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RETINOPATHY OF PREMATURITY
Dr Paavan Kalra
Department of Ophthalmology
S P Medical College
Bikaner
INTRODUCTION
โ€ข Disease of retinal vasculature in immature retina of
a premature neonate
โ€ข Results from interruption of normal vascularization
โ€ข Characterized by vaso-obliteration/ vaso cessation
followed by abnormal neovascularization and
ultimately cicatrisation
โ€ข Leading cause of childhood blindness in US
โ€ข Epidemic in low to middle income countries
like India โ€“ โ€˜THE THIRD EPIDEMICโ€™
โ€ข VISION 2020
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
EMBRYOLOGY
Retinal Vascularization begins โ€“ 16 weeks
Phase 1 โ€“ vasculogenesis โ€“ posterior pole
21-22 weeks
Phase 2 โ€“ angiogenesis - progression to ora serrata
Nasal ora โ€“ at term (37th week PMA)
Temporal ora โ€“ 38th week PMA (post natal)
Choroidal Vascularization complete by 21 weeks
โ€ข Hypoxic state in utero - mixed venous blood
PaO2 = 25 mm Hg ๏ƒ  VEGF
โ€ข Placental IGF 1
โ€ข Functional maturation of photoreceptors and
visual pathways at 28 to 32 weeks PMA.
Increase in metabolic demand at 28 to 32 weeks
PATHOGENESIS OF ROP
โ€ข Premature birth ๏ƒ  relative hyperoxia
(PaO2 = 60-80 mm Hg - low VEGF)
๏ƒ Low IGF
โ€ข PHASE I โ€“ birth to 32 weeks PCA
Vaso cessation
โ€ข PHASE II โ€“ after 32 weeks PCA - relative hypoxia
(high VEGF and low IGF)
Vaso proliferation
โ€ข REGRESSION / CICATRIZATION - >38 weeks PCA
(decrease in VEGF and increase inTGF beta)
IMMATURE
VASCULARIZATION
ACUTE ROP
REGRESSION
CICATRICIAL
DISEASE
INCOMPLETE
VASCULARIZATION
COMPLETE
VASCULARIZATION
RISK FACTORS
โ€ข Three crucial risk factors:
โ€“ Birth weight
โ€“ Gestational age
โ€“ Number of days oxygen administered
โ€ข Other risk factors:
โ€“ Multiple births
โ€“ Blood transfusions
โ€“ Intra Uterine Growth Retardation (IUGR)
โ€“ Failure of increase in weight
โ€“ Respiratory Distress Syndrome (RDS)
โ€“ Fluctuations in Sa O2
โ€“ Multiple apneic episodes
โ€“ Hypercarbia, Acidosis
โ€“ Sepsis
โ€“ Intra Ventricular Hemorrhage (IVH)
โ€“ Vit E deficiency
โ€“ Anemia
โ€“ Seizures.
CLASSIFICATION
โ€ข ZONE
โ€ข EXTENT
โ€ข STAGE
โ€ข PLUS
ZONES
EXTENT
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
STAGE 0 : IMMATURE VASCULARIZATION
STAGE I : DEMARCATION LINE
White in color
Abnormal branching or arcading of vessels posteriorly
STAGE II : RIDGE
Popcorn ๏ƒ  Isolated tufts of neovascular tissue
posterior to ridge
level of retina
White to pink
STAGE III : EXTRA RETINAL NEOVASCULARIZATION
MILD MODERATE SEVERE
STAGE IV a STAGE IV b
Macula Spared Macula involved
STAGE IV : PARTIAL RETINAL DETACHMENT
โ€ข STAGE IV RETINAL DETACHMENT
-Exudative, if early
-Tractional, as part of the change over from
acute to cicatricial disease.
-Rhegmatogenous detachments, years later
STAGE V : TOTAL RETINAL DETACHMENT
PLUS
โ€ข posterior venous dilation and arteriolar
tortuosity of at least 2 quadrants
โ€ข Arises gradually or very rapidly.
