Spontaneous exudative retinal detachment in a patient with sturge-weber syndrome after taking arginine, a supplement for erectile dysfunction

Abstract

BackgroundPatients with Sturge-Weber syndrome can have ipsilateral diffuse or circumscribed choroidal hemangiomas. These hemangiomas have been seen to undergo spontaneous exudative or hemorrhagic retinal detachments. There is no definitive treatment for these types of retinal detachments, but radiotherapy, photodynamic therapy, oral propranolol, pegaptinib and bevacizumab have been used.Case presentationA 26-year-old male with Sturge-Weber Syndrome developed an exudative retinal detachment that occurred immediately after taking a supplement containing arginine. The patient was treated with intravitreal bevacizumab 1.25 mg in 0.05 ml solution. Resolution of the retinal detachment was seen after 4 treatments over a six-month period.ConclusionsArginine and other medications that cause a release of nitric oxide may lead to intravascular leakage and exudative retinal detachments in patients who have a choroidal hemangioma.Electronic supplementary materialThe online version of this article (doi:10.1186/s40662-014-0007-x) contains supplementary material, which is available to authorized users.

An Update on Multimodal Ophthalmological Imaging of Diffuse Choroidal Hemangioma in Sturge–Weber Syndrome

“…In contrast, FFA uses fluorescein dye, which remains within the retinal vasculature and is visualized using blue light. ICGA’s longer wavelength and ability to penetrate deeper layers make it particularly suitable for studying choroidal circulation [ 52 ].…”

“…From around 20–180 s, complete dye filling yields intense hyperfluorescence with a mix of hyper- and hypofluorescent dots on an already luminescent background. Concurrently, areas of diminished choroidal circulation in the upper or lower mid periphery emerge, likely attributed to the tumor’s space-occupying effect, which masks the standard choroidal ICGA pattern [ 48 , 49 , 52 ].…”

“…In summary, ICGA provides valuable insight into the intricate choroidal circulation within DCHs [ 48 , 49 , 52 ]. However, it is also important to underline that FFA and ICGA are imaging techniques requiring dye administration.…”

Retinal vascular abnormalities in Sturge-Weber syndrome

“…[3] There is a higher risk of developing exudative retinal detachment either spontaneously or induced by drug. [8] Pan retinal laser photocoagulation has been recommended for the management of high risk proliferative DR, however it has been shown to result in increased choroidal thickness and blood flow till after 1 week. [9] Laser photocoagulation in already thickened and vascular choroid may lead to serious retinal detachment.…”