Bilateral Congenital Cataract And 6.25-Mm Horizontal Corneal Diameter In A Child With Oculodentodigital Dysplasia

To describe the surgical case of a child with oculodentodigital dysplasia, bilateral congenital cataracts and extreme microcornea.

This patient was treated at the Altino Ventura Foundation, a nonprofit organization in Recife, Brazil.

This is a case of an 8-year-old boy with oculodentodigital dysplasia – a rare autosomal dominant disorder that has been diagnosed in less than 300 people worldwide. His vision was restricted to light perception in both eyes (OU). He presented with bilateral membranous congenital cataract, a 6.25-mm horizontal corneal diameter in OU, significant iris pigment dispersion, and 360^o non-continuous posterior synechiae. The anteroposterior diameter was 20.45 mm in the right eye (OD) and 20.16 in the left eye (OS). His visual evoked potential suggested a 20/80 vision in OD and worse than 20/400 vision in OS. After weighing the risks and potential benefits of the surgery with the patient’s mother, we agreed on operating his OD. Intraoperatively, I used a radiofrequency cautery to perform to perform the anterior capsulotomy. I started phacoaspirating with my irrigation/aspiration tip. Intraoperatively, the eye’s iris-crystalline lens complex behaved like that of a high myope, which was only partially minimized by fluidics optimization. After the Descemet membrane near the main incision started detaching and presented a tendency for the rapid progression of this complication, I decided to continue removing the cataract by using viscoelastic and wound depression. On the first and eighth postoperative days, he presented with a transparent cornea, formed anterior chamber and hand motion vision. He and his mother reported how the visual gain has improved his daily activities.

In very challenging pediatric cataract scenarios, having a visual evoked potential is important to support surgical decision. When this exam shows a possible benefit from surgery - despite patients’ age and ocular characteristics - and the caregivers agree with the procedure after understanding all risks involved, the surgeon’s dedication and ability to overcome intraoperative challenges is key. Even though an improvement of light perception to hand motion can seem small, for a low vision patient it completely changes their life. Meticulous surgical maneuvers are important to decrease the risks associated to surgery in these extreme eyes.