Sturge-Weber Syndrome Treatment & Management

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

Approach Considerations

For small degrees of anisometropia, full optical correction of both eyes or at least full correction of the refractive difference between the eyes is desirable. In higher degrees of anisometropia or if the child develops strabismus, treatment to prevent amblyopia and to treat strabismus should be initiated. Anisometropic amblyopia may require occlusion therapy along with correction of the refractive error. In some patients, a contact lens may be required to treat fusion difficulty due to aniseikonia.

Medical treatment of glaucoma in Sturge-Weber syndrome usually fails with time. Most ophthalmologists consider surgical therapy to be the mainstay of glaucoma therapy in patients with Sturge-Weber syndrome.[5]

Next

Pharmacologic Treatment of Glaucoma

Although, as stated, medical treatment of Sturge-Weber syndrome glaucoma usually fails with time, it may be tried initially. This is because a significant reduction in intraocular pressure may be seen, at least temporarily, and may be helpful in clearing the cornea, thus facilitating surgical therapy. Moreover, it can be used to delay filtration surgery in younger patients. This is especially important, because the technical difficulties of operating on a smaller eye are excessive, and there is an increased tendency for scarring at the site of the scleral flap in the younger patient, reducing the chances for long-term surgical success.

Medical therapy can also be used as an adjunct to surgery. Topical antiglaucoma therapy for extended periods of time is sometimes helpful postoperatively to further reduce borderline intraocular pressure elevations without the need for reoperation. Initial medical therapy with a topical beta blocker, followed sequentially with the addition of a carbonic anhydrase inhibitor (systemic in infants and topical in older children) and topical prostaglandin (latanoprost [Xalatan]), is a reasonable protocol in patients with Sturge-Weber syndrome.

Previous
Next

Radiation Treatment of Diffuse Choroidal Hemangiomas

A few patients with diffuse choroidal hemangiomas associated with a bullous, nonrhegmatogenous retinal detachment have been treated with radiation therapy, such as brachytherapy or external beam irradiation. Preliminary reports of such therapy suggest that radiation therapy may be a reasonable alternative to photocoagulation, the currently preferred therapy, in selected patients. However, the ultimate risk-to-benefit ratio for this form of therapy is still unknown. Furthermore, the precise indications and contraindications for such treatment currently are unknown.

Previous
Next

Glaucoma Surgery

As previously stated, most ophthalmologists consider surgical therapy to be the mainstay of glaucoma therapy in patients with Sturge-Weber syndrome,[5] with antiglaucoma medications primarily useful as treatment adjuncts. However, the selection of surgical technique remains controversial.[6] Goniotomy, trabeculotomy, trabeculectomy, combined trabeculotomy-trabeculectomy, argon laser trabeculoplasty, neodymium:yttrium-aluminum-garnet (Nd:YAG) laser goniotomy, and seton procedures have been used in patients with Sturge-Weber syndrome glaucoma. However, the long-term results are often disappointing with any of these procedures as they are associated with a high failure rate compared with the same procedure performed for primary infantile glaucoma.

Because Sturge-Weber–associated glaucoma is relatively rare, no controlled series of cases comparing one intervention to another have been published, nor are standard treatment guidelines available. The objective of therapy is rapid and permanent lowering of the intraocular pressure into the normal range (generally < 20 mm Hg) or to a level slightly higher but without progression of other signs, such as corneal enlargement, increased myopia, or increased optic nerve cupping.

The anesthesiologist should be aware that the patient has Sturge-Weber syndrome, because the presence of a spinal cord or brain hemangioma may increase the risk of intracerebral bleeding or disseminated intravascular coagulation with anesthesia. In addition, an anesthesia protocol should be planned to prevent the development of hypertension, which could result in hemorrhage.

Goniotomy or trabeculotomy is believed by some to be the treatment of choice in early onset glaucoma in infancy when the probable mechanism for pressure elevation is an abnormal outflow angle. These procedures are often unsuccessful in infants with Sturge-Weber syndrome or are successful only after being repeated several times and with the addition of adjunctive medical therapy. Nevertheless, some authors prefer to perform these procedures initially, because they are sometimes successful and goniotomy also is thought to be less likely to cause complications (especially expulsive choroidal hemorrhage or choroidal effusion) that are associated with a precipitous drop in intraocular pressure.

With glaucoma onset in the older age group, when the outflow angle appears clinically normal, glaucoma filtering surgery, either full thickness or partial thickness (trabeculectomy), is more likely to be successful, because it bypasses any component of the glaucoma possibly caused by elevated episcleral venous pressure. Combined trabeculotomy-trabeculectomy may be a reasonable compromise in the older patient with Sturge-Weber syndrome in view of the possible combination of angle abnormality and raised episcleral pressure in causing the glaucoma.

