A 9-year-old Caucasian female was referred for an ocular prosthetic for her right eye. She had been previously diagnosed with persistent hyperplastic primary vitreous (PHPV) with congenital nystagmus. She was reported to have no vision in her right eye and her parents were looking for improved cosmesis as some of the patient’s friends were starting to notice her disfigurement as she approached adolescence.

EXAM FINDINGS

Her corrected entering visual acuities were light perception OD and 20/30 OS with a 4+ afferent pupillary defect (APD) OD by reverse. Figure 1 shows gross observation with leukocoria OD and normal OS. To maintain natural iris detail, a tinted soft contact lens was selected with an occluder pupil. However, her right cornea appeared to be steeper than average after trialing several soft commercial contacts lenses that resulted in fluting. Corneal tomography was attempted, but was questionable at best due to her moderate nystagmus (Figure 2). K_max varied on several scans from 49D to 58D and revealed iris attachment to the corneal endothelium (Figures 3 and 4). A custom soft lens was ordered, fit, and tinted with successful cosmetic appearance (Figure 5).

DISCUSSION

PHPV is a congenital disorder with failure of the fetal vessels to regress in the eye (Sisk et al, 2010). This results in leukocoria, cataracts, deprivation amblyopia, and other ocular complications causing reduced vision (Schulz and Griffiths 2010). While visual prognosis may be variable, cosmesis may still be improved using custom soft tinted contact lenses, even in young, complicated patients. Soft ocular prosthetic contact lenses hold a unique space in the ophthalmic world and can have dramatic effects on our patients lives.

REFERENCES

1. Sisk RA, Berrocal AM, Feuer WJ, Murray TG. Visual and anatomic outcomes with or without surgery in persistent fetal vasculature. Ophthalmology. 2010 Nov;117:2178-83.e1-e2.
2. Schulz E, Griffiths B. Long-term visual function and relative amblyopia in posterior persistent hyperplastic primary vitreous (PHPV). Strabismus. 2006 Jun;14:121-125.