Background: Choroidal hemangiomas are present in 20%-70% of patients with Sturge-Weber syndrome and may develop exudative retinal detachment. We attempted various therapies for refractory diffuse exudative retinal detachment in patients with Sturge-Weber syndrome and report our experience of a case in which proton beam therapy was most effective.
Case presentation: A 7-year-old girl who had hemangiomas in the first and second branches of the trigeminal nerve on the left side of the face from birth was diagnosed with Sturge-Weber syndrome. She had glaucoma in the left eye and received eye drop treatment and underwent surgery for glaucoma. After the third trabeculotomy, the intraocular pressure stabilized. During the follow-up examination, diffuse retinal detachment associated with exudation from choroidal hemangiomas appeared. Because photodynamic therapy, bevacizumab intravitreal injection, and chorioretinal cryocoagulation were not effective, we performed proton beam therapy (20 gray equivalent/10 times), which eliminated the exudative retinal detachment. No recurrence of retinal detachment was observed at 61 months after proton beam therapy. Posterior subcapsular cataract and time-dependent expansion of chorioretinal atrophy were observed as radiation-induced complications.
Conclusions: Proton beam therapy is effective for refractory diffuse exudative retinal detachment associated with choroidal hemangiomas in patients with Sturge-Weber syndrome. Long-term follow-up care is necessary to manage the side effects associated with radiation.
Nippon Ganka Gakkai Zasshi (J Jpn Ophthalmol Soc) 124: 432-440, 2020.