Systemic Associations of Childhood Glaucoma: A Review

To review systemic associations of childhood glaucoma.

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.

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.

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.].