intraocular pressure. Medical therapy is used as an initial treatment, especially in late-onset glaucoma, with surgical therapy initially used in early onset cases. Aqueous suppressants are typically used for initial medical therapy. Prostaglandin analogues may not be as effective in these patients, because the episcleral venous pressure is often elevated by dilated, tortuous episcleral veins. Corticosteroids are used to reduce inflammation.

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Beta Blockers

Class Summary

These agents lower intraocular pressure by decreasing the production of aqueous humor.

Levobunolol 0.25% or 0.5% (Betagan)

 

This is a nonselective beta-adrenergic blocking agent that lowers intraocular pressure by reducing aqueous humor production

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Carbonic Anhydrase Inhibitors

Class Summary

These agents lower intraocular pressure by decreasing aqueous production.

Dorzolamide 2% (Trusopt)

 

Dorzolamide inhibits carbonic anhydrase in the ciliary processes, which decreases aqueous humor formation.

Brinzolamide 1% (Azopt)

 

Brinzolamide inhibits carbonic anhydrase in the ciliary processes, which decreases aqueous humor formation.

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Prostaglandin Analogues

Class Summary

These agents lower intraocular pressure by increasing aqueous outflow through the uveoscleral pathway.

Latanoprost 0.005% (Xalatan)

 

Latanoprost may decrease intraocular pressure by increasing the outflow of aqueous humor.

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Topical Corticosteroids

Class Summary

These medications are used to treat ocular inflammation.

Prednisolone acetate 1% (Pred Forte, Pred Mild, Omnipred)

 

This agent inhibits the edema, fibrin deposition, capillary dilation, and phagocytic migration of the acute inflammatory response, as well as capillary proliferation. It causes the induction of phospholipase A2 inhibitory proteins.

Dexamethasone ophthalmic (Maxidex, Ozurdex)

 

Dexamethasone ophthalmic decreases inflammation by suppressing the migration of polymorphonuclear leukocytes and reducing capillary permeability

Triamcinolone (Triesence)

 

Triamcinolone is used to treat inflammatory reactions that are responsive to steroids. It decreases inflammation by suppressing the migration of polymorphonuclear leukocytes and reversing capillary permeability.

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Antineoplastic Agents

Class Summary

These agents inhibit cell growth and proliferation.

Fluorouracil (Efudex)

 

Fluorouracil interferes with deoxyribonucleic acid (DNA) synthesis by blocking the methylation of deoxyuridylic acid, inhibiting thymidylate synthetase and, subsequently, cell proliferation

Mitomycin

 

Mitomycin interferes with DNA synthesis by alkylation and by cross-linking the strands of DNA.

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Contributor Information and Disclosures
Author

Monte A Del Monte, MD  Skillman Professor of Pediatric Ophthalmology, Professor of Ophthalmology, Pediatrics and Communicable Diseases, Director of Pediatric Ophthalmology and Strabismus, W K Kellogg Eye Center, University of Michigan Medical School

Monte A Del Monte, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Ophthalmology, American Association for Pediatric Ophthalmology and Strabismus, American Medical Association, Association for Research in Vision and Ophthalmology, International Society for Genetic Eye Diseases and Retinoblastoma, Pan-American Association of Ophthalmology, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Coauthor(s)

Michael Taravella, MD  Director of Cornea and Refractive Surgery, Rocky Mountain Lions Eye Institute; Professor, Department of Ophthalmology, University of Colorado School of Medicine

Michael Taravella, MD is a member of the following medical societies: American Academy of Ophthalmology, American Medical Association, American Society of Cataract and Refractive Surgery, Contact Lens Association of Ophthalmologists, and Eye Bank Association of America

Disclosure: AMO/VISX None Consulting

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, and 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, and American Ophthalmological 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, Pan-American Association of Ophthalmology, Sigma Xi, and Southern Medical Association

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

References
  1. [Guideline] American Association of Neuroscience Nurses. Care of the patient with seizures. 2nd ed. Glenview (IL): American Association of Neuroscience Nurses; 2007.

  2. Govori V, Gjikolli B, Ajvazi H, Morina N. Management of patient with Sturge-Weber syndrome: a case report. Cases J. Dec 23 2009;2:9394. [Medline].

  3. Parsa, CF. Sturge-Weber Syndrome:A Unifified Pathophysiologic Mechanism. Curr Treat Options Neurol. 2008;10:47-54. [Medline].

  4. Eibschitz-Tsimhoni M, Lichter PR, Del Monte MA, et al. Assessing the need for posterior sclerotomy at the time of filtering surgery in patients with Sturge-Weber syndrome. Ophthalmology. Jul 2003;110(7):1361-3. [Medline].

  5. [Best Evidence] [Guideline] Patrianakos TD, Nagao K, Walton DS. Surgical management of glaucoma with the sturge weber syndrome. Int Ophthalmol Clin. 2008;48(2):63-78. [Medline].

  6. Audren F, Abitbol O, Dureau P. Non-penetrating deep sclerectomy for glaucoma associated with Sturge-Weber syndrome. Acta Ophthalmol Scand. Oct 2006;84(5):656-60. [Medline].

  7. Sharan S, Swamy B, Taranath DA, et al. Port-wine vascular malformations and glaucoma risk in Sturge-Weber syndrome. J AAPOS. Aug 2009;13(4):374-8. [Medline].

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A child with Sturge-Weber syndrome that primarily affects the distribution of cranial nerve V2-3, with milder involvement of cranial nerve V1. Secondary glaucoma is evident. Ocular melanocytosis involving the sclera of both eyes is an associated finding. Image courtesy of Dr. Lamia Salah Elewa.
Close-up view of the left eye, showing the Ahmed valve implanted in the inferotemporal quadrant after multiple failed filtration procedures induced severe superior conjunctival scarring. Intraocular pressure was controlled. Image courtesy of Dr. Lamia Salah Elewa.
T1-weighted, axial MRI images demonstrate left cerebral hemiatrophy associated with leptomeningeal angiomatosis. Image courtesy of Dr. Lamia Salah Elewa.
Ocular ultrasonogram of the posterior segment demonstrating the diffuse choroidal thickening seen in a diffuse choroidal hemangioma with "tomato-catsup fundus." Image courtesy of Dr. Lamia Salah Elewa.
Choroidal hemangioma. Image courtesy of Thomas M. Aaberg, Jr, MD.
Choroidal hemangioma. Image courtesy of Thomas M. Aaberg, Jr, MD.
Circumscribed hemangioma. Image courtesy of F. Ryan Prall, MD.
Circumscribed hemangioma. Image courtesy of F. Ryan Prall, MD.
B-scan of a choroidal hemangioma showing medium-to-high internal reflectivity. This is a circumscribed choroidal hemangioma. The patient was not diagnosed with Sturge-Weber Syndrome. Image courtesy of Abdhish R Bhavsar, MD.
 
 
 
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