アルゴンレーザー光凝固療法を行ったBloch-Sulzberger症候群の1例

A case of Bloch-Sulzberger syndrome treated by argon laser photocoagulation

　生後4日目に眼底病変を認めた　Bloch—Sulzberger症候群の女児の1例を報告する。

　両眼底とも網膜静脈は怒張蛇行し，網膜血管は赤道部まで伸びておらず，周辺部では動静脈吻合，新生血管，網膜硝子体出血を認めた。螢光造影検査では，右眼の網膜血管は血流を認めず，左眼でも視神経乳頭の耳側では2乳頭径，鼻側では4乳頭径まで周辺より無血管帯が及んでいた。両眼底ともに，増殖性変化が進行するため，アルゴンレーザーを用いて汎網膜光凝固術を施行した。光凝固後，右眼では血流がみられるようになり，両眼ともに網膜静脈の怒張蛇行の減少，網膜血管の無血管帯への伸展，螢光漏出の減少が認められた。生後1年目の眼所見では，右眼視神経萎縮と内斜視を認めるものの，眼球は正常大で，眼底に増殖性の変化はない。

A baby was diagnosed as Bloch-Sulzberger syn-drome at birth. On day 4 after birth, we found the retina to be vascularized posterior to the equator only. There were dilatation of veins, arteriovenous anastomosis, neovascularization and hemorrhages along the peripheral margin of the vascularizedretina. Fluorescein angiography showed absence of retinal circulation in the right eye. Retinal circula-tion was confined within an area of 3 to 4 disc diameters from the optic disc.

We treated both eyes with argon laser panretinal photocoagulation to halt progression of the proliferative lesions. Laser treatment was complet-ed on day 20 after birth. It was followed by initia-tion of retinal blood flow and lesser dye leakage from retinal vessels. No proliferative lesions were present one year after birth.