Heterotopia of the macula (ectopic macula): an unusual presentation

Heterotopia of the macula is a condition in which the macula is displaced markedly from its normal position. Only 40 cases have been reported in the literature. Of those cases more than 90% have occurred in patients presenting with an apparent exotropia (macula displaced temporally), a large positive angle lambda, and an actual esotropia. We report a unique case of heterotopia of the macula in which the patient presented with an apparent esotropia and a large negative angle lambda. This is the only reported case of ectopic macula in which the macula has been dragged nasally toward the disc in association with a suspected retinopathy of prematurity. There have been three other cases of heterotopia of the macula in which the macula was displaced nasally, but these were associated with chorioretinitis.