硝子体出血を伴った結節性硬化症の眼底病変

An unusual macular involvement with vitreous hemorrhage in tuberous sclerosis

　片眼の黄斑部を中心とする眼底後極部に，広範な輪状白斑，網膜腫瘤とそれに連なる新生血管を伴う網膜隆起部（滲出性網膜剥離）を認め，経過観察中に硝子体出血を生じた21歳の結節性硬化症例を報告した．若年の本疾患例における内長型（endophytictype）の網膜腫瘤およびこれに連続する隆起病変は，網膜硝子体癒着および後部硝子体剥離に伴う硝子体出血などを生じ，高度の二次性視力障害の原因となることを示唆した．

A 21-year-old female presented with severe men-tal retardation, epilepsy and adenoma sebaceum with impaired visual acuity and afferent pupillary defect in her right eye. The right fundus showed juxtapa-pillary hemartoma of endophytic type associated with localized exudative retinal detachment extend-ing to involve the macula. A circinate retinal lesion surrounded the hamartoma and the retinal detach-ment. A fine capillary network was seen in the hamartomatous area. Four months later, we detect-ed subhyaloid and intravitreal hemorrhage conse-quent to posterior vitreous detachment in the affect-ed eye.

The present case illustrates that the retinal hamartoma of endophytic type in tuberous sclerosis may induce vitreoretinal adhesion and later poste-rior vitreous detachment with grave visual con-sequences.