BILATERAL KERATOCONUS WITH OCULOCUTANEOUS ALBINISM

The aim of our work is to present a very rare case of bilateral keratoconus in oculocutaneous albinism which has not been reported recently in literature.

Adult ophthalmology department, hospital August 20, Casablanca, Morocco

A 23 year-old albino male, a known case of oculocutaneous albinism (OCA) type 1a, presented to us with diminution of vision since his childhood with deterioration for the past six months, a frequent eye rubbing and photophobia. He had no family history. On physical examination, he was seen to have light dewy skin and light white hair. On ophthalmological examination, both eyes were found to have visual acuity (he was able to see the number of fingers held close to her face), horizontal pendular nystagmus, right esotropia, and a positive Munson′s sign in both eyes. On slit lamp examination, his cornea showed thinning with conical protrusion in both eyes (the right more than the left), bilateral stromal scarring, Fleisher′s ring, and Vogt′s striae. 

The anterior surface corneal topography of the right eye has showed a maximum keratometry readings of 79.3 diopter in right eye and 62.5 diopter in the left eye. Corneal pachymetry measurements at the thinnest locations were 428 microns and 464 microns for the right and left eye, respectively. The patient is a condidact for keratoplasty.

Keratoconus is a progressive corneal disease with an onset typically in teenage years. While it often presents as an isolated condition, keratoconus may also be associated with many systemic and/or ocular diseases, such as oculocutaneous albinism.