Background: Sturge-Weber syndrome is associated with a high incidence of choroidal hemangioma and glaucoma. Here, we report on a patient in whom external beam irradiation was effective for exudative retinal detachment associated with choroidal hemangioma. However, outflow tract reconstruction was ineffective for associated glaucoma in this patient, and glaucoma drainage implant surgery was required.
Patient: The patient, a girl aged 7 years and 2 months, presented with facial hemangioma and was diagnosed with Sturge-Weber syndrome. Exudative retinal detachment due to choroidal hemangioma was observed in the right eye, and external irradiation therapy was administered at a total dose of 21 Gy. Subretinal fluid disappeared completely after 3 months, with no recurrence for at least 2 years after treatment. Despite continuing ophthalmic treatment for glaucoma, high intraocular pressure (IOP), optic disc rim, and progressive thinning of the retinal nerve fiber layer was observed in optical coherence tomography (OCT). Thus, outflow tract reconstruction (suture trabeculotomy) was performed. On day 2 after the surgery, anterior chamber irrigation was performed because progressive anterior chamber hemorrhage and high IOP were observed. Although improvements in the anterior chamber hemorrhage were observed, high IOP persisted; hence, glaucoma drainage implant surgery was performed, subsequently resulting in normalization of IOP.
Conclusions: Continuous follow-up and appropriate treatment for choroidal hemangioma and glaucoma are important when treating Sturge-Weber syndrome. When glaucoma is present, the possibility that outflow tract reconstruction may be ineffective should be considered, particularly if late glaucoma onset is observed.
Nippon Ganka Gakkai Zasshi (J Jpn Ophthalmol Soc) 125: 142-149, 2021.