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Editors Selection IGR 10-3

Clinical Forms of Glaucoma: Long-term outcome of primary congenital glaucoma

James Brandt

Comment by James Brandt on:

46086 Long-term outcome of primary congenital glaucoma, de Silva DJ; Khaw PT; Brookes JL, Journal of AAPOS, 2011; 15: 148-152


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The care of infants and children with glaucoma is among the most challenging, but ultimately rewarding, areas of glaucoma practice. Those of us with a particular interest in this field like to think that angle surgery like goniotomy or trabeculotomy can be 'curative', but the reality is that many of our patients end up needing further surgery in their teenage or adult years.

What a difference long-term follow up makes! In this study from Moorfields Eye Hospital in London, de Silva et al. (765) identified 30 eyes from 16 patients for whom at least 20 years of follow up was available. They divided their cohort into one of three groups: (1) patients with stable glaucoma and no visual impairment; (2) patients with evidence of progressive glaucoma with sight-threatening consequences; and (3) patients with poor visual acuity as a result of ocular comorbidities such as retinal detachment or corneal failure.

What a difference long-term follow up makes!

The results are sobering. At a mean follow up of 34 years, despite achieving reasonably good control of IOP, lack of progression ('success') dropped from 90% at one year to less than half with 40 years of follow up. The authors found that in primary congenital glaucoma (PCG) patients, glaucomatous progression could occur after many years of apparent stability and at any time during the follow-up. Glaucomatous progression was observed in about one third of these eyes and only one in three had no apparent visual impairment with stable glaucoma ('success'). This rate is lower than has been reported by other studies limited by shorter follow-up. Although this study is itself limited by small numbers, its strengths include its very long-term follow up (as much as 59 years) and its careful categorization of the underlying glaucoma.

Like any long-term study spanning decades, these results are not necessarily predictive of what might be expected among pediatric glaucoma patients treated today. Advances in surgical approaches and medications will probably lead to better long-term results, but we must recognize that long-term follow up is needed to really know this for certain. Given the relative rarity of PCG and other pediatric glaucoma syndromes, an Internet-based registry of pediatric glaucoma patients will go a long way to generate the numbers to really understand our long-term outcomes.



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