RETINOPATHY RETINOPATHY OF OF

PREMATURITYPREMATURITY

DR. AJAY I DUDANIDR. AJAY I DUDANI M.S.,DNB,FCPS,DOMSM.S.,DNB,FCPS,DOMS

ASSOCIATE PROFFESSOR, K.J. SOMAIYA ASSOCIATE PROFFESSOR, K.J. SOMAIYA HOSPITAL,HOSPITAL,

CONSULTANT VITREORETINAL SURGEON,CONSULTANT VITREORETINAL SURGEON,BOMBAY HOSPITALBOMBAY HOSPITAL

ROP – why important?ROP – why important?

India shares 20% of world childhood India shares 20% of world childhood blindness.blindness.

Out of 100 preterm infants, 20-40 develop Out of 100 preterm infants, 20-40 develop ROP, out of which 3-7 become ultimately ROP, out of which 3-7 become ultimately blind.blind.

The incidence of ROP is increasing due to The incidence of ROP is increasing due to better survival of LBW & preterm babies better survival of LBW & preterm babies availing modern neonatal facilities and care.availing modern neonatal facilities and care.

With early detection and timely intervention With early detection and timely intervention blindness is preventable.blindness is preventable.

INTRODUCTIONINTRODUCTION

1st described by Terry in 1942 in 6 1st described by Terry in 1942 in 6 month premature infant.month premature infant.

Campbell first brought to notice Campbell first brought to notice relationship of intensive oxygen therapy relationship of intensive oxygen therapy & subsequent development of ROP.& subsequent development of ROP.

Kinsey clearly established that ROP was Kinsey clearly established that ROP was inversely proportional to birth weight.inversely proportional to birth weight.

TWO OVERLAPPING TWO OVERLAPPING PHASESPHASES

Acute phase- normal vasculogenesis is Acute phase- normal vasculogenesis is interrupted & a response to injury is interrupted & a response to injury is observable in retina.observable in retina.

Chronic or late proliferative phase- Chronic or late proliferative phase- membranes grow into vitreous causing membranes grow into vitreous causing tractional RD, ectopia or scarring of tractional RD, ectopia or scarring of macula leading to severe visual loss.macula leading to severe visual loss.

>90% cases undergo spontaneous >90% cases undergo spontaneous regression, <10% cases develop regression, <10% cases develop significant cicatrization.significant cicatrization.

RISK FACTORSRISK FACTORS Prematurity & LBW.Prematurity & LBW.

(<31 wks,<1500gms)(<31 wks,<1500gms)

<28wks,<1000gms are at highest risk.<28wks,<1000gms are at highest risk. Factors causing shift in oxygen –Hb Factors causing shift in oxygen –Hb

dynamics like :dynamics like :

-multiple blood transfusions-multiple blood transfusions

-intraventricular haemorrhage-intraventricular haemorrhage

- cyanosis, apnoea , seizures- cyanosis, apnoea , seizures

- neonatal sepsis - neonatal sepsis

- shock - shock

Endogenous antioxidant deficiency.(vit E and Endogenous antioxidant deficiency.(vit E and others)others)

In multiple pregnancy, those with better wt gain In multiple pregnancy, those with better wt gain develop severe ROPdevelop severe ROP

Supplemental oxygen did not cause additional Supplemental oxygen did not cause additional progression of prethreshold ROP (STOP-ROP)progression of prethreshold ROP (STOP-ROP)

Ambient light reduction in hospital nurseries Ambient light reduction in hospital nurseries has no effect on the development of ROP has no effect on the development of ROP (LIGHT-ROP)(LIGHT-ROP)

Progression to threshold ROP may be influenced Progression to threshold ROP may be influenced by genetic differences in VEGF production.by genetic differences in VEGF production.

INTERNATIONAL INTERNATIONAL CLASSIFICATION OF ROPCLASSIFICATION OF ROP

On the basis ofOn the basis of Location on the retinaLocation on the retina Degree or stage of proliferationDegree or stage of proliferation Extent of proliferation in Extent of proliferation in

circumferential manner.circumferential manner.

