Systemic Associations of Childhood Glaucoma: A Review

Purpose: To review systemic associations of childhood glaucoma.

Methods: Patients younger than 15 years and diagnosed as having glaucoma were divided into four groups: isolated primary congenital glaucoma, glaucoma with other congenital ocular anomalies, congenital glaucoma with known systemic diseases, and secondary glaucoma. Prevalence and type of systemic associations in each group were studied.

Results: A retrospective analysis of 371 patients diagnosed as having glaucoma was done. In the primary congenital glaucoma group, 13 of 218 (5.9%) patients had an associated systemic illness: congenital heart disease and global developmental delay were the most common systemic manifestations. In the congenital ocular anomalies group, 10 of 63 (15.8%) patients had an associated systemic illness. Axenfeld-Reiger syndrome, aniridia, and Peters' anomaly frequently had systemic comorbidities with congenital heart disease. In the known systemic diseases group, all 18 (100%) patients had systemic manifestations of an associated syndrome: Sturge-Weber and Down syndrome were the most frequent. In the secondary glaucoma group, 9 of 72 (12.5%) patients had systemic involvement, which was often seen as the most common cause after congenital cataract surgery. These children had congenital heart disease and global developmental delay as a consequence of congenital rubella and congenital cytomegalovirus infection.

Conclusions: The study found that 12.9% of patients with childhood glaucoma had an associated systemic abnormality. Patients with congenital glaucoma and other ocular anomalies have a three times higher risk of an underlying systemic anomaly than patients with isolated primary congenital glaucoma. A team comprising an ophthalmologist, pediatrician, and anesthesiologist is recommended to treat these cases. [J Pediatr Ophthalmol Strabismus. 2018;55(6):397-402.].