Sturge-Weber syndrome (encephalofacial angiomatosis)
These eyes carry high risk for expulsive choroidal hemorrhage upon entering the eye causing sudden pressure change.
They are more likely to develop choroidal effusion from choroidal cavernous hemangioma.
Reducing the IOP as low as possible and performing posterior sclerotomies prior to entering the eye may reduce complications.
In a study by Iwach et al, intraoperative choroidal expansion was detected in 24% of patients who underwent trabeculectomy. [6]
In Agarwal's series of 18 patients with Sturge-Weber syndrome who underwent the trabeculotomy-trabeculectomy procedure, the following complications were noted: intraoperative hyphema (22.2%), vitreous loss (16.7%), and vitreous hemorrhage (5.6%). [5]
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Axenfeld-Rieger syndrome with iris atrophy, corectopia, and pseudopolycoria.
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Female patient with plexiform neurofibroma (NF-1). Upper right eyelid involvement, associated with ipsilateral buphthalmos. In Image A (left), patient is aged 8 months; in Image B (right), patient is aged 8 years.
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Female infant with Sturge-Weber syndrome. Facial port-wine nevus involves the left eyelid, associated with ipsilateral buphthalmos.