Adjunctive antimetabolites used in conjunction with a filtering surgery may create a more satisfactory degree of intraocular pressure control in this patient population, by slowing wound healing and scar formation. The most commonly used clinical agents are 5-fluorouracil (5-FU) and mitomycin-C. 5-FU usually is given as a series of subconjunctival injections postoperatively. Mitomycin-C is usually applied intraoperatively, using a sponge saturated with mitomycin solution.

Postoperative subconjunctival injections usually are impossible in very young patients; thus, intraoperative application of mitomycin-C most frequently is required in these patients. Mitomycin-C and 5-FU are associated with thinner, more cystic blebs and may carry a higher rate of complications, such as wound leaks, chronic hypotony, and, possibly, late endophthalmitis.

Corticosteroids should be used after filtration surgery to diminish postoperative inflammation and scarring of the bleb. A sub-Tenon injection of a short-acting corticosteroid (eg, dexamethasone, triamcinolone) at the completion of surgery and the use of topical corticosteroid drops or ointment after surgery are recommended.

Cyclocryotherapy is difficult to control and has a high complication rate. Therefore, it should be used only when all other procedures have failed or are not feasible, to save useful vision or to prevent or relieve severe pain. New types of cyclodestructive procedures, such as Nd:YAG transscleral laser and therapeutic ultrasonic cyclodestructive procedures, have had only limited trial in pediatric and Sturge-Weber syndrome glaucoma, and their potential for long-term success, as well as for complications, are not fully understood in the young patient.

Seton devices also are being used when routine filtering surgery has failed. Encouraging initial results have been reported using various posterior tube shunt implant devices, but long-term follow-up results are not yet available.

Nd:YAG laser goniotomy and argon laser trabeculoplasty have been used to a limited extent in pediatric glaucoma, and favorable results in some patients with Sturge-Weber syndrome have been reported.

Previous
Next

Additional Surgical Treatments

Any significant strabismus that is still present after the completion of amblyopia therapy, refractive lens correction, and orthoptics is treated best with eye muscle surgery. Avoiding or carefully cauterizing the dilated subconjunctival and episcleral vessels during strabismus surgery is important to prevent bleeding and scarring, so that the conjunctiva and anterior sclera are preserved for future glaucoma procedures.

Unfortunately, no surgical or medical treatment has been shown to be very effective in preventing or reversing the visual deterioration associated with the secondary changes of ocular structures overlying the diffuse choroidal hemangioma.

Management of affected eyes emphasizes the reduction of subretinal fluid as the main therapeutic aim in an attempt to stabilize or reverse, if possible, visual impairment caused by nonrhegmatogenous retinal detachment. However, no reliable treatment of the retinal detachment that develops in these patients has been found, and, even in the exceptional case in which the retina can be reattached, fibrous metaplasia of the retinal pigment epithelium and cystoid degeneration of the retina overlying the choroidal hemangioma prevent good visual result. Many such eyes eventually become blind and painful and must be enucleated.

Attempts at repairing the nonrhegmatogenous retinal detachment variously involve cryotherapy and diathermy, xenon arc or argon laser photocoagulation, subretinal fluid drainage, and radiation therapy. A critical factor in a successful outcome appears to be the early initiation of treatment.

Laser photocoagulation is generally considered to be the preferred therapeutic intervention. Placement of light photocoagulation scars over the entire tumor is completed in an attempt to strengthen the adhesion between the retina and the underlying pigment epithelium and, thus, to prevent the spread of the retinal detachment. This form of treatment has afforded limited success. However, retinal detachment often recurs even after photocoagulation therapy, and, in some patients, complete reattachment of the retina is not possible. Furthermore, treatment success with large hemangiomas, as well as with diffuse, infiltrating tumors of the macula, is limited.

External drainage of subretinal fluid with or without scleral buckling in conjunction with xenon photocoagulation has been used to treat diffuse choroidal hemangiomas associated with large, exudative retinal detachments in Sturge-Weber syndrome.

Pars plana vitrectomy, endolaser, and internal drainage of subretinal fluid can be performed. Cryotherapy and penetrating diathermy are of limited use because of the posterior location of the tumor.

Treatment of the cutaneous port-wine stain with dye laser photocoagulation has been helpful in reducing the cosmetic blemish from the cutaneous vascular dilatation.[7]

Previous
Next

Postoperative Care and Long-Term Monitoring

Postoperative care frequently requires repeated examination under anesthesia in infants and young children to assess surgical success. If continued borderline intraocular pressure elevation is found, then a trial of adjunctive medical therapy with close follow-up care may be continued safely as long as no evidence of progression of glaucoma damage is observed. However, if intraocular pressure remains clearly elevated or evidence for progressive glaucomatous damage is detected, then repeat glaucoma surgery should be performed.

All patients with Sturge-Weber syndrome (SWS) must have regular ophthalmologic examinations for life, even when no ocular abnormalities are detected initially, to avoid later loss of vision secondary to late-onset glaucoma.

Previous
Proceed to Medication
 
 
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].

Previous
Next
 
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.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.