โ€ข Due to AV shunting mainly in ridge tissue
โ€ข Severity indicator
โ€ข Often associated
iris vessel engorgement
miosis
resistance to dilating medications
vitreous haze
tunica vasculosa lentis
Preplus disease: vascular abnormalities of the
posterior pole more than normal, less than
PLUS
The newly accepted preplus serves as a warning
CLINICALLY SIGNIFICANT TERMS
โ€ข Threshold ROP: CRYO ROP study
Zone I stage III with Plus
Zone II Stage III with Plus
( 5 contigous or total 8 clock hours)
โ€ข 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
๏ƒ Plus disease has increased in importance while the extent
(clock hours) of disease has diminished
โ€ข AP-ROP: aggressive posterior ROP
-Earlier known as โ€˜RUSH Diseaseโ€™
-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
TREATMENT
โ€ข RETINAL ABLATION
โ€“ CRYO
โ€“ LASER
โ€ข SCLERAL BUCKLING
โ€ข VITRECTOMY
โ€“ LENS SPARING
โ€“ With LENSECTOMY
โ€ข Under GA
โ€ข Distance from ridge to limbus noted
โ€ข Applied to the anterior avascular area wherever
ridge is present
โ€ข Ridge avoided
โ€ข SPOTS โ€“ Preferrably Transconjunctival
Contiguous
15 โ€“ 30
End point โ€“ creamy white
Copious irrigation
โ€ข Delivered through INDIRECT OPHTHALMOSCOPE + 28D
โ€ข Ridge Avoided
โ€ข SPOTS
Size =100 microns
Half burn width apart
End point โ€“ grade II gray burn
After LASER treatment
โ€ข zone 2 ROP
โ€“ generally regresses after a single treatment session.
โ€ข APROP
โ€“ may regresses but can reactivate with return of plus
disease
โ€“ progressive posterior hyaloidal contraction, and
progression to tractional posterior retinal detachment
โ€“ Post-treatment vigilance is necessary
AP ROP : Treatment in 2 Steps
Ist โ€“ upto Flat Neovascular Fronds
IInd โ€“ after regression of Fronds
(area beneath fronds continue to remain source
of VEGF and hence reappearance of disease)
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
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.
REGRESSION
Involution or disappearance
Gradual, can be very prolonged
Difficult to recognize early in its course
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
MACULAR ECTOPIA MACULAR FOLD
SCREENING
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
โ€ข Neonatal ICU
โ€ข Combined to neonatal checkup
โ€ข Monitoring of systemic status
โ€ข Antisepsis
โ€ข Warm, dry and fed
โ€ข Pupillary Dilation : 2.5% phenyl ephrine + 0.5%
Tropicamide โ€“ Twice, 15 mins apart 30 mins
before exam
โ€ข Speculum
โ€ข INDIRECT ophthalmoscope ( with small pupil
attachment ), 28/30D lens, scleral depressor
โ€ข PLUS DISEASE to be looked for before
speculum and scleral depression
END OF SCREENING
โ€ข COMPLETE VASCULARIZATION
โ€ข VASCULARIZATION in ZONE III (till 1 DD of
temporal ora) โ€“ if no previous ROP in zone I & II
โ€ข REGRESSED ROP ( b/w 40 -44 weeks PCA)โ€“ no
active disease left
โ€ข 45 weeks PCA with less than pre threshold
disease
RETCAM
โ€ข WIDE FIELD CONTACT RETINAL PHOTOGRAPHY โ€“ 130 deg
โ€ข Easy use by nurses and technicians
โ€ข Eliminates inter observer variability
โ€ข Teaching tool
โ€ข Overcomes logistics of screening
โ€ข More cost effective than examination
โ€ข Tele ophthalmological screening
โ€ข โ€œREFFERAL WARRANTED ROPโ€
โ€ข PHOTO ROP
โ€ข KID ROP
Fluorescein Angiography can be done
OPTICAL COHERENCE TOMOGRAPHY
THANK YOU

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Retinopathy of prematurity

  • 1. RETINOPATHY OF PREMATURITY Dr Paavan Kalra Department of Ophthalmology S P Medical College Bikaner
  • 2. INTRODUCTION โ€ข Disease of retinal vasculature in immature retina of a premature neonate โ€ข Results from interruption of normal vascularization โ€ข Characterized by vaso-obliteration/ vaso cessation followed by abnormal neovascularization and ultimately cicatrisation
  • 3. โ€ข Leading cause of childhood blindness in US โ€ข Epidemic in low to middle income countries like India โ€“ โ€˜THE THIRD EPIDEMICโ€™ โ€ข VISION 2020
  • 4.
  • 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 โ€“ 16 weeks Phase 1 โ€“ vasculogenesis โ€“ posterior pole 21-22 weeks Phase 2 โ€“ angiogenesis - progression to ora serrata Nasal ora โ€“ at term (37th week PMA) Temporal ora โ€“ 38th week PMA (post natal) Choroidal Vascularization complete by 21 weeks
  • 7. โ€ข Hypoxic state in utero - mixed venous blood PaO2 = 25 mm Hg ๏ƒ  VEGF โ€ข Placental IGF 1 โ€ข 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 โ€ข Premature birth ๏ƒ  relative hyperoxia (PaO2 = 60-80 mm Hg - low VEGF) ๏ƒ Low IGF โ€ข PHASE I โ€“ birth to 32 weeks PCA Vaso cessation โ€ข PHASE II โ€“ after 32 weeks PCA - relative hypoxia (high VEGF and low IGF) Vaso proliferation โ€ข REGRESSION / CICATRIZATION - >38 weeks PCA (decrease in VEGF and increase inTGF beta)
  • 9.
