Congenital Anterior Corneal Staphyloma In A 2-Year-Old Child: A Rare Unilateral Presentation

To present a rare case of unilateral congenital anterior corneal staphyloma associated with posterior staphyloma and marked axial elongation in a young child, highlighting diagnostic and therapeutic challenges.

2nd Department of Ophthalmology, Aristotle University of Thessaloniki, Papageorgiou General Hospital, Thessaloniki, Greece

A 2-year-old female child was referred for evaluation of a congenital opacity and marked protrusion of the right cornea. Comprehensive ophthalmic examination included slit-lamp biomicroscopy, tonometry, anterior segment OCT imaging and B-Scan ultrasonography 

Examination of the right eye revealed an ectatic, opaque, and vascularized cornea consistent with congenital anterior corneal staphyloma. Measured CCT was 280 μm. Visualization of anterior segment structures was not possible.The IOP was 12 mmHg; however, given the extremely thin cornea the true intraocular pressure is likely underestimated. Posterior segment evaluation was not possible clinically. B-Scan ultrasonography demonstrated an axial length of 27.38 mm and revealed a posterior staphyloma, suggesting significant axial elongation.The fellow eye appeared structurally normal with a sole abnormal finding of reduced pharmacologic mydriasis. The child also demonstrated sensory nystagmus, likely related to asymmetric visual development.

Congenital anterior corneal staphyloma is a rare developmental anomaly that may be associated with significant axial elongation and posterior staphyloma, as demonstrated in this case. Extreme corneal thinning complicates accurate tonometry and clinical visualization of intraocular structures. Ultrasound imaging and biometry are essential for evaluation and management planning. Early recognition is important for appropriate monitoring, amblyopia management, and consideration of surgical options such as keratoplasty in selected cases.