Ten-year follow-up of familial nanophthalmos in three siblings

Nanophthalmos is a rare congenital disorder associated with a high incidence of angle-closure glaucoma. We followed the clinical manifestations of three siblings to evaluate their responses to various treatments.

Three sisters with nanophthalmos were followed from 2000 to 2013. Glaucoma and cataract treatments were performed whenever indicated.

The oldest sister had chronic elevation of intraocular pressure (IOP) and underwent laser peripheral iridotomy (LPI) on both eyes, followed by uneventful phacoemulsification with intraocular lens (IOL) implantation on the left eye and phacotrabeculectomy with IOL implantation on the right eye. The middle sister had acute elevation of IOP and initially underwent phacoemulsification combined with implantation of two IOLs on her left eye and LPI on her right eye. Severe uveal effusion occurred when phacoemulsification was performed on her right eye 6 years later, but ultimately was completely resolved. In both sisters, stable IOP and visual results were achieved after lensectomy. The youngest sister, who had suspected angle-closure, achieved a stable IOP and visual results with prophylactic LPI alone.

In nanophthalmic eyes, the severity of the disease may foreshadow the severity of surgical complications and responses to therapy.