This document discusses retinopathy of prematurity (ROP), which is a disease of the retinal vasculature that can occur in premature infants. It causes interrupted retinal vascularization that leads to abnormal neovascularization. The document covers the pathogenesis, risk factors, classification system, screening guidelines, and treatments for ROP, including retinal ablation using cryotherapy or laser, scleral buckling, and vitrectomy. Effective screening and timely treatment can help prevent ROP from progressing and potentially causing blindness.

1. RETINOPATHY OF PREMATURITY
Dr Paavan Kalra
Department of Ophthalmology
S P Medical College
Bikaner

2. INTRODUCTION
• Disease of retinal vasculature in immature retina of
a premature neonate
• Results from interruption of normal vascularization
• Characterized by vaso-obliteration/ vaso cessation
followed by abnormal neovascularization and
ultimately cicatrisation

3. • Leading cause of childhood blindness in US
• Epidemic in low to middle income countries
like India – ‘THE THIRD EPIDEMIC’
• VISION 2020

5. LANDMARK STUDIES
Corroborative study for role of
O2 - 1950s
ICROP - 1984, 1987, 2005
CRYO ROP
ETROP
LIGHT ROP
STOP ROP
HOPE ROP
PHOTO ROP
BEAT ROP

6. EMBRYOLOGY
Retinal Vascularization begins – 16 weeks
Phase 1 – vasculogenesis – posterior pole
21-22 weeks
Phase 2 – angiogenesis - progression to ora serrata
Nasal ora – at term (37th week PMA)
Temporal ora – 38th week PMA (post natal)
Choroidal Vascularization complete by 21 weeks

7. • Hypoxic state in utero - mixed venous blood
PaO2 = 25 mm Hg  VEGF
• Placental IGF 1
• Functional maturation of photoreceptors and
visual pathways at 28 to 32 weeks PMA.
Increase in metabolic demand at 28 to 32 weeks

8. PATHOGENESIS OF ROP
• Premature birth  relative hyperoxia
(PaO2 = 60-80 mm Hg - low VEGF)
Low IGF
• PHASE I – birth to 32 weeks PCA
Vaso cessation
• PHASE II – after 32 weeks PCA - relative hypoxia
(high VEGF and low IGF)
Vaso proliferation
• REGRESSION / CICATRIZATION - >38 weeks PCA
(decrease in VEGF and increase inTGF beta)

10. IMMATURE
VASCULARIZATION
ACUTE ROP
REGRESSION
CICATRICIAL
DISEASE
INCOMPLETE
VASCULARIZATION
COMPLETE
VASCULARIZATION

11. RISK FACTORS
• Three crucial risk factors:
– Birth weight
– Gestational age
– Number of days oxygen administered
• Other risk factors:
– Multiple births
– Blood transfusions
– Intra Uterine Growth Retardation (IUGR)
– Failure of increase in weight
– Respiratory Distress Syndrome (RDS)
– Fluctuations in Sa O2
– Multiple apneic episodes
– Hypercarbia, Acidosis
– Sepsis
– Intra Ventricular Hemorrhage (IVH)
– Vit E deficiency
– Anemia
– Seizures.

12. CLASSIFICATION
• ZONE
• EXTENT
• STAGE
• PLUS

13. ZONES

14. EXTENT

15. STAGE
Stage 0 Immature Vascularization
Stage 1 Line of demarcation
Stage 2 Ridge of elevated tissue
Stage 3 Extra retinal fibrovascular
proliferation (neovascularization)
Stage 4 Partial retinal detachment
4a Macula spared
4b Macula involved
Stage 5 Total retinal detachment
Open Open Funnel
Open Narrow Funnel
Narrow Open Funnel
Narrow Narrow Funnel

16. STAGE 0 : IMMATURE VASCULARIZATION

17. STAGE I : DEMARCATION LINE
White in color
Abnormal branching or arcading of vessels posteriorly

18. STAGE II : RIDGE
Popcorn  Isolated tufts of neovascular tissue
posterior to ridge
level of retina
White to pink

19. STAGE III : EXTRA RETINAL NEOVASCULARIZATION

20. MILD MODERATE SEVERE

21. STAGE IV a STAGE IV b
Macula Spared Macula involved
STAGE IV : PARTIAL RETINAL DETACHMENT

22. • STAGE IV RETINAL DETACHMENT
-Exudative, if early
-Tractional, as part of the change over from
acute to cicatricial disease.
-Rhegmatogenous detachments, years later

23. STAGE V : TOTAL RETINAL DETACHMENT

25. PLUS
• posterior venous dilation and arteriolar
tortuosity of at least 2 quadrants
• Arises gradually or very rapidly.
• Due to AV shunting mainly in ridge tissue
• Severity indicator

26. • Often associated
iris vessel engorgement
miosis
resistance to dilating medications
vitreous haze
tunica vasculosa lentis

