DSEK for Congenital aniridia. An interesting and challenging story

Congenital aniridia (CA) is a rare condition affecting a wide range of ocular structures from the ocular surface to the retina. We present the case of a PAX6 and WT1 negative Congenital Aniridia 59-year-old female patient with Aniridia associated Keratopathy and progressive endothelial dysfunction with corneal decompensation following cataract surgery. Patient underwent successful ultrathin Descemet’s Stripping Endothelial Keratoplasty. Despite the challenges faced with an unstable irido–lenticular diaphragm, we were pleasantly surprised to see improvement not only of corneal oedema and endothelial function but also of the whole cornea including the anterior corneal anatomy and appearance.
Endothelial transplantation in a patient with aniridia resulted in improvement of all the corneal structures from the endothelium to the stroma, epithelium and possibly even the ocular surface.