Primary Congenital and Childhood Glaucoma-A Complex Clinical Picture and Surgical Management.

Abstract

Childhood glaucoma encompasses a group of rare but severe ocular disorders characterized by increased intraocular pressure (IOP), posing significant risks to vision and quality of life. Primary congenital glaucoma has a prevalence of one in 10,000-68,000 people in Western countries. More worryingly, it is responsible for 5-18% of all childhood blindness cases. According to the Childhood Glaucoma Research Network (CGRN), this spectrum of disease is classified into primary glaucoma (primary congenital glaucoma and juvenile open-angle glaucoma) and secondary glaucomas (associated with non-acquired ocular anomalies, non-acquired systemic disease, acquired conditions, and glaucoma after cataract surgery). They present very specific ocular characteristics, such as buphthalmos or progressive myopic shift, corneal modifications such as Haab striae, corneal edema or increased corneal diameter, and also glaucoma findings including high intraocular pressure, specific visual fields abnormalities, and optic nerve damage such as increased cup-disc ratio, cup-disc ratio asymmetry of at least 0.2 and focal rim thinning. Surgical intervention remains the cornerstone of treatment, and initial surgical options include angle surgeries such as goniotomy and trabeculotomy, aimed at improving aqueous outflow. For refractory cases, trabeculectomy and glaucoma drainage devices (GDDs) serve as second-line therapies. Advanced cases may require cyclodestructive procedures, including transscleral cyclophotocoagulation, reserved for eyes with limited visual potential. All in all, with appropriate management, the prognosis of PCG may be quite favorable: stationary disease has been reported in 90.3% of cases after one year, with a median visual acuity in the better eye of 20/30. Immediate recognition of the specific signs and symptoms by caregivers, primary care providers, and ophthalmologists, followed by prompt diagnosis, comprehensive surgical planning, and involving the caregivers in the follow-up schedule remain critical for optimizing outcomes in childhood glaucoma management.