A rare case of congenital corneal clouding with anterior staphyloma of the eye

Abstract

There are only few case reports of congenital anterior staphyloma, an extremely rare condition. We report a case of congenital anterior staphyloma presenting as corneal clouding. A preterm girl baby was noted at birth to have bilateral corneal opacity with left sided microcornea. Ophthalmological evaluation revealed anterior staphyloma in right eye &total leucomatous corneal opacity in left eye. Despite antiglaucoma treatment, right sided bupthalmos & corneal thinning worsened. Corneal transplant was done at 7 months of age & baby improved symptomatically.Congenital anterior staphyloma occurs due to developmental aberration or secondary to inflammation. There are various genetic, metabolic, developmental & idiopathic causes of congenital corneal clouding like congenital glaucoma, birth trauma, peters anomaly, dermoid tumors, sclerocornea, infectious/inflammatory processes, metabolic causes & excess prenatal maternal consumption of alcohol. Complete evaluation of congenital corneal clouding include slit lamp biomicroscopy, funduscopy, tonometry, gonioscopy, ultrasonography, photo screening, ultrasound biomicroscopy & CT scanning in selected cases. Treatment is primarily surgical followed by management of amblyopia & optical therapy. Early penetrating keratoplasty, primary combined trabeculotomy-trabeculectomy & corneal grafting are associated with a favorable visual outcome. Early identification of ophthalmological problems in infancy & prompt intervention is mandatory for conditions like congenital corneal clouding.