Abstract

Diffuse choroidal hemangiomas associated with Sturge-Weber syndrome (SWS) are classically treated with external beam radiotherapy (EBR), but there are a few reports usually of single cases indicating the usefulness of plaque therapy. We present our observations on two cases of diffuse choroidal hemangiomas with exudative retinal detachment associated with SWS treated with Ruthenium-106 plaque therapy. Outcomes included best-corrected visual acuity (BCVA) and regression in tumor thickness measured by ultrasonography. The initial BCVA of the affected eyes was counting fingers at 1 meter and light projection. Pretreatment tumors thickness was 3.5 mm and 4.7 mm. In a follow-up period of 18–24 months, significant reduction in thickness of choroidal hemangiomas up to 1.2 mm and 1.4 mm with prompt resolution of exudative retinal detachment was observed. BCVA achieved 20/200 and 20/400, respectively. The findings in this paper indicate that Ruthenium-106 plaque therapy is effective in treatment of diffuse choroidal hemangiomas associated with SWS.

Highlights

  • The Sturge-Weber syndrome (SWS), called encephalotrigeminal angiomatosis, is a sporadically occurring neurocutaneous disorder with capillary venous angiomas involving the skin of the face, typically in the ophthalmic (V1) and maxillary (V2) distributions of the trigeminal nerve, choroid, and leptomeninges [1]

  • We present our observations on two cases of diffuse choroidal hemangiomas with exudative retinal detachment associated with SWS treated with Ruthenium-106 plaque therapy

  • external beam radiotherapy (EBR) has been recommended for diffuse choroidal hemangiomas with the presence of exudative retinal detachment [4, 5]

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Summary

Introduction

The Sturge-Weber syndrome (SWS), called encephalotrigeminal angiomatosis, is a sporadically occurring neurocutaneous disorder with capillary venous angiomas involving the skin of the face, typically in the ophthalmic (V1) and maxillary (V2) distributions of the trigeminal nerve, choroid, and leptomeninges [1]. Choroidal hemangiomas are estimated to occur in approximately one third of SWS cases, and they may occur in childhood or in early adulthood [2]. Diffuse choroidal hemangiomas may be associated with an exudative retinal detachment with macular involvement, photoreceptor cell loss and cystoid degeneration of the sensory retina causing visual loss [3]. These benign tumors are classically treated with external beam radiotherapy (EBR) [4, 5]. We present our observations on two cases of diffuse choroidal hemangiomas with exudative retinal detachment associated with SWS treated with Ruthenium-106 plaque therapy

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