Congenital Corneal Staphyloma

A 15-month-old boy was referred to the Oculoplastic Clinic at Labbafinejad Medical Center for treatment of a bulging and deformed blind left eye. He was the result of a normal full-term pregnancy and there had been no ocular discharge at birth. Examination of the left eye revealed a keratinized and ectatic cornea bulging 9 mm anteriorly (Fig. 1). The right eye had a chorioretinal coloboma. Due to severe deformity of the blind eye, we considered enucleation to be the most appropriate treatment. The enucleated left globe measured 38×21×20 mm; horizontal and vertical corneal diameters were 21×20 mm respectively. The opaque cornea showed surface keratinization and a cystic forward projection measuring 16 mm. The cornea was edematous and up to 4 mm in thickness. The anterior chamber angle was occluded by iris adhesions. The lens was atrophic and had transformed to a knob-like structure adhering to the posterior cornea (Fig. 2). However, the posterior segment and optic nerve appeared normal. On microscopic examination, the cornea demonstrated diffuse ectasia and the corneal epithelium showed hyperkeratosis, parakeratosis, acanthosis and irregular elongation of rete pegs. Bowman’s layer was thin or absent in many areas. The corneal stroma was edematous, vascularized and diffusely infiltrated by acute and chronic inflammatory cells. Descemet’s membrane and the endothelium were completely absent (Fig. 3). The posterior aspect of the cornea was entirely lined by atrophic iris tissue obliterating the anterior chamber angle. The optic disk, retina and sclera appeared normal. Figure 1 Keratinized corneal ectasia and severe bulging of the left eye at the time of enucleation.