What is the role of Sturge-Weber syndrome in the etiology of glaucoma?

Updated: Jun 25, 2020
  • Author: Andrew A Dahl, MD, FACS; Chief Editor: Hampton Roy, Sr, MD  more...
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Answer

Sturge-Weber syndrome

Sturge-Weber syndrome (encephalotrigeminal angiomatosis) involves hamartomatous, vascular tissues and produces a characteristic port-wine hemangioma of the skin along the trigeminal distribution and ipsilateral angioma of the meninges and the brain.

Estimates vary, but one study showed the presence of glaucoma in up to 50% of cases in which the port-wine stain involves the ophthalmic and maxillary divisions of the trigeminal nerve. Glaucoma develops before age 2 years in 60% of patients, with the remainder developing by early adulthood.

Ipsilateral conjunctival and episcleral vascular dilation may signal arteriovenous malformation, leading to elevated episcleral venous pressure.

In the same study, 69% of 51 patients had conjunctival or episcleral hemangiomas, and glaucoma was present in 71% of these patients. [4]

Various other studies have shown anterior chamber angle anomalies, including angle neovascularization. The major mechanism may involve increased episcleral venous pressure, causing delay in the aqueous outflow.

Management consists of combining medical, laser, and surgical options for late-onset glaucoma and primarily surgical intervention in infants.

In one study, the median period of control was determined as the following: goniotomy (12 mo), trabeculotomy (21 mo), trabeculectomy (21 mo), argon laser trabeculopexy (ALT) (25 mo), and medications (57 mo). [5] However, trabeculectomy was associated with a high incidence of choroidal effusions and expulsive hemorrhage.


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