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Primary congenital glaucoma in Denmark, 1977-2016.
Karen B Pedersen1, Per Kappelgaard1, Line Kessel1,2
1Department of Ophthalmology, University Hospital of Copenhagen, Rigshospitalet-Glostrup, Glostrup, Denmark.
Acta Ophthalmologica
|October 31, 2019
Summary
Primary congenital glaucoma (PCG) affects 4.8 per 100,000 live births, with a higher incidence in boys. Diagnostic delay for PCG remains consistent over four decades, highlighting the need for standardized pediatric screening.
Area of Science:
- Ophthalmology
- Pediatrics
- Epidemiology
Background:
- Primary congenital glaucoma (PCG) is a rare, severe form of glaucoma present at birth.
- Understanding PCG epidemiology is crucial for early detection and management in children.
- Nationwide data on PCG incidence and risk factors are limited.
Purpose of the Study:
- To conduct a 40-year epidemiological survey of primary congenital glaucoma (PCG) in Denmark.
- To determine the incidence and identify risk factors for PCG.
- To evaluate diagnostic delay and age at diagnosis for PCG.
Main Methods:
- Retrospective nationwide study of all children diagnosed with PCG in Denmark (1977-2016).
- PCG defined as isolated angle dysgenesis, excluding other congenital abnormalities.
- Analysis of incidence, risk factors (gender, consanguinity, family history, ethnicity, comorbidity, prematurity), and diagnostic timelines stratified by decade.
Main Results:
- Identified 118 patients with an annual PCG incidence of 4.8 per 100,000 live births.
- Boys had a significantly higher relative risk (RR 1.60). 62.3% of cases were bilateral.
- Median diagnostic delay was 75 days, unchanged across decades. Unilateral cases had later symptom onset and diagnosis.
Conclusions:
- This study provides unique 40-year, nation-based PCG incidence data.
- Male gender is a significant risk factor for PCG.
- Consistent diagnostic delays and high comorbidity rates suggest a need for standardized pediatric screening for PCG.
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