Coexistent congenital hereditary endothelial dystrophy and congenital glaucoma.

PURPOSE

To retrospectively evaluate the coexistence of congenital glaucoma with congenital hereditary endothelial dystrophy.

METHODS

Ten infants presented to our hospital with diffuse corneal edema and bilaterally elevated intraocular pressure (IOP). These patients were diagnosed with congenital glaucoma. All patients underwent trabeculotomy with trabeculectomy for control of IOP. Although IOP was normalized in all patients, corneal edema persisted. These patients underwent penetrating keratoplasty, and the buttons were subjected to histopathologic examination.

RESULTS

The corneal grafts remained clear in all patients. The histopathologic examination of the excised corneal buttons showed diffuse stromal edema, loss of the endothelial cell layer, and thickening of the posterior non-banded portion of the Descemet membrane, suggestive of congenital hereditary endothelial dystrophy.

CONCLUSIONS

Congenital hereditary endothelial dystrophy may coexist with congenital glaucoma. This combination should be suspected where persistent and total corneal opacification fails to resolve after normalization of IOP.