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A 40-year-old man with refractory glaucoma was referred for decreased vision in the right eye after an aqueous shunting surgical procedure 3 weeks prior. The surgical procedure was a 2-staged Baerveldt implantation after which he developed fluctuating but progressive visual decline. The patient’s ocular history was significant for Sturge-Weber syndrome with bilateral cutaneous port-wine stains, secondary glaucoma in the right eye, and an irregular globe with a white cataract and chronic retinal detachment (RD) in the left eye.
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Diffuse choroidal hemangioma with exudative retinal detachments
C. Treat with radiotherapy
The patient had classic ophthalmologic features of Sturge-Weber syndrome, including eyelid angiomas, glaucoma, and diffuse choroidal hemangioma (DCH). Early-onset glaucoma can develop from anterior chamber abnormalities and late onset from increased episcleral venous pressure.1 Glaucoma in the patient’s right eye, likely owing to the latter, was refractive to medical therapy. He underwent 2-stage Baerveldt insertion—in the first stage, the plate was attached to the globe and 1 month later, allowing for scarring and capsule formation, the tube was placed into the anterior chamber.2 This approach has been reported effective at avoiding rapid intraocular pressure lowering that could result in fluid transudation due to increased hydrostatic pressure between the choroidal and abnormally high-pressure Sturge-Weber syndrome venous system.3 Despite these measures, an exudative RD associated with DCH developed.
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Corresponding Author: Evangelos Gragoudas, MD, Department of Ophthalmology, Massachusetts Eye and Ear, 243 Charles St, Boston, MA 02114 (firstname.lastname@example.org).
Published Online: March 4, 2021. doi:10.1001/jamaophthalmol.2020.4665
Conflict of Interest Disclosures: None reported.
Additional Contributions: We thank the patient for granting permission to publish this information.
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