Prevalence of Severe Visual Disability Among Preterm Children With Retinopathy of Prematurity and Association With Adherence to Best Practice Guidelines

Key Points

Question

What is the prevalence of visually disabling retinopathy of prematurity in Sweden and how is it associated with quality of care?

Findings

In this population-based cohort study of 1 310 227 infants born alive in Sweden in 2004 to 2015, 1 in 77 000 or 1 in 1000 very preterm infants (<32 weeks of gestational age) became severely visually disabled by retinopathy of prematurity. In 11 of 17 infants (65%), disability was considered avoidable because of nonadherence to best practice, as well as flaws in structural capacity.

Meaning

Severe visual impairment associated with retinopathy of prematurity is preventable in most cases.

Abstract

This cohort study investigates the prevalence of severe visual disability due to retinopathy of prematurity (ROP) among children in Sweden and examines the association between prevalence of disability and adherence to best practice guidelines for treatment of ROP.

Abstract

Importance

Retinopathy of prematurity (ROP) can cause severe visual disability even in high-resource settings. A better understanding of the prevalence and processes leading to ROP-induced severe visual impairment may help health care professionals design preventive measures.

Objectives

To determine the prevalence of severe visual disability among children born preterm in Sweden, evaluate adherence to best practice, and determine the health system’s structural capacity.

Design, Setting, and Participants

Population-based, nationwide cohort study of 1 310 227 children born between January 1, 2004, and December 31, 2015, in Sweden, of whom 17 588 (1.3%) were born very preterm (<32 weeks of gestation). Children born preterm with a verified diagnosis of severe visual disability had their medical records reviewed for evaluation of ROP screening, diagnosis, and treatment. In addition, a questionnaire on structural capacity was sent to all ophthalmology departments.

Exposures

Stages 4 and 5 ROP.

Main Outcomes and Measures

The primary outcome was prevalence of severe visual disability (visual acuity ≤20/200 for both eyes) associated with ROP stages 4 and 5. Secondary outcomes included adherence to national ROP guidelines using a predefined protocol with 15 key performance indicators for screening, diagnosis, and treatment; assessment of whether visual disability was deemed avoidable; and examination of structural capacity, including information on equipment and facilities, staffing, and patients.

Results

Seventeen children (10 boys; mean [range] birth weight, 756 [454-1900] g; mean [range] gestational age, 25 [22-33] weeks) became severely visually disabled because of ROP, corresponding to a prevalence of 1 in 1000 very preterm infants (<32 weeks of gestational age) and 1 in 77 000 for all live births. Severe visual impairment was considered potentially avoidable in 11 of 17 affected children (65%) owing to untimely or no screening, missed diagnosis, or untimely and suboptimal treatment. Large variations in infrastructure (facilities, guidelines, staffing, and annual patient numbers) were also identified as potential contributors to these findings.

Conclusions and Relevance

Retinopathy of prematurity still causes severe visual disability in Sweden, resulting in 1 affected infant per 1000 very preterm births. In most of these infants, noncompliance with best practice was identified, indicating that a significant proportion could have been avoided.