Primary Congenital Glaucoma in the Developing World

Abstract

Despite its rarity, primary congenital glaucoma and other forms of childhood glaucoma were once a leading cause of admission to schools for the blind in the United States in the first part of the 20th century. Goniotomy and trabeculotomy surgery were introduced in the middle part of the 20th century, and both procedures have been used with excellent long-term success in primary congenital glaucoma, and with much improved visual outcomes. In the last 30 years, a marked decrease in admission of childhood glaucoma to schools for the blind has been noted in the United States and Ireland. However, access to these highly specialized surgical treatments is frequently difficult for many who live in the developing world. A study of worldwide blindness noted that the majority of the estimated 1.4 million blind children live in the poorest regions of Africa and Asia, with recent improvements noted in the treatment of vitamin A deficiency in some countries. In this issue of Ophthalmology, 2 articles are presented that discuss the surgical treatment of primary congenital glaucoma in the developing world. The authors of these articles are to be commended for their efforts to provide much-needed services for a rare condition that might otherwise have gone untreated, with long-term consequences for the disabled individual affected by the condition. Bowman et al present the results of goniotomy surgery to treat primary congenital glaucoma in Tanzania, demonstrating 60% success after 1 goniotomy and 89% success after 2 gonitomies in 36 eyes followed up for 12 months. Ben-Zion et al used trabeculotomy (and Ahmed Glaucoma Valves) to treat 38 eyes of 20 patients with primary congenital glaucoma in Ethiopia. They noted successful reduction in intraocular pressure (IOP) and improved visual behavior in these severely affected patients (average age at surgery of 3.3 years, mean IOP 54 mmHg, 33 eyes with nonambulatory vision), albeit with some serious complications associated with these extremely buphthalmic eyes. These 2 articles point to numerous issues and problems associated with treating primary congenital glaucoma in the developing world: (1) overcoming fears of the surgical treatment and improving awareness of the disease (2 cases in the Ben-Zion article were brought to care only after spontaneous perforation had occurred); (2) limited access and long travel distances to care centers, leading to late presentation of the disease in an advanced state when it is less amenable to surgical treatment, along with inadequate follow-up of treated cases; (3) limited number of surgeons versed in the microsurgical treatment necessary for successful control of the disease due to its rarity (the primary surgeon in Bowman et al had only observational surgical exposures despite pediatric fellowship training in the United Kingdom). Bowman et al describe broad-based community and national outreach programs to overcome some of these issues and bring patients and families in for initial treatment