[MIM 305 390]

Following the premature termination of the retineal vascularisation, there persists an avascular zone in the temporal periphery of the retina. This avascular area can be isolated or associated with sickle-shaped retinal folds, or retineal detachment.

In case of temporal retinal folds, the macula may be attracted toward the temporal zone ('macular ectopia'). Retineal detachment  causes a progressive loss of vision. In some cases, this could be a minor form of Norrie disease (see this term) due to a mutation of the NDP gene.

Anesthetic implications:

common ophthalmic surgery, eye protection, visual impairment.

References : 

Updated: May 2019