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Retinal Detachment in a 40-Year-Old Man With Sturge-Weber Syndrome

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

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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Article Information

Corresponding Author: Evangelos Gragoudas, MD, Department of Ophthalmology, Massachusetts Eye and Ear, 243 Charles St, Boston, MA 02114 (evangelos_gragoudas@meei.harvard.edu).

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.

References
1.
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2.
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3.
Bellows  AR , Chylack  LT  Jr , Epstein  DL , Hutchinson  BT .  Choroidal effusion during glaucoma surgery in patients with prominent episcleral vessels.   Arch Ophthalmol. 1979;97(3):493-497. doi:10.1001/archopht.1979.01020010243011 PubMedGoogle ScholarCrossref
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Karimi  S , Nourinia  R , Mashayekhi  A .  Circumscribed choroidal hemangioma.   J Ophthalmic Vis Res. 2015;10(3):320-328. doi:10.4103/2008-322X.170353 PubMedGoogle ScholarCrossref
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Shields  CL , Dalvin  LA , Lim  LS ,  et al.  Circumscribed choroidal hemangioma: visual outcome in the pre-photodynamic therapy era versus photodynamic therapy era in 458 cases.   Ophthalmol Retina. 2020;4(1):100-110. doi:10.1016/j.oret.2019.08.004 PubMedGoogle ScholarCrossref
6.
Tsipursky  MS , Golchet  PR , Jampol  LM .  Photodynamic therapy of choroidal hemangioma in Sturge-Weber syndrome, with a review of treatments for diffuse and circumscribed choroidal hemangiomas.   Surv Ophthalmol. 2011;56(1):68-85. doi:10.1016/j.survophthal.2010.08.002 PubMedGoogle ScholarCrossref
7.
Queirós  L , Sioufi  K , Shields  CL .  Diffuse choroidal hemangioma treated with low-dose plaque brachytherapy.   Retina Today. November/December 2016:38-40. Accessed January 15, 2021. https://retinatoday.com/pdfs/1116RT_Oncology.pdfGoogle Scholar
8.
Randon  M , Lévy-Gabriel  C , Abbas  R ,  et al.  Results of external beam radiotherapy for diffuse choroidal hemangiomas in Sturge-Weber syndrome.   Eye (Lond). 2018;32(6):1067-1073. doi:10.1038/s41433-018-0024-4 PubMedGoogle ScholarCrossref
9.
Arepalli  S , Shields  CL , Kaliki  S , Emrich  J , Komarnicky  L , Shields  JA .  Diffuse choroidal hemangioma management with plaque radiotherapy in 5 cases.   Ophthalmology. 2013;120(11):2358-2359, 2359.e1-2359.e2. doi:10.1016/j.ophtha.2013.07.058 PubMedGoogle ScholarCrossref
10.
Chan  RV , Yonekawa  Y , Lane  AM ,  et al.  Proton beam irradiation using a light-field technique for the treatment of choroidal hemangiomas.   Ophthalmologica. 2010;224(4):209-216. doi:10.1159/000260226 PubMedGoogle ScholarCrossref
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