Retinopathy of

Prematurity
CHAPTER 8
AAO READING
SUBDIV VITREO-RETINA
 Definition

• Retinopathy of prematurity (ROP) is a complex disease

process initiated in part by a lack of complete or normal
retinal vascularization in premature infants.

• The absence of retinal vessels in portions of the immature

retina can result in retinal ischemia, leading to the release
of growth factors that promote vascular growth

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 Introduction

• As the disease progresses, vitreous hemorrhage and tractional

retinal detachment can occur
• The end stage of untreated ROP is the development of a
dense, white, fibrovascular plaque behind the lens and
complete tractional retinal detachment. (retrolental
fibroplasia)
• The main risk factors for developing this condition are
prematurity and low birth weight.

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 Epidemiology

• US —> 1100-1500 (severe ROP+Treatment)

• 400–600 infant —> VA <20/200

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 Terminology and Classification

• The Internasional Classification of retinopathy of

prematurity:
• Lokasi
• Severity
• Extent
• Plus Diaease

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 Location/zona

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 Severity/Stage

• Stage 1: Presence of demarcation line between

vascularized and nonvascularized retina.

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 Severity/Stage

• Stage 2: Presence of demarcation line that has height,

width, and volume (ridge); small, isolated tufts of
neovascular tissue lying on the surface of the retina,
commonly called ‘popcorn’, may be present

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 Severity/Stage

• Stage 3: Ridge with extraretinal fibrovascular proliferation

that may be mild, moderate, or severe as judged by the
amount of proliferative tissue present

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 Severity/Stage

• Stage 4: partial retinal detachment

• A. extrafoveal
• B. retinal detachment including fovea

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 Severity/Stage

• Stage 4: partial retinal detachment

• A. extrafoveal
• B. retinal detachment including fovea

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 Severity/Stage

• Stage 5 : total retinal detacment with funnel configuration;

combination are listed in order of frequency : top row
configuration is the most common configuration and bottom
row the least common configuration

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 Severity/Stage

• Plus disease: vascular dilatation (venous) and turtoisity

(arteriolar) of posterior retinal vessels in at least 2 quadrant
of the eye; iris vascular dilatation and venous haze may be
present

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Extent

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 Classification of Acute ROP

• Based on this terminology, ROP can be classified into several

disease stages and severities to aid clinicians in making
management and treatment decisions

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 Classification of Acute ROP
1. (Aggressive Posterior ROP)

• the presence of vascularization that ends in zone I or very

posterior zone II
• rapidly progresive
• severe form of ROP
• Rush disease

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 Classification of Acute ROP
2. (Threshold disease )

• 5 or more contigous or 8 cumulative clock hours of

extraretinal neovascularisation/stage3 with plus disease
either zone 1 or 2
• risk of blindness-50%
• acc to CRYO study risk of blindness can be reduced to 25%
with treatment

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Classification of Acute ROP
3. (Pre Threshold disease )

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 Patophysiology ROP (the onset of progress)

• PHASE 1: involve an initial insult such as hyperoxia,

hypoxia or hypotension at critical point in retinal
vascularization - causes vasoconstriction and dicrease blood
supply to developing retina with subsequent arrest in
vascular development

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 Patophysiology ROP (the onset of progress)

• PHASE 2: Neovascularization occurs (driven by VEGF-

upregulated by hypoxic avascular retina
• New vessels growing through the retina into the vitreous
• Area permeable leading to haemorrhage and eddema
• Extensive and severe extra retinal fibrovascular proliferation
leads to retinal detachment and abnormal retinal function

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 Natural Course

• ROP is a transient disease in the majority of infants, and

spontaneous regression occurs in 85% of eyes
• Threshold ROP eventually develops in approximately 7%–
10% of infants with a birth weight of 1250 g or less.

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 Associated Conditions and Late Sequelae

• myopia with astigmatism

• anisometropia
• strabismus amblyopia
• cataract
• glaucoma
• macular pigment epitheliopathy
• vitreoretinal scarring
• abnormal vitreoretinal interface/adhesions
tractional retinal detachment
• anomalous foveal anatomy
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 Screening Recommendations

• dilatedfundus examination using binocular indirect

ophthalmoscopy
• alternatively,there are telemedicine (photographic)
screening approaches

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 Screening Criteria

• Birth weight <1500g

• ≤ 30 weeks of gestation
• Between 1500-2000g or gestasional age >30 week
• Need for cardio respiratory support
• Prolonged oxygen threaphy
• Apnea of prematurity
• Anemia needing blood transfusion
• Neonatal sepsis

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 Screening Intervals

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 Screening Intervals
Retinal screening examinations can usually be discontinued when any one of the
following criteria is met:

1. Zone III retinal vascularization attained without previous zone I or II ROP (if
there is examiner doubt about the zone or if the postmenstrual age is less than 35
weeks, confirmatory examinations may be warranted);

2. Full retinal vascularization in close proximity to the ora serrata for 360°—that is,
the normal distance found in mature retina between the end of vascularization and
the ora serrata.

- This criterion should be used for all cases treated for ROP solely with bevacizumab;

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 Slide Fundus Photographic
Screening of ROP

• Ultra-wide-angle (120°) fundus photography of premature infant

eyes is very useful for documenting the findings, for assessing
any progression, and for use in fundus photographic screening.

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 Prevention and Risk Factors

Prevention: Risk Factor:

• optimal prenatal, perinatal, and • very sick premature infants
postnatal care
• sepsis
• Avoiding extremely low birth
• blood transfusion,
weight
• short gestational ages • a slow rate of postnatal weight
gain
• Diet

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 Treatment

• Cryotheraphy (mostly outdated)

• Laser treatment (gold standard)
• Anti VEGF (adjuvant) before laser and surgery-
newer approach in management
• Surgery

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 Laser and Cryoablation Surgery

• threshold or prethreshold type 1 ROP

• admisitered 72 hours
• conjunction with pediatric consultation
• systemic monitoring

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 Anti-VEGF Drugs

• The BEAT-ROP > compared laser therapy and anti vegf

for zone I or zone II posterior stage 3 ROP with plus
disease :
•a statistically significant treatment benefit for
bevacizumab was demonstrated for zone I ROP, whereas
zone II disease had similar outcomes with either
treatment.
• recurrences occurred significantly later with
bevacizumab than with laser therapy.
• Therefore, prolonged, close follow-up is essential.

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 Intravitreal injection technique

• can be performed at the patient’s bedside in the neonatal

intensive care unit (NICU)
• The neonatal nurse prepares the infant for the injection.
• The eye is sterilized with a 5% betadine solution.
• A sterile eyelid speculum for each eye is used to retract
the lids
• Theinjection site should be 1.5 mm posterior to the
limbus

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 Vitrectomy and Scleral Buckling Surgery

• Eyes with stage 4 ROP

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