HETEROTOPIA of the macula is an extremely rare finding, and a survey of the literature reveals that there are 6 reported cases to the present time from all causes. In addition, there are 3 pathologic demonstrations of this condition in fetal eyes.
The earliest case report is that of Krüger,1 who described a congenital anomaly of the posterior pole in which both the disk and the macula were displaced laterally so that the nerve head lay in the pupillary axis and the macula was assumed to be far temporal. Triebenstein,2 in 1919, reviewed the German literature and described 4 previously reported cases, adding 2 of his own. In the 4 former cases, both disk and macula were displaced concomitantly down, up or up and out. These abnormalities were found in conjunction with foci of chorioretinitis, fibrous traction bands and/or colobomas of the choroid and optic nerve.3 Triebenstein
COHEN IJ, WEISBERG HK. VERTICAL HETEROTOPIA OF THE MACULA. Arch Ophthalmol. 1950;44(3):419-423. doi:10.1001/archopht.1950.00910020428010