eMedicine Specialties > Ophthalmology > Phakomatoses

Sturge-Weber Syndrome: Follow-up

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
Coauthor(s): Maya Eibschitz-Tsimhoni, MD, Assistant Professor of Ophthalmology, Pediatric Ophthalmology and Adult Strabismus, Department of Ophthalmology and Visual Sciences, Kellogg Eye Center, University of Michigan Medical Center
Contributor Information and Disclosures

Updated: Feb 15, 2007

Follow-up

Further Outpatient Care

• All patients 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.

Complications

• Surgical management of secondary open-angle glaucoma in patients with SWS, with filtering surgery and Seton procedures, bears an increased risk for a number of surgical complications, the most vision threatening and feared are expulsive choroidal hemorrhage and intraoperative massive choroidal effusion.
• Sudden change in the intraocular pressure gradient when the eye is opened may result in expulsive choroidal hemorrhage from the choroidal hemangioma. Treatment involves rapid closure of all scleral incisions with restoration of intraocular pressure. Transscleral drainage of suprachoroidal blood also may be indicated.
• The intraoperative formation of a massive choroidal effusion without hemorrhage also occurs frequently during filtering surgery in patients with SWS. Increased episcleral venous pressure in these patients is assumed to cause a similar increase in the venous pressure within the ciliary body and choroid.
  • During surgery, when the eye is opened and the intraocular pressure falls, rapid transudation of fluid from the intravascular to the extravascular space results. The extravasation of fluid can be massive enough to cause choroidal detachment instantaneously as well as later serous retinal detachment. Although the mechanism is probably similar to the more commonly seen benign postoperative choroidal detachment, the degree and the speed of fluid extravasation during surgery makes this entity more serious. This intraoperative event can mimic an expulsive suprachoroidal hemorrhage because of rapid fluid accumulation after the commencement of surgery; however, it differs because, if the suprachoroidal space is evacuated, clear, copious amounts of yellow fluid are found and the eye can be decompressed transiently.
  • It seems that once the intraoperative effusion is treated with immediate drainage, the postoperative prognosis becomes excellent despite the persistence of some degree of choroidal and serous retinal detachment.
• Postoperative smaller serous choroidal detachment may develop.
• Serous retinal detachment often occurs in association with choroidal effusion and hypotony. It is possible that a choroidal effusion temporarily interferes with the metabolic transport systems of the retinal pigment epithelium. These serous retinal detachments usually resolve spontaneously as the intraocular pressure normalizes.
• Various preoperative and perioperative prophylactic measures to counteract or prevent these complications have been suggested, including use of hyperosmotics, maximum antiglaucoma therapy preoperatively, prophylactic posterior sclerotomy, prophylactic radiotherapy or laser photocoagulation of the choroidal hemangioma, and electrocautery of the anterior episcleral vascular anomaly.
• Eibschitz-Tsimhoni and colleagues recently demonstrated minimal risk of subchoroidal hemorrhage or effusion in a large case series of patients with SWS undergoing filtration surgery using modern surgical techniques. The authors questioned the need for prophylactic posterior sclerotomy in patients with SWS.
• Suggested steps to minimize the intraoperative and postoperative hypotony are preplacement of scleral flap sutures, injection of a viscoelastic prior to excision of the trabecular meshwork, and tight suturing of the scleral flap with releasable sutures that could be lysed after surgery with argon laser, removed at the slit lamp, or at the time of examination under anesthesia.
• Any recent intraocular surgery predisposes the eye to the risk of bacterial endophthalmitis. Patients with filtering blebs, especially the thin avascular blebs seen with the use of mitomycin-C, are at increased risk to develop bacterial endophthalmitis months and even years after surgery. Because this risk is increased further by contact lens wear, the use of any type of contact lens in these patients is discouraged. Other potential sources of infection include normal conjunctival flora, episodes of bacterial conjunctivitis, and contaminated medicine dropper bottle tips.

Patient Education

• Support group
  • Sturge-Weber Foundation, PO Box 418, Mount Freedom, NJ, 07970-0418
  • Web site: The Sturge-Weber Foundation

Miscellaneous

Medicolegal Pitfalls

• Failure to perform a complete ophthalmologic evaluation to rule out glaucoma

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