Retinopathy of prematurity (ROP). Pediatrics disease

1. Retinopathy
Of Prematurity (ROP)
- Paediatric optometry
- SANIKA GURAV
ROLL NO. 05

2. WHAT IT IS?
 it is retinal vascular disease
 Earlier it was known as retrolental fibroplasia
 It is proliferative retinopathy
 It affect preterm infants who are exposed to
high concentration of oxygen.
 Bilateral disease, but severity of Both Eye can be
different

3. BASIC ANATOMY OF RETINA

4. PATHOGENESIS In normal term baby In preterm baby
Retina has no blood vessels till 4th
month of gestation, after which it start
arising from hyaloid vessels at optic
disc & then they grow to periphery of
retina.
These vessels reach nasal periphery
after 8 month of gestation & reach
temporal periphery 1 month after
birth.
This incompletely vascularize temporal
retina is more prone to oxygen damage
especially in preterm infants
In utero blood vessels grow nice & flat
along back of retina until it is fully
vascularize.
in preterm baby vessels start to grow
forward in posterior vitreous, if vessels
grow forward it will bleed, form scar in
vitreous & start pulling retina causing
retinal detachment
IGF-1 plays imp role in normal foetal
development of eye & cause VEGF to
grow blood vessels.
as eye’s retina has got used to high
oxygen & it was using it for nutrition
but when baby brought out of
incubation, retina is not getting high
oxygen like before so it again start
releasing VEGF to do
neovascularization

5. TYPES
TYPE 1 ROP TYPE 2 ROP
require treatment require observation
e.g., zone 1 + any
stage+
zone 1 + stage 3
without plus
zone 2 + stage 2/3+
e.g., zone 1 + stage
1/2 without plus
zone 2 + stage 3
without plus

6. CLINICAL FEATURES
Signs & symptom:
• avascular peripheral retina
• vitreous haemorrhage
• Retinal Detachment
• leukocoria
• In older children, VA decrease, amblyopia,
myopia, squint, macular dragging
Regression- in about 80% of infants, ROP spontaneously leave by
leaving few residues. It can occur in pt. with partial Retinal
Detachment.
Progression- Retinal Detachment is rare, its development precede by
signs: progression of plus disease development of fresh vitreous haze,
increase pre-retinal & vitreous hemorrhage, failure of pupil to dilate.
Factors that decide if it will progress or regress: overall health, birth
weight, stage of ROP at initial diagnosis, presence or absence of plus
disease

7. International classification of ROP
(ICROP) is based on
STAGES ZONES EXTENT
Stage 1 = demarcation line
formed, separate vascular &
avascular retina
Stage 2 = ridged
demarcation line
Stage 3 = ridged line with
extraretinal fibrovascular
proliferation/
neovascularization into
vitreous
Stage 4a = partial RD with
no macula involve, Retinal
Detachment develops when
infant is about 10 weeks old
Stage 4b = partial Retinal
Detachment with macula
involve
Stage 5 = total Retinal
Detachment
Zone 1 = twice the distance
from centre of disc to fovea
as radius. Any ROP in this
zone is usually severe.
Zone 2 = distance from
centre of disc to nasal ora
serrata as radius
Zone 3 = remaining
temporal periphery
denoted by no. of clock
hours. extent of disease is
described in segment as if
top of eye were 12 on face
of analogue clock e.g., stage
1 from 4:00 to 7:00
VASCULAR
CHARACTERISTICS
Pre Plus disease
Plus disease
Aggressive Posterior
ROP

8. STAGES OF ROP

9. ZONES OF ROP
12
3
6
9
12
EXTENT OF ROP

10. Plus disease –
• tortuous & dilated vessels in atleast
2 quadrants
• vitreous & anterior chamber haze
• immature blood vessels growing
over lens
• notated by adding plus sign (+) after
number of stage of ROP e.g., stage
2+
Pre-plus disease –
• It is a pre stage which develop in plus
disease.
• venous dilation & tortuosity is more
than normal but insufficient to be
defined as plus disease
Aggressive posterior ROP (AP-
ROP) –
• a.k.a ‘rush’ disease.
• requires immediate treatment.
• may progress rapidly to stage 5 ROP
without passing through other stages.
• Severe dilation & tortuous vessels.
• ROP in zone 1 with plus disease/ ROP in
posterior zone 2 with severe plus disease.
Threshold disease –
• needs laser therapy in less than 72
hour.
• it has 50% likelihood of progressing
to Retinal Detachment.
• Progression to stage 4/5 will cause
partial/ total loss of vision
Pre-threshold disease-
• zone 1 less than threshold
• zone 2+
• zone 2 + stage 3 +/ without + but
less than 5 continuous/ 8
discontinuous clock hours.

