経過中にうっ血乳頭を呈した結節性硬化症

Papilledema developed in a case of tuberous sclerosis

　17歳男子の結節性硬化症において4年間の経過観察中にうっ血乳頭を呈し，側脳室上衣下腫瘍の著明な増大を認めた．脳神経外科的に腫瘍摘出に成功したが，生命予後および視機能を保つ上でうっ血乳頭をはじめとする眼科的なフォローアップの重要性を示した．

We followed up a 17-year-old male with tuberous sclerosis. He manifested mental retardation, epilep-tic attacks, adenoma sebaceum, retinal hamartoma with full visual acuity, and a small calcified lesion in the left ventricle as detected by CT scan.

At a scheduled follow-up examination at the age of 21, we detected bilateral papilledema with slight visual disturbance. Enhanced CT scan showed the presence of a highly dense mass in the right ventri-cle. The tumor was successfully removed neurosur-gically and was diagnosed as subependymal giant cell astrocytoma. Intracranial hypertension and papilledema subsided after surgery. Visual acuity recovered without optic atrophy.

The case illustrates the importance of systematic ophthalmological examination for the early detec-tion of intracranial lesion in this disease.

Rinsho Ganka (Jpn J Clin Ophthalmol) 41(7) : 871-875, 1987