Primary congenital glaucoma with extreme axial elongation and pseudo-lens coloboma: A case report

We report a case of neglected bilateral primary congenital glaucoma (PCG) in a 4-year-old boy who presented with progressive bilateral globe enlargement and diminution of vision over two years. On examination, the child had enlarged globes, epiphora, and photophobia. Visual acuity was hand movements (HMs) in the right eye (OD) and counting fingers at 1 m in the left eye (OS), with digitally tense globes. Examination under general anesthesia revealed megalocornea (horizontal corneal diameter, 16.5 mm; vertical corneal diameter, 15 mm), Haab’s striae, corneal haze, and average intraocular pressures of 49 mmHg in OD and 42 mmHg in OS. Axial lengths were markedly increased, measuring 34.27 mm in OD and 33.15 mm in OS. Bilateral pseudo-lens coloboma with angle dysgenesis involving all quadrants was identified. Fundus examination demonstrated advanced glaucomatous optic nerve cupping in both eyes. The patient underwent sequential combined external trabeculotomy-trabeculectomy with mitomycin C in both eyes.