先天無虹彩に伴う緑内障に対しバルベルト®緑内障インプラントが有効であった1例

Baerveldt® implant was effective for glaucoma in a case of congenital aniridia

要約　目的：先天無虹彩に伴う緑内障に対し，バルベルト^®緑内障インプラント（BGI）が有効であった症例の報告。

症例：生後20日の女児が無虹彩として紹介受診した。妊娠中に異常はなく，出生時の体重は2,106gであった。父親に先天無虹彩と緑内障があり，母方祖父に網膜色素変性があった。

所見と経過：全身麻酔下での眼圧は右10mmHg，左9mmHgで，両眼に虹彩の全欠損，水晶体の前囊と後囊下の混濁，乳頭の陥凹拡大があった。角膜径は両眼とも11mmで，眼軸長は右20.38mm，左20.33mmであった。2歳11か月に眼圧が左右とも30mmHgになり，複数の点眼治療を開始した。4歳から6歳までに両眼に線維柱帯切除術が3回行われた。6歳2か月のときに右眼圧が30mmHgに上昇し，BGI手術が行われた。手術3日目に低眼圧と脈絡膜剝離が生じたが，8日目にチューブを結紮し，以後18か月後の現在まで経過は良好である。

結論：先天無虹彩に伴う緑内障は難治であることが多い。線維柱帯切除術が奏効しない症例に対して，BGI手術は有効な選択肢の1つである。

Abstract　Purpose: To report a case of congenital aniridia in whom Baerveldt^® implant was effective for glaucoma.

Case: A female baby was referred to us on day 20 after birth for aniridia. There was reportedly no problem during pregnancy. She was born with the birthweight of 2,106 g. Her father had aniridia with glaucoma. Her maternal grandfather had retinitis pigmentosa.

Findings and Clinical Course: Intraocular pressure（IOP）under general anesthesia was 10 mmHg right and 9 mmHg left. Both eyes showed total aniridia, lens opacity and excavation of optic disc. Corneal diameter was 11 mm in either eye. Axial length was 20.38 mm right and 20.33 mm left. IOP increased to 30 mmHg in both eyes at the age of 2 years 11 months, necessitating treatment with multiple eyedrops. Trabeculotomy was performed 3 times on both eyes between 4 and 6 years of age. Baerveldt^® implant surgery was performed on the right eye at the age of 6 years 2 months. Hypotony and choroidal detachment developed on day 3 and was treated by ligature of implant. She has been doing well for 18 months until present.

Conclusion: Glaucoma secondary to congenital aniridia may be refractory to treatment. Baerveldt^® implant surgery promises to be effective in such cases.