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Retinopathy Of Prematurity
(ROP)
BY CYNTHIA OLOO
Overview
• Introduction
• Risk factors
• Pathophysiology
• Forms of ROP
• International classification of ROP
• Treatment and follow-up
• Complication
• Ddx
Introduction
• Is a vasoproliferative retinal disorder unique to premature infants
• First described in 1950s
• ROP is leading cause of childhood blindness
• Second only to cerebral visual impairment
Risk factors
• Premature births < 30 weeks gestational age
• Low birth weight <1500g
• Systemic illness e.g anaemia, sepsis ,low vitamin E levels
Pathophysiology
• Retinal vascularization begins during week 16 of gestation
• Mesenchymal tissue grows centrifugally from the optic disc
• Reaching nasal ora serrata by 36 weeks
• Temporal ora serrata by 40 weeks
• ROP results in abnormal growth of these retinal blood vessels in
premature infants
• Because of complex interaction between VEGF and insulin growth
factor (IGF-1)
• Can occur in phases
Phases of interaction of VEGF and IGF-1
PHASE 1
Occur at 22-30 weeks gestational age
Retina is hyperoxic (relative to intrauterine oxygen levels)
VEGF levels are low
Retinal blood vessels stop growing; this arrested growth is
1. worsened by high oxygen levels
2. Low levels of IGF-1
3. Correlated with poor weight gain
PHASE 2
Occurs at 31-34 weeks gestation
Avascular retina is hypoxic
VEGF levels rise
Neovascularization occur
International Classification of ROP
• 1. Location
• Zone I ( posterior pole)
• A circle centered on the optic disc with a radius equal to twice the
distance from the center of the disc to the macula
• Clinically, the temporal edge of zone I is visible with a 25 or 28 D lens
• With the other edge of the field of view centered on the nasal disc
margin
• Zone II
• A circle center on the optic disc with a radius equal to the distance
from the center of the optic disc to the nasal ora serrata
• Zone III;
• Residual cresent anterior to zone II
2. Extent-specified as hours of the clock as observers looks at each eye
Grading of ROP according to location
• Stages for active ROP
STAGE 1-Demarcation line presence
STAGE 2-Ridge formation
STAGE 3- Ridge with Extraretinal vascular proliferation
STAGE 4A-Partial extrafoveal retinal Detachment
 B-Retinal detachment including fovea
STAGE 5- Total retinal detachment
3.Stages
Stage 1- presence of demarcation line
stage 2 ROP- ridge formation
stage 3 ROP-Ridge with extraretinal fibrovascular
proliferation
stage 4A ROP- partial extrafoveal retinal
detachment
stage 5 ROP- total retinal detachment
Forms of ROP
1. Pre Plus and plus disease
2. Aggressive posterior ROP (AP-ROP)
3. Cryotherapy for ROP (CRYO-ROP)
4. Early treatment ROP (ETROP)
PRE PLUS DISEASE;
• Dilatation and tortuosity that are abnormal but less than that seen in
standard photograph
PLUS DISEASE;
• Marked arteriolar tortuosity and engorgement of the posterior pole
vasculature at least 2/3 of posterior fundus
• Is diagnosed by comparison with standard photograph
• It implies vascular shunting through the new vessels
• Other features include failure of the pupil to dilate and vitreous haze
plus disease pre-plus disease
• Signifies severe disease
AGGRESSIVE POSTERIOR ROP;
• Formerly known as Rush disease
• Severe form of ROP
• Defined as zone I or posterior zone II disease
• Associated with plus disease
• Involving all 4 quadrants of the posterior pole retina vessels, shunt
vessels and flat neovascularization at the junction between
Vascularised and avascularised retina
• Without treatment AP-ROP progress to stage 4 or 5 ROP
CRYOTHERAPY FOR ROP ; TRIAL
• Threshold disease as 5 contiguous or 8 total clock hours of stage 3
ROP in zone I or II in the presence of plus disease
EARLY TREATMENT ROP TRIAL;
• Prethreshold disease as all zone I and II ROP changes
• That do not meet threshold treatment criteria
Type of ETROP classification
1. Type 1
• Zone I , any stage ROP with plus disease
• Zone I, stage 3 ROP without plus disease
• Zone II, stage 2 or 3 ROP with plus disease
• Treatment is recommended within 72 hours
2. Type 2
• Zone I, stage 1 or 2 ROP without plus disease
• Zone II, stage 3 ROP without plus disease
• Requires observation
Diagnosis
• SCREENING, who to be screened;
Babies born at or before 30-32 weeks gestational age
Babies weighing 1500g or less
Severe-illness for other premature
• EXAMINATION,
Dilation of pupil- combination eye drop of relatively low concentration
Cyclopentolate 0.2-0.5% and phenylephrine 1-2.5%
Anaesthetic is instilled
Neonatal eyelid speculum is used
Monitor apnoea
Can use indirect ophthalmoscope with 28 D lens or2.2 panfundoscopic
volk lens and scleral depression
Wide field retinal camera with careful oversight
• RECOMMENDATION INTERVALS FOLLOW-UP EYE EXAMINATION FOR
ROP WITHOUT EYE DISEASE
1 week or less
Immature vascularization :zone I or posterior zone II
Stage 1 or 2ROP : zone I
Stage 3 ROP :zone II
Presence or suspected presence of aggressive posterior ROP
1 or 2 weeks
