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Editors Selection IGR 13-1

Epidemiology

Comment by Sharon Freedman on:

19604 The British Infantile and Childhood Glaucoma (BIG) Eye Study, Papadopoulos M; Cable N; Rahi J et al., Investigative Ophthalmology and Visual Science, 2007; 48: 4100-4106

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The pediatric glaucomas constitute a rare, heterogeneous, sight-threatening group of diseases. Although numerous reports on pediatric glaucoma and its treatment have been published, contemporary studies of the epidemiology and clinical outcomes for these children are sparce. Papadopoulos et al. (818) present results of a well-designed, prospective, national population-based study of pediatric glaucoma in the United Kingdom (UK). Data gathered included the incidence, detection patterns, current clinical management practices, and one-year intraocular pressure (IOP) outcomes, for children ≤ 16 years, with newly diagnosed primary or secondary glaucoma, over a one-year period (December 2001 November 2002). This prodigious project was facilitated by the British Ophthalmic Surveillance Unit (BOSU), created in 1997 to query all consultant ophthalmologists in the UK about uncommon ophthalmic disorders (Stanford, Br J Ophthalmol 1997; 81: 932-933). Participation by consultant ophthalmologists was excellent (95% of notifications were completed), and only 3% of eligible children were lost to follow-up. Statistical methods were robust, although small numbers of these rare conditions limited power in some comparisons. Ninety-nine cases of pediatric glaucoma were newly diagnosed in the UK (Great Britain and the Republic of Ireland) over the inclusion period, almost evenly divided between primary (45 primary congenital glaucoma, two juvenile open-angle glaucoma) and secondary glaucoma (53). Using number of live births over the same period, the incidence of PCG in the first year of life (82% of all PCG cases) was calculated to be 1/18,500 in Great Britain. Of interest was the large difference in primary congenital glaucoma (PCG) incidence based upon ethnicity, ranging from a low of 0.28 per 100,000 population in Caucasians, to 2.46 per 100,000 in those of Pakistani ethnicity, 67% of whom were products of consanguineous marriages. Secondary glaucomas presented at an older mean age than did primary glaucomas (46 vs 11 months, respectively, p < 0.0001), and included a large variety of diagnoses, 50% of which occurred after removal of cataracts, in both aphakic and pseudophakic eyes.

IOP control was successful in the majority of pediatric glaucomas at one yea

Newly-diagnosed primary glaucoma cases were identified due to parental concern in most cases (73%), while most secondary glaucoma cases were identified by clinical surveillance of children with known ocular disease (52%), and by parental concern in only 11%. Management included surgery for almost all PCG cases (96%), with angle surgery (goniotomy) the most common procedure, and additional surgery common, even over the short span of a year. IOP control (defined as ≤ 21 mmHg) was achieved in 60% without medications, and 94% with additional medications; mean IOP was 13 mmHg in PCG after one year.

Management of secondary glaucomas varied by specific diagnosis, with various medications and surgical procedures applied, with more than one surgery required in 55% of those initially operated upon. IOP control was achieved in 86% at one year, with 71% on medications, and mean IOP 15 mmHg. This ambitious study provides a real-time snapshot of the incidence of pediatric glaucoma in the UK over a one-year timeframe. Without any attempt to standardize the medical or surgical management of these rare, but potentially blinding diseases, the authors confirm successful IOP control in the majority of pediatric glaucomas at one year, lending hope that increased awareness of the diseases' presenting signs and risk patterns, and continued advances in treatment strategies and genetic understanding, may promote further improvement in visual outcomes and quality of life for these very special children.

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