[Two cases of Senior-Loken syndrome]

We report two rare cases of Senior-Loken syndrome, one with fundus lesions simulating Coats disease. CASE 1: A 14-year-old boy had juvenile nephronophthisis and bilateral retinitis pigmentosa with retionochoroidal atrophy and salt and pepper fundus. Electroretinogram (ERG) showed non-recordable changes and the Goldmann perimeter showed ring scotomas. CASE 2: A 14-year-old boy had bilateral retinitis pigmentosa and juvenile nephronophthisis with continuous ambulatory peritoneal dialysis. Additionally, both eyes showed massive exudates seen in Coats disease in the entire periphery, exudative retinal detachment, proliferative changes in the inferior periphery, yellow opacitas corporis vitrei, keratoconus, and cataract. The left eye had vitreous hemorrhages. Both eyes received vitreous surgery and endophotocoagulation. After the surgery the left eye showed neovascular glaucoma and a cyclophotcoagulation was performed. The massive edema in the superior retina of both eyes disappeared and intra-ocular pressure in the left eye was normalized. However, despite these treatments, both eyes finally had no light perception.

Case 1 was a typical SLS, and case 2 was a rare one with fundus lesions simulating Coats disease, keratoconus, and cataract. We suspect the exudative changes resulted from various vessel changes and choroidal circulatory disturbance of renal retinopathy, retinal vascular damage, and destruction of blood-retinal barrier with retinitis pigmentosa, the inflammatory changes of vessels, and other things.