Immature vascularization : posteror zone II
Stage 2 ROP :zone II
Unequivocally regressing ROP :zone I
2 Weeks
Stage 1 ROP :zone II
Immature vascularization :zone II
Unequivocally regressing ROP :zone II
2 to 3 weeks
Stage 1 or 2 ROP :zone III
Regressing ROP: zone III
• CRITERIA FOR DISCONTINUATION OF ROP SCREENING EXAMINATION
1.Full vascularized retina
2. Zone III vascularization without previous zone I or II ROP
3. Lack of development of prethreshold or worse ROP by 50 weeks
4. Regression of ROP in zone III without abnormal vascular
TREATMENT
• Laser ablation- of avascular peripheral retina, replaced CRYOTHERAPY
• Intravitreal anti-VEGF agents- The BEAT-ROP (Bevacizumab Eliminate
the Angiogenic Threat of Retinopathy Of Prematurity)
• Pars plana vitrectomy- for tractional retinal detachment not involving
the macular (stage 4A) -90% success & visual outcome .
-stage 4B -60%
-stage 5- 20%
• Lensectomy or anterior vitrectomy
COMPLICATION
• Early
Myopia
Strabismus
Ambylopia
Refractive error
Dragging of the macula
• Late
• pseudoexotropia
• Glaucoma
• Early development of cataracts
• Phthisis bulbi
DDX
• Persistent hyperplastic primary vitreous
• Familial exudative vitreoretinopathy
REFERRENCE
• KANSKI 9 TH EDITION
• AMERICAN ACADEMY, PEADIATRIC OPHTHALMOLOGY
• NATIONAL GUIDELINE OF SCREENING,PREVENTION AND TREATMENT
OF ROP

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Retinopathy Of Prematurity.By Cynthia Oloo JKUAT student Bsc comprehensive ophthalmology and cataract surgery

  • 2. Overview • Introduction • Risk factors • Pathophysiology • Forms of ROP • International classification of ROP • Treatment and follow-up • Complication • Ddx
  • 3. Introduction • Is a vasoproliferative retinal disorder unique to premature infants • First described in 1950s • ROP is leading cause of childhood blindness • Second only to cerebral visual impairment
  • 4. Risk factors • Premature births < 30 weeks gestational age • Low birth weight <1500g • Systemic illness e.g anaemia, sepsis ,low vitamin E levels
  • 5. Pathophysiology • Retinal vascularization begins during week 16 of gestation • Mesenchymal tissue grows centrifugally from the optic disc • Reaching nasal ora serrata by 36 weeks • Temporal ora serrata by 40 weeks • ROP results in abnormal growth of these retinal blood vessels in premature infants • Because of complex interaction between VEGF and insulin growth factor (IGF-1) • Can occur in phases
  • 6. Phases of interaction of VEGF and IGF-1 PHASE 1 Occur at 22-30 weeks gestational age Retina is hyperoxic (relative to intrauterine oxygen levels) VEGF levels are low Retinal blood vessels stop growing; this arrested growth is 1. worsened by high oxygen levels 2. Low levels of IGF-1 3. Correlated with poor weight gain
  • 7. PHASE 2 Occurs at 31-34 weeks gestation Avascular retina is hypoxic VEGF levels rise Neovascularization occur
  • 8. International Classification of ROP • 1. Location • Zone I ( posterior pole) • A circle centered on the optic disc with a radius equal to twice the distance from the center of the disc to the macula • Clinically, the temporal edge of zone I is visible with a 25 or 28 D lens • With the other edge of the field of view centered on the nasal disc margin • Zone II • A circle center on the optic disc with a radius equal to the distance from the center of the optic disc to the nasal ora serrata
  • 9. • Zone III; • Residual cresent anterior to zone II 2. Extent-specified as hours of the clock as observers looks at each eye
  • 10. Grading of ROP according to location
  • 11. • Stages for active ROP STAGE 1-Demarcation line presence STAGE 2-Ridge formation STAGE 3- Ridge with Extraretinal vascular proliferation STAGE 4A-Partial extrafoveal retinal Detachment  B-Retinal detachment including fovea STAGE 5- Total retinal detachment
  • 12. 3.Stages Stage 1- presence of demarcation line
  • 13. stage 2 ROP- ridge formation
  • 14. stage 3 ROP-Ridge with extraretinal fibrovascular proliferation
  • 15. stage 4A ROP- partial extrafoveal retinal detachment
  • 16. stage 5 ROP- total retinal detachment
  • 17. Forms of ROP 1. Pre Plus and plus disease 2. Aggressive posterior ROP (AP-ROP) 3. Cryotherapy for ROP (CRYO-ROP) 4. Early treatment ROP (ETROP)
  • 18. PRE PLUS DISEASE; • Dilatation and tortuosity that are abnormal but less than that seen in standard photograph PLUS DISEASE; • Marked arteriolar tortuosity and engorgement of the posterior pole vasculature at least 2/3 of posterior fundus • Is diagnosed by comparison with standard photograph • It implies vascular shunting through the new vessels • Other features include failure of the pupil to dilate and vitreous haze
  • 20. • Signifies severe disease AGGRESSIVE POSTERIOR ROP; • Formerly known as Rush disease • Severe form of ROP • Defined as zone I or posterior zone II disease • Associated with plus disease • Involving all 4 quadrants of the posterior pole retina vessels, shunt vessels and flat neovascularization at the junction between
  • 21.