  • 11. RISK FACTORS โ€ข Three crucial risk factors: โ€“ Birth weight โ€“ Gestational age โ€“ Number of days oxygen administered โ€ข Other risk factors: โ€“ Multiple births โ€“ Blood transfusions โ€“ Intra Uterine Growth Retardation (IUGR) โ€“ Failure of increase in weight โ€“ Respiratory Distress Syndrome (RDS) โ€“ Fluctuations in Sa O2 โ€“ Multiple apneic episodes โ€“ Hypercarbia, Acidosis โ€“ Sepsis โ€“ Intra Ventricular Hemorrhage (IVH) โ€“ Vit E deficiency โ€“ Anemia โ€“ Seizures.
  • 13. ZONES
  • 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 ๏ƒ  Isolated tufts of neovascular tissue posterior to ridge level of retina White to pink
  • 19. STAGE III : EXTRA RETINAL NEOVASCULARIZATION
  • 21. STAGE IV a STAGE IV b Macula Spared Macula involved STAGE IV : PARTIAL RETINAL DETACHMENT
  • 22. โ€ข 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
  • 24.
  • 25. PLUS โ€ข posterior venous dilation and arteriolar tortuosity of at least 2 quadrants โ€ข Arises gradually or very rapidly. โ€ข Due to AV shunting mainly in ridge tissue โ€ข Severity indicator
  • 26. โ€ข 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 โ€ข Threshold ROP: CRYO ROP study Zone I stage III with Plus Zone II Stage III with Plus ( 5 contigous or total 8 clock hours) โ€ข 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 ๏ƒ Plus disease has increased in importance while the extent (clock hours) of disease has diminished
  • 29. โ€ข AP-ROP: aggressive posterior ROP -Earlier known as โ€˜RUSH Diseaseโ€™ -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
  • 30.
  • 31. TREATMENT โ€ข RETINAL ABLATION โ€“ CRYO โ€“ LASER โ€ข SCLERAL BUCKLING โ€ข VITRECTOMY โ€“ LENS SPARING โ€“ With LENSECTOMY
  • 32.
  • 33. โ€ข Under GA โ€ข Distance from ridge to limbus noted โ€ข Applied to the anterior avascular area wherever ridge is present โ€ข Ridge avoided โ€ข SPOTS โ€“ Preferrably Transconjunctival Contiguous 15 โ€“ 30 End point โ€“ creamy white Copious irrigation
  • 34.
  • 35. โ€ข Delivered through INDIRECT OPHTHALMOSCOPE + 28D โ€ข Ridge Avoided โ€ข SPOTS Size =100 microns Half burn width apart End point โ€“ grade II gray burn
  • 36. After LASER treatment โ€ข zone 2 ROP โ€“ generally regresses after a single treatment session. โ€ข APROP โ€“ may regresses but can reactivate with return of plus disease โ€“ progressive posterior hyaloidal contraction, and progression to tractional posterior retinal detachment โ€“ Post-treatment vigilance is necessary
  • 37. AP ROP : Treatment in 2 Steps Ist โ€“ upto Flat Neovascular Fronds IInd โ€“ after regression of Fronds (area beneath fronds continue to remain source of VEGF and hence reappearance of disease)
  • 38.
  • 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
  • 42.
  • 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
  • 46.
  • 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. โ€ข Neonatal ICU โ€ข Combined to neonatal checkup โ€ข Monitoring of systemic status โ€ข Antisepsis โ€ข Warm, dry and fed
  • 49. โ€ข Pupillary Dilation : 2.5% phenyl ephrine + 0.5% Tropicamide โ€“ Twice, 15 mins apart 30 mins before exam โ€ข Speculum โ€ข INDIRECT ophthalmoscope ( with small pupil attachment ), 28/30D lens, scleral depressor โ€ข PLUS DISEASE to be looked for before speculum and scleral depression
  • 50. END OF SCREENING โ€ข COMPLETE VASCULARIZATION โ€ข VASCULARIZATION in ZONE III (till 1 DD of temporal ora) โ€“ if no previous ROP in zone I & II โ€ข REGRESSED ROP ( b/w 40 -44 weeks PCA)โ€“ no active disease left โ€ข 45 weeks PCA with less than pre threshold disease
  • 51. RETCAM โ€ข WIDE FIELD CONTACT RETINAL PHOTOGRAPHY โ€“ 130 deg โ€ข Easy use by nurses and technicians โ€ข Eliminates inter observer variability โ€ข Teaching tool โ€ข Overcomes logistics of screening โ€ข More cost effective than examination โ€ข Tele ophthalmological screening โ€ข โ€œREFFERAL WARRANTED ROPโ€ โ€ข PHOTO ROP โ€ข KID ROP Fluorescein Angiography can be done
  • 53.
  • 54.