Abstract General Information

Título / Title

TURNING THE TIDE: BRACHYTHERAPY SUCCESS IN A CHILD WITH STURGE-WEBER SYNDROME AND TOTAL SEROUS RETINAL DETACHMENT

Introdução / Purpose

Sturge-Weber syndrome (SWS) is a neurocutaneous disorder featuring facial capillary malformations, leptomeningeal angiomas, and diffuse choroidal hemangiomas, seen in ~40% of cases and often ipsilateral to facial port-wine stains. These vascular lesions can cause complications like exudative retinal detachment and macular edema, leading to vision loss. Management options include photodynamic therapy, external beam radiotherapy, and brachytherapy, with the latter chosen in the Brazilian public healthcare system due to limited resources. This case report aims to present a successful case of diffuse hemangioma treated with brachytherapy.

Material e Método / Methods

Patient's medical records review

Resultados / Results

An 8-year-old boy presented with a month-long history of painless, decreased visual acuity in the left eye (OS). Examination revealed a port-wine stain on the left hemiface, BCVA of 20/25 in the right eye (OD), and "hand movements" (HM) in OS. Fundoscopy showed total serous retinal detachment in OS. Ultrasound confirmed a diffuse choroidal hemangioma measuring 6.64 mm in thickness.
Initial treatments, including intravitreal injections and cryotherapy, failed. Brachytherapy with a Ruthenium-106 plaque was performed, resulting in retinal reattachment, tumor reduction, and BCVA improvement to 20/400. Two years later, the patient remains stable without subretinal fluid, neovascularization, or secondary glaucoma.

Discussão e Conclusões / Conclusion

Brachytherapy with a Ruthenium-106 plaque is an effective treatment for diffuse choroidal hemangiomas associated with Sturge-Weber syndrome. Despite its high cost and limited availability, it results in tumor regression, resolution of subretinal fluid, and stabilization or improvement in VA. A study on five patients treated with doses of 30.98–47.36 Gy showed tumor regression and fluid resolution in all cases. This case illustrates brachytherapy's success in achieving retinal reattachment and long-term stability after conventional treatments failed.

Palavras Chave

Diffuse Hemangioma
Sturge-Weber Syndrome
Brachytherapy