Senior-loken syndrome complicated with severe coats disease-like exudative retinopathy.

BACKGROUND: Senior-Loken syndrome is a rare disorder that combines juvenile nephronophthisis with retinitis pigmentosa. METHODS: Case report. RESULTS: A 9-year-old Japanese girl diagnosed with Senior-Loken syndrome subsequently developed severe Coats disease-like exudative retinopathy. Although retinal coagulation, pars plana lensectomy, and vitrectomy were performed, she lost light perception in both eyes. CONCLUSION: Faulty vascular morphogenesis and its dysfunction might contribute to the development of Coats disease-like exudative retinopathy in Senior-Loken syndrome.