27. Preplus disease: vascular abnormalities of the
posterior pole more than normal, less than
PLUS
The newly accepted preplus serves as a warning

28. CLINICALLY SIGNIFICANT TERMS
• Threshold ROP: CRYO ROP study
Zone I stage III with Plus
Zone II Stage III with Plus
( 5 contigous or total 8 clock hours)
• Prethreshold ROP: ETROP study
High risk Prethreshold
Zone I Stage I, II, III with plus
Stage III without plus
Zone II Stage II and III with plus
Plus disease has increased in importance while the extent
(clock hours) of disease has diminished

29. • AP-ROP: aggressive posterior ROP
-Earlier known as ‘RUSH Disease’
-posterior location,
-rapidly evolving preplus and plus disease
neovascularization that may be subtle or even intraretinal
in nature.
-Progress to stage IV & V in 2-3 weeks without passing
through characteristic stages II and III
- requires laser treatment more than once

31. TREATMENT
• RETINAL ABLATION
– CRYO
– LASER
• SCLERAL BUCKLING
• VITRECTOMY
– LENS SPARING
– With LENSECTOMY

33. • Under GA
• Distance from ridge to limbus noted
• Applied to the anterior avascular area wherever
ridge is present
• Ridge avoided
• SPOTS – Preferrably Transconjunctival
Contiguous
15 – 30
End point – creamy white
Copious irrigation

35. • Delivered through INDIRECT OPHTHALMOSCOPE + 28D
• Ridge Avoided
• SPOTS
Size =100 microns
Half burn width apart
End point – grade II gray burn

36. After LASER treatment
• zone 2 ROP
– generally regresses after a single treatment session.
• APROP
– may regresses but can reactivate with return of plus
disease
– progressive posterior hyaloidal contraction, and
progression to tractional posterior retinal detachment
– Post-treatment vigilance is necessary

37. AP ROP : Treatment in 2 Steps
Ist – upto Flat Neovascular Fronds
IInd – after regression of Fronds
(area beneath fronds continue to remain source
of VEGF and hence reappearance of disease)

39. SCLERAL BUCKLE
Under GA
Peritomy
2.5 mm encircling band passed beneath 4 Recti
One anchoring mattress suture applied in all
quadrants
Removal after 3-6 months

40. VITRECTOMY
Necessary in advanced cases
Lensectomy avoided
Peeling of membranes
Relieve of traction
No attempt to drain Sub Retinal Fluid
AIM : Ambulatory vision ie being able to see objects
and move around a room without stumbling or
bumping into obstacles.

41. REGRESSION
Involution or disappearance
Gradual, can be very prolonged
Difficult to recognize early in its course

43. CLASSIFICATION OF CICATRICIAL MACULAR CHANGES
MACULAR SCORE ANATOMICAL DEFINITION
MS-0 Normal
MS-1 Macular ectopia
MS-2 Macular fold
MS-3 Macular detachment
MS-4 Total detachment

44. MACULAR ECTOPIA MACULAR FOLD

45. SCREENING

47. INTERNATIONAL
Birth Weight <1500 g
GA < 32 weeks
Higher BW/GA with risks(unstable babies)
31 weeks PCA or 4 weeks CA, which is later
INDIAN
BW <1500 g / 1750 g
GA < 34-35 weeks
Higher BW/GA with risks(unstable babies)
31 weeks PCA or 4 weeks CA, which is earlier
(VLBW babies at 2-3 weeks CA)
RECOMMENDATIONS

48. • Neonatal ICU
• Combined to neonatal checkup
• Monitoring of systemic status
• Antisepsis
• Warm, dry and fed

49. • Pupillary Dilation : 2.5% phenyl ephrine + 0.5%
Tropicamide – Twice, 15 mins apart 30 mins
before exam
• Speculum
• INDIRECT ophthalmoscope ( with small pupil
attachment ), 28/30D lens, scleral depressor
• PLUS DISEASE to be looked for before
speculum and scleral depression

50. END OF SCREENING
• COMPLETE VASCULARIZATION
• VASCULARIZATION in ZONE III (till 1 DD of
temporal ora) – if no previous ROP in zone I & II
• REGRESSED ROP ( b/w 40 -44 weeks PCA)– no
active disease left
• 45 weeks PCA with less than pre threshold
disease

51. RETCAM
• WIDE FIELD CONTACT RETINAL PHOTOGRAPHY – 130 deg
• Easy use by nurses and technicians
• Eliminates inter observer variability
• Teaching tool
• Overcomes logistics of screening
• More cost effective than examination
• Tele ophthalmological screening
• “REFFERAL WARRANTED ROP”
• PHOTO ROP
• KID ROP
Fluorescein Angiography can be done

52. OPTICAL COHERENCE TOMOGRAPHY

55. THANK YOU