光凝固が奏効した網膜症を伴うincontinentia pigmentiの1症例

A case of incontinentia pigmenti with retinopathy successfully treated by xenon arc photocoagulation

（1）典型的なincontinentia pigmentiの初期眼底病変が，両眼にみられ，経過中に活動性病変の進行増悪を呈したため，光凝固を施行し，病変の沈静化・退縮をみた症例を報告した。

（2）初期網膜症は未熟児網膜症と類似した点も多いが，網膜静脈の著明な拡張・蛇行や，動静脈の異常吻合形成などが特有であった。

（3）本症例の治療経験より，光凝固はincontinentia pigmentiの初期眼底病変に対する有効な治療になりうると考える。患児の眼底において，滲出性・活動性病変が進行増悪し，網膜剥離への進展が予測されるような場合は，期を逸せず光凝固を施行すべきである。

Retrolental fibroplasia is one of the commonest and serious ocular manifestations of incontinentia pigmenti (Bloch-Sulzberger).

We observed a female infant with incontinentia pigmenti who manifested typical fundus changes shortly after birth. She was born at full term and weighed, 3,950g. While the fundus changes closely simulated those of active phase of retinopathy of prematurity, the marked engorgement and tortuosi-ty of retinal veins and aberrant retinal vasculariza-tion were specific for incontinentia pigmenti. A wide avascular zone was located in the peripheral retina.