What is the role of surgery in the treatment of Sturge-Weber syndrome (SWS) glaucoma?

Updated: Dec 26, 2018
  • Author: Masanori Takeoka, MD; Chief Editor: George I Jallo, MD  more...
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Most ophthalmologists consider surgical therapy to be the mainstay of glaucoma therapy in patients with Sturge-Weber syndrome, [92] with antiglaucoma medications primarily useful as treatment adjuncts. However, the selection of surgical technique remains controversial. [111] Surgical options in SWS glaucoma include the following:

  • Goniotomy

  • Trabeculotomy

  • Full-thickness filtration surgery

  • Partial-thickness filtration surgery (trabeculectomy)

  • Combined trabeculotomy-trabeculectomy

  • Argon laser trabeculoplasty

  • Neodymium:yttrium-aluminum-garnet (Nd:YAG) laser goniotomy

  • Seton procedures

Trabeculectomy increases the release of aqueous fluid from the anterior chamber and opens the outflow pathway. Goniotomy is similar but is done through the eye. A Molteno valve can be placed (similar to a shunt), and cyclodestructive procedures with either freezing or laser decrease the production of aqueous fluid. [49]

The long-term results of SWS glaucoma surgery are often disappointing, as they are associated with a high failure rate compared with the same procedure performed for primary infantile glaucoma.

Because SWS 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 IOP 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 SWS, 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.

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