Abstract

Following reports by Sanders et al. and Wise et al. of optic disc drusen associated with retinal bleeding, an additional 19 cases were studied and followed up for 1 to 10 years (average 5 years). Four of them showed haemorrhages at the optic disc and its borders only, but 15 showed extensive retinopathy with macular involvement. In 3 cases retinopathy was serous and not haemorrhagic. Apart from cases in which bleeding was limited to the disc and its immediate vicinity the main clinical features of the syndrome were as follows: (1) the presence of optic disc drusen; (2) extensive retinal haemorrhage; (3) intraretinal extravasation of serum with or without the presence of blood; (4) elevated mounds lifting the retina and causing striae radiating from the mounds towards the macula and beyond; (5) pigmentary disturbance of the macula or a wider area of papillo-macular bundle after the resolution of acute stages which, while often clinically severe, is compatible with recovery of normal or near normal vision. It is proposed that the syndrome be designated the 'optic disc drusen retinopathy.'