Acute hydrops as an atypical presentation of primary congenital glaucoma

Primary congenital glaucoma (PCG) is a disease of childhood characterized by elevated intraocular pressure (IOP) that causes stretching of the eye’s outer coats, namely sclera and cornea. This results in the elongation of the eyeball and expansion of the horizontal corneal diameter giving the appearance of a buphthalmos eye. Aggressive disease with high IOP readings causes excessive mechanical stretching that may be poorly tolerated by the corneal Descemet’s membrane, leading to large breaks in it with subsequent corneal edema due to sudden influx of the aqueous humor into the exposed stroma, resulting in acute corneal hydrops. While acute hydrops is a potential sequel of PCG, it is considered one of its rare presentations. We present two cases who presented to our hospital with acute hydrops secondary to PCG. Both patients were managed surgically where the first patient underwent combined trabeculotomy-trabeculectomy with mitomycin C, while the second patient underwent deep sclerectomy with mitomycin C. The surgical procedures effectively controlled the IOP and aided in clearing corneal edema in both patients. Early diagnosis and timely surgical intervention are of paramount importance to improve visual outcomes, enhance ocular maturation, and prevent potential irreversible vision loss, especially in this young-age group of patients that are prone to amblyopia.