STAGES OF ROPSTAGES OF ROP

1) Demarcation line1) Demarcation line 2) Demarcation ridge2) Demarcation ridge 3) Ridge with extraretinal fibrovascular3) Ridge with extraretinal fibrovascular

proliferationproliferation 4A) Subtotal RD4A) Subtotal RD

B) Subtotal RD involving the maculaB) Subtotal RD involving the macula 5) Total RD 5) Total RD

STAGES OF ROP

STAGE 2—DEMARCATION RIDGE

STAGE 3 ROP

STAGE 5 ROP

ZONES OF ROPZONES OF ROP

1.1. Circle drawn from center of the Circle drawn from center of the disc with a radius of twice the disc with a radius of twice the distance from the disc to the distance from the disc to the maculamacula

2.2. Nasal edge of zone 1 to the ora Nasal edge of zone 1 to the ora nasally and upto the equator nasally and upto the equator temporallytemporally

3.3. Temporal crescent of retina Temporal crescent of retina anterior to zone 2.anterior to zone 2.

ZONES OF ROP

PLUS DISEASEPLUS DISEASE

Increased dilatation & tortuosity of Increased dilatation & tortuosity of posterior pole vesselsposterior pole vessels

Iris vascular engorgementIris vascular engorgement Pupillary rigidityPupillary rigidity Vitreous haze Vitreous haze Normal posterior pole vasculature is a Normal posterior pole vasculature is a

reliable marker for the absence of stage 3, reliable marker for the absence of stage 3, when examination is difficult on account when examination is difficult on account

of poor pupillary dilatation in premature of poor pupillary dilatation in premature infantsinfants

PLUS DISEASE

THRESHOLD ROPTHRESHOLD ROP

Stage 3 disease involving >5 contiguous or Stage 3 disease involving >5 contiguous or 8 interrupted clock hrs with plus disease8 interrupted clock hrs with plus disease

PRETHRESHOLD ROPPRETHRESHOLD ROP Any extent stage 3 in zone 1 with or without Any extent stage 3 in zone 1 with or without

plus dsplus ds Zone 2 stage 3, < (5 contiguous or 8 Zone 2 stage 3, < (5 contiguous or 8

noncontiguous clock hrs)noncontiguous clock hrs) Zone 2 stage 3, 5 contiguous or 8 Zone 2 stage 3, 5 contiguous or 8

noncontiguous clock hrs without plus ds noncontiguous clock hrs without plus ds

RUSH DISEASE & RUSH DISEASE & CICATRICIAL ROPCICATRICIAL ROP

Unusually aggressive pattern which may Unusually aggressive pattern which may proceed very rapidly to severe ROP & RDproceed very rapidly to severe ROP & RD

CicatrizationCicatrization Sequelae include high myopia, vitreretinal Sequelae include high myopia, vitreretinal

membranes, areas of irregular pigmentation membranes, areas of irregular pigmentation in periphery & dragging of vessels including in periphery & dragging of vessels including macula to periphery. Falciform retinal folds in macula to periphery. Falciform retinal folds in severe casessevere cases

In most severe cases, totally detached retina In most severe cases, totally detached retina forms thickened mass behind lens- Retrolental forms thickened mass behind lens- Retrolental fibroplasiafibroplasia

CICATRITIAL ROP

TEMPORAL DRAGGING OF MACULA

FALCIFORM VITREORETINAL FOLD

TREATMENT OF ROPTREATMENT OF ROP

CryotherapyCryotherapy Laser therapyLaser therapy Surgical managementSurgical management

Threshold ROP is treated within 72hrs Threshold ROP is treated within 72hrs by ablationby ablation

of the avascular retina by laser or of the avascular retina by laser or cryotherapy. cryotherapy.

