RETINOPATHY OF

PREMATURITY
AAO READING
INTRODUCTION

 ROP is a retinal  First described by Terry in 1942 as

vascular disease in “retrolental fibroplasia” due to the
premature infant appearance of a complete retinal
detachment behind the lens
EPIDEMIOLOGY

In the United States, ROP that is severe enough to require

treatment occurs in approximately 1100–1500 infants annually

Among these infants, 400–600 will never achieve vision better

than 20/200

Studies from India have reported ROP in 20% to 52% of screened

neonates.
RISK FACTORS

• Very low birth weight

MAJOR RF • Prematurity 
• High Oxygen concentration

ASSOCIATED • Apneu, Respiratory Distress Syndrome, Sepsis,

RF Blood Transfusion, Seizures
PATHOGENESIS
Retinal vascularization normally begins at 15 to 18 weeks gestation

Retinal blood vessels extend out from the optic disc (where the optic
nerve enters the eye) and grow peripherally

Vascularization in the nasal retina is complete at approximately 36 weeks

Vascular development usually is complete in the temporal retina by 40 weeks,

although maturation may be delayed until 48 to 50 in preterm infants
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PATHOGENESIS
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CLASSIFICATION

The classification consists of four components based on

The International Classification of retinopathy of
prematurity (ICROP)

• Location
• Severity
• Extent
• Plus Disease
LOCATION

N
SEVERITY (STAGE)
Stage 1 ROP Stage 2 ROP
STAGE 3 ROP
STAGE 4 ROP
STAGE 5 ROP
 Disease extent is The clock hours recorded
recorded as clock hours is the total clock hours

DISEASE EXTENT
1-12 hours or as twelve involved, not just the
30° sectors or 360° contiguous sectors
PLUS DISEASE

Plus disease can be present as a

major complicating factor at any
stage. It is characterized by :

• Significant level of venous dilation

• Arteriolar tortuosity of the Posterior retinal vessels.
• Two quadrants of the eye retina must be involved for
the changes to be characterized as plus disease
Pre-Plus Disease
• Pre-plus disease indicates posterior pole
tortuosity and dilatation that are not
sufficiently abnormal to reach the criteria
of plus disease, but is nevertheless
greater than that regarded as normal.
(More then normal, less than Plus) 
• Pre-plus disease may or may not progress
to plus disease.
PLUS DISEASE
WITH ROP
Classification of Acute ROP

Aggressive Posterior ROP

Threshold Disease

Pre Threshold Disease

Aggressive Posterior ROP

• Rapidly progresive and severe form of

ROP

• Observed most commonly in Zone I, it

may also occur in posterior Zone II

• Rush Disease
 Threshold Disease
5 or more contigous or 8 cumulative clock Stage 3 with plus disease either
hours of extraretinal neovascularisation zone 1 or 2

CRYO study risk of blindness can be

Risk of blindness-50% reduced to 25% with treatment
 Pre Threshold Disease
All zone I and II changes, except Zone II stage 1 and Zon
e II stage 2 without Plus Disease

Type 1 • Zone I, any stage ROP with Plus Disease

• Zone I, stage 3 ROP without Plus Disease
(High Risk Pre
• Zone II, stage 2 or 3 ROP with Plus Disease
Threshold)

Type 2 • Zone I, stage 1 or 2 ROP without Plus Disease

• Zone II, stage 3 ROP without Plus Disease
(Low Risk Pre Threshold)
 SCREENING RECOMENDATION
Screening Criteria

infants with a birth weight

All infants with a birth weight who are believed to be at
between 1500 g and 2000 g
of less than 1500 g or a high risk by their attending
or a gestational age greater
gestational age of 30 weeks pediatricians or neonatologists
than 30 weeks, with an
or less should be screened should be screened
unstable clinical course
 When to Screen ? 
First screening at 4 weeks of birth

ROP usually does not manifest before 2-3 weeks of birth

Infants with Gestational age < 28 weeks or < 1200 g birth should be
first screened at 2-3 weeks after delivery

Treatable ROP rarely occurs before 31 weeks

Screening Intervals

1 Week or Less Follow-up

• immature vascularization: zone I—no ROP
• immature retina extends into posterior zone II, near the boundary of
zone I
• stage 1 or 2 ROP: zone I
• stage 3 ROP: zone II
• the suspected presence of aggressive posterior ROP
Screening Intervals

1-2 Week Follow-up

• immature vascularization; posterior zone II
• stage 2 ROP: zone II
• unequivocally regressing ROP: zone I
Screening Intervals

2 Week Follow-up
• stage 1 ROP: zone II
• immature vascularization: zone II—no ROP
• unequivocally regressing ROP: zone II
Screening Intervals

2-3 Week Follow-up

• stage 1 or 2 ROP: zone III
• regressing ROP: zone III
Retinal screening examinations can usually be discontinued
when any one of the following criteria is :

Zone III retinal vascularization Full retinal vascularization in 50 weeks cronologist age and
attained without previous close proximity to the ora no prethreshold disease or
zone I or II ROP serrata for 360° more severe ROP

Regression of ROP (care must be

taken to ensure that there is no
abnormal vascular tissue present
that is capable of reactivation and
progression in zone II or III)
TREATMENT

The principle of treatment is to remove the stimulus for

growth of new blood vessels by ablating the peripheral
avascular retina. So, it may reduce the incidence of retinal
detachment and consequent blindness

Abolishes hypoxic of retina (mediated by over-

expression of VEGF). This results in regression of
established ROP
TIMING OF TREATMENT

Treatment is warranted
Ideally within 2-3 days within 48 hours of
of the diagnosis diagnosis of classical form
of disease 

The rational is that the

As soon as possible in disease can advance rapidly
and any delay in treatment
APROP will reduce the chances of
success
TYPE OF TREATMENT

Cryotherapy Laser treatment

(mostly outdated) (gold standard) 

Anti-VEGF (adjuvant Surgery (indicated for

before laser & surgery) stage 4 ROP)
Follow Up
ROP management Once treated, lifelong
doesn’t end with laser followup (yearly) is
or surgery mandatory

To rule out  refractive

Yearly followup till the
errors (most common),
age of 5 years is
amblyopia, cataract,
advisable
gaucoma, etc