11. RISK FACTORS
• prematurity less than 32 week
of gestation
• birth weight less than 1500 gm
• supplemental oxygen therapy/
fluctuation in oxygen level
• poor postnatal growth
• Infection
• Respiratory problem
COMPICATIONS:
They are at great risk of developing strabismus,
acute angle closure glaucoma, cataract,
myopia, amblyopia, Retinal Detachment,
blindness later in life so they should be
examined yearly to help prevent/ detect them

12. INVESTIGATION
• when to examine- on discharge from hospital/ at 6–
8-week post birth
• Who to examine- birthweight under 1500 gm, any
baby with greater than 4 week of oxygen use, while
examination mention use of phenylephrine greater
than 2.0% or cyclopentolate greater than 0.25%
• Things needed for examine: infant lid speculum,
topical anaesthetic, indirect ophthalmoscope,
indirect lens, 28 D
• Examination is done to determine how far retinal
blood vessels have grown (zone), whether vessels
are growing flat along wall of eye (stage). Once
vessels have grown into zone 3 it is safe to
discharge child from further screening for ROP.
SCREENING
• Most useful time to screen infant is between 7 & 9
week of life bcoz ROP rarely appear for 1st time
after 9 week & Retinal Detachment hardly develop
before that. Pupil of premature infant dilated with
cyclopentolate 0.5%, phenylephrine 2.5%
• Immature retina labelled when vessels are short of
1 disc diameter of nasal/temporal ora but ROP is
not developed
• ROP when present should be classified

14. DIFFERENTIAL DIAGNOSIS
Familial exudative
vitreoretinopathy (FEVR) –
Genetic disorder that disrupt
retinal vascularization in full
term infants. Can be present
within 1st week of life as well
Norrie disease- rare X linked
disorder with fibrovascular
changes that appear similar to
ROP. Appears at birth &
progresses throughout infancy
Coats disease- X linked
condition. Leads to total Retinal
Detachment. Often unilateral &
found in males
Retinoblastoma – most
common intraocular malignant
tumor.
Nerve cells in retina develop
genetic mutation
It occurs before 5 years.

15. • Laser photocoagulation-
Done with laser indirect ophthalmoscope.
For patient with high risk of ROP.
Post laser – antibiotic & steroid eyedrops prescribed for week.
burn avascular retina, can cause peripheral vision loss.
• Cryotherapy- reduce adverse reaction by 50%. cryo probe to freeze avascular retina. earlier technique in which
regional retinal destruction was done using probe to freeze desired areas.
• Anti-VEGF injection- intravitreal injection, but condition can reoccur. E.g. bevacizumab (Avastin)- supportive
measure in aggressive posterior ROP. reduction in use of anaesthesia, preservation of peripheral retina, reduced
incidence of high refractive error
• Surgical repair of Retinal Detachment either by scleral buckling/ vitrectomy/ both- patient with stage 4a, 4b & 5
require lens sparing vitrectomy along with laser photocoagulation & retinal reattachment measures. for severe
ROP as it may progress into Retinal Detachment
TREATMENT

16. SCLERAL BUCKLING
VITRECTOMY + LASER

17. FOLLOW-UP
Immature retina but
no ROP = biweekly
Stage 1 & 2 (zone 1 &
2) = weekly
Stage 3 (zone 2) =
weekly
In older children =
need examination
every 6 mt. to rule out
any further
complication

18. MANAGEMENT:
• premature not
placed in incubator
with oxygen
concertation more
than 95%
• Avoid infections
• Early diagnosis &
treatment
PREVENTION:
• change in oxygen
level
• minimizing
operation
• Proper blood
transfusion &
monitoring

19. LATEST RESEARCH