The Case

Abstract

A 13-month-old girl was seen for further evaluation of a “large right eye” that had been present since infancy, was more apparent during the past 6 weeks, and was unassociated with chronic tearing or photophobia. “Big and beautiful eyes” had frequently been noted by family members and friends, but the medical evaluation attributed the ocular size to normal variability. On physical examination, she appeared healthy. On ocular examination, she presented with poor fixation in the right eye and normal visual responses in the left eye. External examination observed cornea enlargement asymmetry confirmed by corneal diameter measurements of 13.8 and 12.1 mm in the right and left eyes, respectively (Figure). Mild conjunctival injection was noted in the right eye, associated with central stromal thickening and opacification. The anterior chambers were deep and greater in the right eye than in the left eye. Each iris possessed normal stromal tissue with a normal pupillary ruff and well-developed collarette. The lenses were clear bilaterally. The intraocular pressures measured were 45 mm Hg in the right eye and 18 mm Hg in the left eye. Each disc was well vascularized with central cupping in the right eye. Gonioscopy demonstrated well-developed angle structures in both eyes, with visible sclera spurs and moderately wide ciliary body bands. The ciliary body band in the right eye was less dark in appearance than in the left eye. A goniotomy was recommended for the right eye. Three months after an uncomplicated procedure, the intraocular pressure in the right eye was 14 mm Hg and the cornea had cleared, revealing a horizontal tear in Descemet’s membrane.