High measured intraocular pressure in children with recessive congenital hereditary endothelial dystrophy.

PURPOSE: To report the phenomenon of high measured intraocular pressure in recessive congenital hereditary endothelial dystrophy.

METHODS

A case series was retrospectively reviewed.

RESULTS

Five infants with significant congenital corneal haze had increased measured intraocular pressure that remained high despite drug treatment and surgery to decrease intraocular pressure. The clinical diagnosis of recessive congenital hereditary endothelial dystrophy without glaucoma was made based on the absence of buphthalmos, a distinct pattern of mosaic corneal haze with significant corneal thickness, and absence of cupping in healthy-appearing optic nerve heads.

CONCLUSION

Intraocular pressure can be falsely elevated in some children with recessive congenital hereditary endothelial dystrophy, leading to confusion with congenital glaucoma.