2740 - Lens-Sparing IMRT for the Management of Sturge-Weber Syndrome Associated Diffuse Choroidal Hemangiomas: An Institutional Experience

A. Ufondu¹, A. Singh¹, and S. Cherian²; ¹Cleveland Clinic Foundation, Cleveland, OH, ²Department of Radiation Oncology, Cleveland Clinic Foundation, Cleveland, OH

Purpose/Objective(s): Sturge-Weber syndrome is a disease characterized by maldeveloped blood-vessels resulting in the characteristic port-wine stain on the face, as well as capillary-venous malformations in the brain and eye. One of the less common ocular findings is characterized by diffuse of circumscribed choroidal hemangiomas. We used Intensity-Modulated Radiotherapy (IMRT) to manage the choroidal hemangiomas and analyze the results, paying attention to visual acuity and dose to organs at risk. Materials/

Methods: Single institutional retrospective review of patients with Sturge-Weber syndrome associated diffuse choroidal hemangioma managed with IMRT (20Gy in 10 fractions). Prior to IMRT, visual acuity was measured. This was compared to after IMRT. The dose to organs at risk was also collated from planning system after IMRT to determine potential long-term risks.
Results: Five patients (5 eyes) are examined in this review, which were treated between 2019 and 2023. Subretinal fluid was present in 3 of the patients prior to radiation. There is an average follow-up time of 17 months. At last follow-up, SRF height had decreased in all patients. All patients, but most recently treated (<6 weeks), have had improvement or stability of visual acuity in treated eyes. Average mean dose to the PTV was 2072 cGy. Average mean dose to the ipsilateral lens was 854 cGy, while average mean dose to the contralateral lens was 172 cGy. Average mean dose to the ipsilateral fovea and optic disc were 2065 cGy and 2085 respectively.
Conclusion: Use of IMRT 20Gy in 10 fractions provides efficient control in patients with SWS diffuse choroidal hemangiomas. In this small cohort of patients, we have been able to demonstrate efficacy in visual stabilization or improvement in the follow-up period. This is with reasonable sparing of the ipsilateral lens, excellent sparing of the contralateral lens, and safe doses delivered to the ipsilateral fovea and optic disc.