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Ophthalmology

What Do the Zones Mean in Retinopathy of Prematurity?

At a Glance

In retinopathy of prematurity, Zones I, II, and III show where abnormal or incomplete blood-vessel growth is located in the retina. Zone I is closer to the optic nerve and is higher risk, but treatment depends on the zone, stage, and plus disease.

Hearing that your baby has “Zone I” Retinopathy of Prematurity (ROP) can be incredibly frightening. However, a zone label on its own does not determine your baby’s visual outcome. In an ROP report, the “zones” simply map out the location of normal and abnormal blood vessel growth across your baby’s retina [1]. It is only one part of the diagnosis—your doctor will evaluate the zone alongside other features to decide the best care plan.

The Bullseye Map

During pregnancy, a baby’s eye blood vessels grow from the center of the eye outward. In premature babies, this growth is interrupted, leaving peripheral areas of the retina immature (avascular) [2]. Doctors use the International Classification of Retinopathy of Prematurity (ICROP) to map where this normal vessel growth has stopped [1].

Think of the retina as a map centered on the optic nerve (the cable connecting the eye to the brain). Right next to the optic nerve is the macula (the area responsible for detailed central vision). The eye is mapped into three regions:

  • Zone I (The Center): This is the innermost circle around the optic nerve and macula. If ROP is in Zone I, the normal vessels stopped growing very early, leaving a larger portion of the retina immature.
  • Zone II (The Middle Ring): A larger area surrounding Zone I. Normal blood vessels here have grown further outward but have not yet reached the far edges.
  • Zone III (The Outer Edge): A crescent-shaped area, mostly toward the ear-side of the eye. If vessel growth has reached Zone III, normal vessels have completed most of their journey. While usually lower risk than Zone I, Zone III disease is not entirely risk-free and still requires monitoring.

Decoding the ROP Report: Zone vs. Stage vs. Plus Disease

To fully understand the severity of your baby’s condition, you need more than just the zone. A complete diagnosis uses three distinct classification features [1]:

  • Zone (Where): Where is the disease located? (Zones I, II, or III).
  • Stage (What): What does the abnormal growth at the edge of the vessels look like? (Ranked Stage 1 through 5).
  • Plus Disease (Activity): Are the main blood vessels in the back of the eye enlarged and twisted? Plus disease lies on a continuous spectrum and indicates how active or severe the condition is [1].

What Does Zone I Mean for My Baby?

Because Zone I encompasses the critical optic nerve and macula, disease located here is considered a higher-risk location [2]. It means there is a larger area of the retina without normal blood flow. This lack of blood flow (called ischemia) can contribute to the growth of fragile, abnormal blood vessels [3].

However, a Zone I finding does not automatically mean your baby needs immediate treatment. Treatment decisions are based on a specific combination of Zone, Stage, and Plus disease. For example, mild early-stage disease in Zone I might be observed very closely without intervention. But if the disease progresses or shows “Plus” features, it might reach a treatment threshold (often called Type 1 ROP). Type 1 disease typically requires therapies like laser treatments or anti-VEGF medication injections (such as bevacizumab) to prevent retinal detachment and protect vision [4][5].

The Importance of Strict Follow-Up

ROP often has no visible signs that a parent can detect from the outside. Because premature infants are at risk of rapid progression, time-sensitive eye exams at exact intervals are essential to catch changes before they become destructive [6].

Even if your baby receives treatment, long-term and vigilant follow-up remains critical. ROP can reactivate weeks or even months later, particularly if it was treated with anti-VEGF medications, making continued surveillance vital for protecting your child’s future vision [7][1].

Common questions in this guide

What do Zones I, II, and III mean in ROP?
The zones are map locations showing where normal blood-vessel growth has reached or where ROP is located in the retina. Zone I is the central area around the optic nerve and macula, Zone II is the surrounding middle ring, and Zone III is the outer crescent. The zone is only one part of the diagnosis and does not determine the outcome by itself.
Does Zone I ROP automatically mean my baby needs treatment?
No. Zone I is a higher-risk location because blood-vessel growth stopped closer to the optic nerve and macula, leaving more retina without normal blood flow. The eye team also considers the stage, plus or pre-plus disease, and whether the findings are changing before recommending treatment or close observation.
How are ROP zone, stage, and plus disease different?
The zone describes where ROP is located in the retina. The stage describes what the abnormal growth looks like at the edge of the blood vessels, while plus disease describes enlarged and twisted blood vessels in the back of the eye. Doctors use all three features to judge how active the condition is and plan care.
Why does my baby need repeated ROP eye exams?
ROP may worsen without any signs that a parent can see from outside the eye. Examinations scheduled at exact intervals help the eye team detect changes before they threaten vision. Follow-up remains important after treatment because ROP can reactivate weeks or months later, especially after anti-VEGF injections.
What should I record after each ROP examination?
Ask for the Zone, Stage, and Plus or pre-plus status for each eye, along with how much retina is involved and the date of the next examination. Keep these details together so you can track changes over time. Ask the eye team what findings would require an earlier visit or treatment.

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References

References (7)
  1. 1

    International Classification of Retinopathy of Prematurity, Third Edition.

    Chiang MF, Quinn GE, Fielder AR, et al.

    Ophthalmology 2021; (128(10)):e51-e68 doi:10.1016/j.ophtha.2021.05.031.

    PMID: 34247850
  2. 2

    Posterior zone I retinopathy of prematurity: spectrum of disease and outcome after laser treatment.

    Katoch D, Dogra MR, Aggarwal K, et al.

    Canadian journal of ophthalmology. Journal canadien d'ophtalmologie 2019; (54(1)):87-93 doi:10.1016/j.jcjo.2018.03.005.

    PMID: 30851780
  3. 3

    Clinical Features of Spontaneous Regression of Retinopathy of Prematurity in China: A 5-Year Retrospective Case Series.

    Wang L, Li M, Zhu J, et al.

    Frontiers in medicine 2021; (8()):731421 doi:10.3389/fmed.2021.731421.

    PMID: 34532335
  4. 4

    OUTCOME OF EYES TREATED FOR RETINOPATHY OF PREMATURITY IN POSTERIOR ZONE I: An Eastern India Study.

    Padhi TR, Bhunia S, Shah M, et al.

    Retina (Philadelphia, Pa.) 2024; (44(6)):1073-1082 doi:10.1097/IAE.0000000000004067.

    PMID: 38346099
  5. 5

    OUTCOMES AFTER LASER VERSUS COMBINED LASER AND BEVACIZUMAB TREATMENT FOR TYPE 1 RETINOPATHY OF PREMATURITY IN ZONE I.

    Yoon JM, Shin DH, Kim SJ, et al.

    Retina (Philadelphia, Pa.) 2017; (37(1)):88-96 doi:10.1097/IAE.0000000000001125.

    PMID: 27347645
  6. 6

    Screening Examination of Premature Infants for Retinopathy of Prematurity.

    Fierson WM, , , et al.

    Pediatrics 2018; (142(6)) doi:10.1542/peds.2018-3061.

    PMID: 30478242
  7. 7

    Characteristics of Eyes Developing Retinal Detachment After Anti-vascular Endothelial Growth Factor Therapy for Retinopathy of Prematurity.

    Kondo C, Iwahashi C, Utamura S, et al.

    Frontiers in pediatrics 2022; (10()):785292 doi:10.3389/fped.2022.785292.

    PMID: 35463897

This page is for informational purposes only and does not constitute medical advice. Your baby's ophthalmology team should interpret the Zone, Stage, and Plus findings and set the follow-up plan.