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Epidemiology and long-term outcomes of primary congenital glaucoma: a population-based study

Eye volume 37, pages 2673–2678 (2023)Cite this article

Subjects

Abstract

Background/Objectives

to establish the incidence, prevalence and long-term outcomes of primary congenital glaucoma (PCG) in Northern Ireland’s general and Irish Traveller (IT) populations over a 59-year period.

Subjects/Methods

chart review of all PCG cases in Northern Ireland between 1962 and 2020. Incidence and prevalence were calculated with the aid of national population statistics. Long-term outcomes were analysed for eyes with at least 5 years follow up. Visual outcomes were stratified into groups: good–VA ≥ 6/12; moderate–VA 6/12 to 6/60, poor–VA < 6/60. Kaplan–Meier analysis was constructed to determine the probability of eyes retaining a good visual outcome over time. Outcomes of different surgeries were compared with regard to final vision, re-operations and complications.

Results

57 PCG cases were identified between 1962 and 2020. Overall incidence was 3.4 per 100,000 live births. PCG prevalence in general and IT populations in 2019 was 4.3 per 100,000 and 238 per 100,000, respectively. Sixty eyes had sufficient data for long-term outcome analysis with a mean of 20.5 years follow up, 58% of which had good final visual outcome. Fifty-seven percent of eyes required multiple surgeries. Visual outcomes between trabeculectomies and tubes were comparable; however, there were more serious complications and reoperations associated with tubes.

Conclusions

Incidence of PCG in NI is similar to Great-Britain and Ireland; however, PCG appears to be particularly prevalent within IT community. Although a good visual outcome can be achieved in the majority of patients, a subset of cases remain challenging to manage despite surgical advances.

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Data availability

The datasets generated during and/or analysed during the current study are available from the corresponding author on reasonable request. Northern Ireland birth/population statistics and data relating to the Irish Traveller population utilised in this study are available online through the Northern Ireland Statistics and Research Agency (see references [12,13,14]).

References

  1. Lewis CJ, Hedberg-Buenz A, DeLuca AP, Stone EM, Alward WLM, Fingert JH. Primary congenital and developmental glaucomas. Hum Mol Genet. 2017;26:R28–36.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  2. Badawi AH, Al-Muhaylib AA, Al Owaifeer AM, Al-Essa RS, Al-Shahwan SA. Primary congenital glaucoma: An updated review. Saudi J Ophthalmol. 2019;33:382–8.

    Article  PubMed  PubMed Central  Google Scholar 

  3. Ferák V, Gencik A, Gencikova A. Population genetic aspects of primary congenital glaucoma. II. Fitness, parental consanguinity, founder effect. Hum Genet. 1982;61:198–200.

    Article  PubMed  Google Scholar 

  4. de Silva DJ, Khaw PT, Brookes JL. Long-term outcome of primary congenital glaucoma. J AAPOS. 2011;15:148–52.

    Article  PubMed  Google Scholar 

  5. Yassin SA. Long-term visual outcomes in children with primary congenital glaucoma. Eur J Ophthalmol. 2017;27:705–10.

    Article  PubMed  Google Scholar 

  6. Zhang X, Du S, Fan Q, Peng S, Yu M, Ge J. Long-term surgical outcomes of primary congenital glaucoma in China. Clin (Sao Paulo). 2009;64:543–51.

    Article  Google Scholar 

  7. Vimalanathan M, Gupta P, Vardhan SA, Pillai MR, Uduman MS, Krishnadas SR, et al. Long-term surgical outcomes of primary congenital glaucoma in a South Indian population. Ophthalmol Glaucoma. 2020;4:522-30.

  8. Zagora SL, Funnell CL, Martin FJ, Smith JE, Hing S, Billson FA, et al. Primary congenital glaucoma outcomes: lessons from 23 years of follow-up. Am J Ophthalmol. 2015;159:788–96.

    Article  PubMed  Google Scholar 

  9. Esfandiari H, Prager A, Hassanpour K, Kurup SP, Mets-Halgrimson R, Yoon H, et al. The long-term visual outcomes of primary congenital glaucoma. J Ophthalmic Vis Res. 2020;15:326–30.

    PubMed  PubMed Central  Google Scholar 

  10. Mohammedsaleh A, Raffa LH, Almarzouki N, Jubran RM, Al-Harbi A, Alluqmani AH, et al. Surgical outcomes in children with primary congenital glaucoma: an eight-year experience. Cureus. 2020;12:e9602.

    PubMed  PubMed Central  Google Scholar 

  11. Thau A, Lloyd M, Freedman S, Beck A, Grajewski A, Levin AV. New classification system for pediatric glaucoma: implications for clinical care and a research registry. Curr Opin Ophthalmol. 2018;29:385–94.

    Article  PubMed  Google Scholar 

  12. NISRA: Birth Statistics 1887–2019. https://www.nisra.gov.uk/publications/birth-statistics

  13. NISRA: 2019 Mid Year Population Estimates for Northern Ireland. https://www.nisra.gov.uk/publications/2019-mid-year-population-estimates-northern-ireland

  14. NIHE: Irish Traveller Accommodation Survey 2018–2019. https://www.nihe.gov.uk/Documents/Research/Travellers-Accommodation-Survey-2018-19/Irish-Traveller-Accommodation-Survey-2018-19-(pdf-.aspx

  15. Papadopoulos M, Cable N, Rahi J, Khaw PT. BIG eye study investigators. the british infantile and childhood glaucoma (BIG) eye study. Invest Ophthalmol Vis Sci. 2007;48:4100–6.

    Article  PubMed  Google Scholar 

  16. McGinnity FG, Page AB, Bryars JH. Primary congenital glaucoma: Twenty years experience. Ir J Med Sci. 1987;156:364–5.

    Article  CAS  PubMed  Google Scholar 

  17. Gencik A, Gencikova A, Ferák V. Population genetical aspects of primary congenital glaucoma. I. Incidence, prevalence, gene frequency, and age of onset. Hum Genet. 1982;61:193–7.

    Article  CAS  PubMed  Google Scholar 

  18. Rolim-de-Moura C, Esporcatte BLB, F Netto C, Paranhos A Jr. Baerveldt implant versus trabeculectomy as the first filtering surgery for uncontrolled primary congenital glaucoma: a randomized clinical trial. Arq Bras Oftalmol. 2020;83:215–24.

    PubMed  Google Scholar 

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Acknowledgements

Professor Sir Peng T Khaw, Miss Maria Papadopoulos, Mr John L Brookes and Ms Rosie Brennan, co-managers of our patients with PCG outside of the Belfast Trust.

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Authors and Affiliations

  1. Department of Ophthalmology, Royal Victoria Hospital, Belfast Trust Health and Social Care Northern Ireland, Belfast, Northern Ireland

    Robert John Nutt, Mohammad Samir Dowlut & Eibhlin McLoone

  2. School of Electronics, Electrical Engineering and Computer Science, Queen’s University Belfast, Belfast, Northern Ireland

    Seán Francis McLoone

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  1. Robert John Nutt
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Contributions

EM was responsible for instigating, supervising and reviewing this work. MD collected much of the data up until 2012 and contributed to the first draft. SM advised on and performed statistical analysis. RN collected study data and was the main author of this article.

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Correspondence to Robert John Nutt.

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The authors declare no competing interests.

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Nutt, R.J., Dowlut, M.S., McLoone, S.F. et al. Epidemiology and long-term outcomes of primary congenital glaucoma: a population-based study. Eye 37, 2673–2678 (2023). https://doi.org/10.1038/s41433-023-02382-6

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  • DOI: https://doi.org/10.1038/s41433-023-02382-6

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