  • 22. Vascularised and avascularised retina • Without treatment AP-ROP progress to stage 4 or 5 ROP CRYOTHERAPY FOR ROP ; TRIAL • Threshold disease as 5 contiguous or 8 total clock hours of stage 3 ROP in zone I or II in the presence of plus disease
  • 23. EARLY TREATMENT ROP TRIAL; • Prethreshold disease as all zone I and II ROP changes • That do not meet threshold treatment criteria Type of ETROP classification 1. Type 1 • Zone I , any stage ROP with plus disease • Zone I, stage 3 ROP without plus disease • Zone II, stage 2 or 3 ROP with plus disease • Treatment is recommended within 72 hours
  • 24. 2. Type 2 • Zone I, stage 1 or 2 ROP without plus disease • Zone II, stage 3 ROP without plus disease • Requires observation
  • 25. Diagnosis • SCREENING, who to be screened; Babies born at or before 30-32 weeks gestational age Babies weighing 1500g or less Severe-illness for other premature • EXAMINATION, Dilation of pupil- combination eye drop of relatively low concentration Cyclopentolate 0.2-0.5% and phenylephrine 1-2.5% Anaesthetic is instilled
  • 26. Neonatal eyelid speculum is used Monitor apnoea Can use indirect ophthalmoscope with 28 D lens or2.2 panfundoscopic volk lens and scleral depression Wide field retinal camera with careful oversight
  • 27. • RECOMMENDATION INTERVALS FOLLOW-UP EYE EXAMINATION FOR ROP WITHOUT EYE DISEASE 1 week or less Immature vascularization :zone I or posterior zone II Stage 1 or 2ROP : zone I Stage 3 ROP :zone II Presence or suspected presence of aggressive posterior ROP
  • 28. 1 or 2 weeks Immature vascularization : posteror zone II Stage 2 ROP :zone II Unequivocally regressing ROP :zone I 2 Weeks Stage 1 ROP :zone II Immature vascularization :zone II Unequivocally regressing ROP :zone II
  • 29. 2 to 3 weeks Stage 1 or 2 ROP :zone III Regressing ROP: zone III
  • 30. • CRITERIA FOR DISCONTINUATION OF ROP SCREENING EXAMINATION 1.Full vascularized retina 2. Zone III vascularization without previous zone I or II ROP 3. Lack of development of prethreshold or worse ROP by 50 weeks 4. Regression of ROP in zone III without abnormal vascular
  • 31. TREATMENT • Laser ablation- of avascular peripheral retina, replaced CRYOTHERAPY • Intravitreal anti-VEGF agents- The BEAT-ROP (Bevacizumab Eliminate the Angiogenic Threat of Retinopathy Of Prematurity) • Pars plana vitrectomy- for tractional retinal detachment not involving the macular (stage 4A) -90% success & visual outcome . -stage 4B -60% -stage 5- 20% • Lensectomy or anterior vitrectomy
  • 32. COMPLICATION • Early Myopia Strabismus Ambylopia Refractive error Dragging of the macula • Late • pseudoexotropia • Glaucoma • Early development of cataracts • Phthisis bulbi
  • 33.
  • 34.
  • 35. DDX • Persistent hyperplastic primary vitreous • Familial exudative vitreoretinopathy
  • 36. REFERRENCE • KANSKI 9 TH EDITION • AMERICAN ACADEMY, PEADIATRIC OPHTHALMOLOGY • NATIONAL GUIDELINE OF SCREENING,PREVENTION AND TREATMENT OF ROP