Ophthalmology
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Ectopia lentis et pupillae (ELeP) is a rare congenital inherited disorder characterized by lenticular and pupillary ectopia. Recent speculation on its pathogenesis is based on clinical observation and includes a neuroectodermal defect or persistence of fetal vasculature. None of these reports included histopathology or imaging studies. ⋯ The only histologic reports of this condition are from the beginning of this century in the German literature. Ultrasonography at 50 MHz allows high-resolution in vivo imaging of anterior structures that are not clinically visible. The authors' findings in this case of ELeP are sufficiently different from those of non-ELeP-related aphakia to suggest that they are not solely due to aphakia. Furthermore, the authors' findings are very similar to the only histologic reports suggesting that the pupillary and lenticular ectopia results from mechanical tethering of the pupil with zonular disruption. This hypothesis has not been discussed in the recent literature.