糖尿病網膜症に対して観血的治療を施行したPrader-Willi症候群の1例

Surgery for proliferative diabetic retinopathy in a case of Prader-Willi syndrome

要約 　目的：糖尿病網膜症に対して手術を行ったPrader-Willi症候群症例の報告。症例：23歳男性が両眼の視力低下で受診した。3歳のときに肥満や特異な顔貌からPrader-Willi症候群が疑われ，4歳のときに15番染色体長腕の部分欠失があり，本症候群の診断が確定した。10歳で糖尿病と診断され，9か月前に左右とも1.0の視力があった。所見と経過：身長151cm，体重97kgで，左右眼とも矯正視力は0.04，眼軸長は約21mmで，角膜屈折力は左右眼ともほぼ47Dであった。眼底に増殖糖尿病網膜症があり，右眼には網膜中心静脈閉塞症，左眼には硝子体出血が併発していた。右眼に汎網膜光凝固を行った後，左右眼に硝子体手術と水晶体乳化吸引術を行い，硝子体腔をシリコーンオイルで充填した。右0.05，左0.2の最終視力が得られた。結論：Prader-Willi症候群に併発した糖尿病網膜症に対して硝子体手術が奏効したが，精神発達遅延などが関係する術前・術後の管理に問題があった。早期発見と早期治療が望まれる。

Abstract. 　Purpose：To report a case of Prader-Willi syndrome who received vitreous surgery for proliferative diabetic retinopathy. Case：A 23-year-old male presented with impaired visual acuity in both eyes. Prader-Willi syndrome had been suspected at the age of 3 years for obesity and characteristic face. Partial defect of chromosome 15p led to the definitive diagnosis at the age of 4 years. He had been diagnosed with diabetes mellitus at the age of 10 years. Findings and Clinical Course：He was 151cm tall and 97kg in weight. Corrected visual acuity was 0.04 in either eye. Both eyes had axial length of about 21mm and corneal refraction of 47 diopters. Both eyes showed proliferative diabetic retinopathy. Central retinal vein occlusion was present in the left eye and vitreous hemorrhage in the left. Both eyes received vitrectomy and phacoemulsification-aspiration with filling of vitreous cavity by silicone oil. Final visual acuity was 0.05 right and 0.2 left. Conclusion：Surgery was effective for diabetic retinopathy in the present case of Prader-Willi syndrome. Patients with this syndrome need early detection and treatment due to pre-and postsurgical problems.