What is the recommended diagnostic and treatment approach for a premature infant suspected of having retinopathy of prematurity (ROP)?

Retinopathy of Prematurity: Diagnosis and Management

Direct Recommendation

Infants with a diagnosis or at risk of retinopathy of prematurity must be referred to a pediatric ophthalmologist for specialized screening and management. 1

Screening Criteria and Timing

Who Requires Screening

Screen all infants born at less than 32 weeks gestational age, regardless of clinical stability. 2 While infants between 29-37 weeks with a medically stable course (no supplemental oxygen requirement) may not require screening, a conservative approach is safer. 2

• Infants with chronic lung disease of infancy (CLDI) require screening even if born between 29-37 weeks gestation 2
• Infants with more than 37 weeks gestation at birth do not need screening 2

Timing of First Examination

Perform the first ROP screening examination at the later of either 31-33 weeks postmenstrual age OR 4 weeks chronological age. 2 This timing is critical—no infants born at less than 27 weeks gestation have been found to have severe ROP requiring treatment prior to 31 weeks postmenstrual age, supporting this recommendation. 3

Diagnostic Approach

Examination Requirements

The examination must be conducted by an ophthalmologist experienced in evaluating the premature infant retina. 2 This is non-negotiable for accurate diagnosis and staging.

• Wide-field digital retinal imaging systems are the current standard for screening 4
• Telemedicine approaches are acceptable when coupled with timely referral pathways for abnormal findings 2
• The examination should include evaluation of the optic disc, macula, retina, vessels, and choroid, preferably using indirect ophthalmoscopy with a condensing lens after adequate dilation 1

Classification and Risk Stratification

The examination determines the zone and stage of ROP, which dictates follow-up intervals:

• Zone I or Zone II disease: Requires ophthalmology visits every 1-2 weeks due to higher risk of progression to threshold ROP 2, 5
• Zone III with regressing ROP: Vessels passed into Zone 3 on at least two sequential examinations is extremely unlikely to progress to threshold 2, 5

Treatment Approach

Primary Treatment Modality

Laser photocoagulation (peripheral retinal ablation) is the gold-standard treatment for threshold ROP. 5, 4, 6 This treatment has proven effective in reducing blindness from ROP, especially in high-risk populations. 5

Alternative and Adjunctive Therapies

• Intravitreal anti-VEGF agents may be combined with laser photocoagulation and are currently being evaluated, particularly for Zone I disease 4, 6
• Surgery is reserved for advanced stages with retinal detachment 4
• Oral propranolol shows encouraging results for prevention and treatment of prethreshold retinopathy, though this remains investigational 6

Critical Management Considerations

Oxygen Management

Careful oxygen management is essential throughout the disease course. 5 High arterial oxygen levels slow normal vascularization, while marginally low oxygen aggravates neovascularization following initial injury. 5

• Once past the age of oxygen-induced retinopathy risk, target oxygen saturation of 95% or higher to prevent pulmonary complications 5
• Oxygen therapy is a well-established risk factor affecting both phases of ROP pathophysiology 2

Follow-Up Coordination

For infants with unresolved ROP and CLDI being discharged home, careful coordination of follow-up ophthalmology appointments is crucial. 2 Missing follow-up appointments can lead to missed treatment opportunities and preventable vision loss. 2, 5

The greatest risk occurs when infants with unresolved ROP in Zone 1 or 2 are discharged home and miss follow-up appointments. 2

Discontinuation of Screening

Screening can be discontinued when complete retinal vascularization is documented. 2, 5

• ROP that is regressing with vessels passed into Zone 3 on at least two sequential examinations is extremely unlikely to progress to threshold 2, 5
• Infants with ROP stage zero Zone III do not require continued screening 5

Common Pitfalls to Avoid

• Do not delay initial screening beyond 31-33 weeks postmenstrual age or 4 weeks chronological age (whichever is later), as this is when treatable disease first appears 2, 3
• Do not discharge infants with Zone I or Zone II disease without ensuring weekly or biweekly ophthalmology follow-up is arranged, as this is when preventable blindness occurs 2, 5
• Do not use general ophthalmologists for screening—only pediatric ophthalmologists with specific ROP expertise should perform these examinations 1, 2