Coats' syndrome: long term follow up

To increase the understanding of the long term results in pseudo-retinoblastoma eyes with infantile Coats' syndrome.

This study design was a retrospective case review. 10 patients were analysed who were initially referred with a diagnosis of retinoblastoma but had Coats' syndrome on the basis of ocular oncological evaluation. Vision, fundus photography, ultrasonography, and computed tomography scans were obtained and evaluated. Changes in vision and retinal status were measured.

The initial age at presentation was 2.4 years (range 0.25-4 years). All patients had retinal detachment at diagnosis. Nine of 10 retinas were reattached after various treatments. Reattached retinas had closure of peripheral telangiectasia and visible intraretinal crystals. Vision was dismal. At last follow up (mean 8.8 years), only two patients had 20/400 or better visual acuities. Five eyes had no light perception despite early treatment to reattach the retina. Nine of 10 eyes remain cosmetically acceptable. One patient wore a cosmetic shell.

Long term results indicate that these eyes can be salvaged and the retina reattached but the visual outcome is poor.