Macular heterotopia secondary to proliferative diabetic retinopathy ha
s been previously reported in clinical cases. To our knowledge, we pre
sent the first clinicopathologic case of macular heterotopia in a youn
g patient with proliferative diabetic retinopathy. The significant pat
hologic findings included dense fibrovascular tissue on the disc to co
rrespond with nasal tractional retinal detachment, a superonasally dis
placed fovea, and an area of stripped and recoiled internal limiting m
embrane overlying a retinal fold. In addition, an area of reduplicated
retinal pigment epithelium was noted corresponding to the clinically
observed hyperpigmented area superior to the heterotopic fovea. Despit
e the pathologic changes, the patient maintained 20/40 visual acuity w
ith the heterotopic fovea for several years. The findings are discusse
d in relation to previous clinical studies.