スタージ・ウェーバー症候群に合併したびまん性脈絡膜血管腫の2症例に対する放射線治療効果

Effect of radiation therapy for diffuse choroidal hemangioma in two cases of Sturge-Weber syndrome

要約　目的：スタージ・ウェーバー症候群に合併したびまん性脈絡膜血管腫に対して放射線治療が効果的であった2症例の報告。

症例：症例1は12歳，女児。症例2は32歳，男性。いずれも顔面に血管腫を認め，スタージ・ウェーバー症候群と診断されている。症例1は左眼のびまん性脈絡膜血管腫による滲出性網膜剝離を認め，光線力学療法を2度施行されるも改善しなかった。総線量20Gyの放射線治療を行い，3か月で網膜下液は完全に消失し，治療後6年間で再発しなかったが，網膜動脈の白線化と後囊下白内障を認めた。症例2は15年前に左眼のびまん性脈絡膜血管腫に対し放射線治療を施行され，滲出性網膜剝離は消失した。網膜剝離の再発を認め，総線量20Gyの放射線再照射を行い，再照射後12か月を経過し網膜剝離の再発や合併症はない。

結論：スタージ・ウェーバー症候群に合併したびまん性脈絡膜血管腫に対する放射線治療は，光線力学療法後の初回例，放射線治療後再発例ともに治療効果が期待できる。

Abstract　Purpose：To report 2 cases of diffuse choroidal hemangiomas in Sturge-Weber syndrome（SWS）that regressed after radiation therapy.

Cases and Findings：Case 1 was 12 year-old girl and case 2 was 32 year-old male. Both of them showed port-wine stains and were diagnosed as SWS. Case 1 presented diffuse choroidal hemangioma associated with exudative retinal detachment in her left eye. Photodynamic therapy（PDT）, performed twice, was unsuccessful in absorption of subretinal fluid. She was treated by external radiation with total dose of 20 Gy and the retina was completely reattached three months after irradiation. She developed the sheathing of retinal vessels and a posterior subcapsular cataract following six years. In the case 2, radiation therapy was performed for diffuse choroidal hemangioma in his left eye 15 years ago and retinal detachment was regressed. Recurrence of the hemangioma occurred and he was treated with repeated irradiation with total dose of 20 Gy. Twelve months after re-treatment, resolution of the retinal detachment and no radiation complication occurred.

Conclusion：Low-dose ocular irradiation may be a promising therapy for diffuse choroidal hemangioma complicated with SWS in both cases of no response to PDT and recurrence after the radiation therapy.