CRYOTHERAPYCRYOTHERAPY

AdvantagesAdvantages Less expensiveLess expensive Widely availableWidely available Faster to administerFaster to administer Can bypass the thick vasculosa lentisCan bypass the thick vasculosa lentis

It acts by eliminating the production of It acts by eliminating the production of

vasoproliferative factor from avascular vasoproliferative factor from avascular retina retina

Multicentric Cryotherapy Trial for ROP Multicentric Cryotherapy Trial for ROP concluded that cryotreatment reduces concluded that cryotreatment reduces the risk of unfavourable retinal & the risk of unfavourable retinal & functional outcome from threshold functional outcome from threshold ROPROP

CRYO-ROP Study Group – 15 yr follow CRYO-ROP Study Group – 15 yr follow up of 254 survivors from 291 preterms up of 254 survivors from 291 preterms with birth wts <1251gms & severe with birth wts <1251gms & severe threshold ROP in one or both eyes.threshold ROP in one or both eyes.

Treated Treated eyeseyes

30%30%

4.5%4.5%

44.5%44.5%

Control Control EyesEyes

51.9%51.9%

7.7%7.7%

64.3%64.3%

Unfavourable structural outcome

Between 10 &15 yrs of age, new retinal folds detachments or obscuring of view of posterior pole

Unfavourable visual acuity outcomes

RESULTS OF CRYO ROP RESULTS OF CRYO ROP STUDYSTUDY

Benefit of cryo for treatment of Benefit of cryo for treatment of threshold ROP for both structural & threshold ROP for both structural & visual functions was maint ained visual functions was maint ained across 15 yrs of follow upacross 15 yrs of follow up

New detachments in eyes with good New detachments in eyes with good structural findings at 10, emphasize structural findings at 10, emphasize value of long term regular follow up value of long term regular follow up of eyes with threshold ROPof eyes with threshold ROP

LASER LASER PHOTOCOAGULATIONPHOTOCOAGULATION

AdvantagesAdvantages Ease of deliveryEase of delivery No need of general anaesthesiaNo need of general anaesthesia More effective in zone1 (posterior More effective in zone1 (posterior

pole ds)pole ds) Less irritatingLess irritating Scars less pronouncedScars less pronounced Less induce myopiaLess induce myopia

OS AT 34wks with ROP

4wks Post Laser Regressed ROP

EARLY TREATMENT OF EARLY TREATMENT OF ROPROP

(ETROP)(ETROP)This group supported retinal ablative therapy forThis group supported retinal ablative therapy for

Type 1 ROP- Type 1 ROP- Zone 1, any Stage with plus dsZone 1, any Stage with plus ds Zone 1, Stage 3 without plus ds orZone 1, Stage 3 without plus ds or Zone 2, Stage 2 or 3 with plus dsZone 2, Stage 2 or 3 with plus ds

And a wait & watch approach forAnd a wait & watch approach for

Type 2 ROPType 2 ROP Zone 1, Stage 1 or 2 without plus dsZone 1, Stage 1 or 2 without plus ds Zone 2, Stage 3 without plus dsZone 2, Stage 3 without plus ds

SURGICAL SURGICAL MANAGEMENTMANAGEMENT

Stage 4A & 4B – scleral bucklingStage 4A & 4B – scleral buckling Stage 5 – difficult, anatomical & visual Stage 5 – difficult, anatomical & visual

results disappointing.results disappointing. Lensectomy & pupilloplasty, mandatory for Lensectomy & pupilloplasty, mandatory for

peripheral approachperipheral approach Retrolental membranes dissected from center Retrolental membranes dissected from center

to periphery with minimal traction on retinato periphery with minimal traction on retina No attempt to drain SRF, Air fluid exchange No attempt to drain SRF, Air fluid exchange

donedone Funnel configuration useful to prognosticate Funnel configuration useful to prognosticate

surgical outcomesurgical outcome

PREOP STAGE 5 ROP

ATTACHED POSTERIOR POLE 12 WKS POST OP

REASONS FOR POOR REASONS FOR POOR POST-OP OUTCOMEPOST-OP OUTCOME

Late disease identification & Late disease identification & presentationpresentation

Lack of prior treatment (cryo or Lack of prior treatment (cryo or laser)laser)

Narrow configuration of RDNarrow configuration of RD Associated ocular abnormalities like Associated ocular abnormalities like

cataract & glaucomacataract & glaucoma

SCREENING GUIDELINES SCREENING GUIDELINES FOR ROP FOR ROP

First done at 32 wks of gestation or 4-5 wks after First done at 32 wks of gestation or 4-5 wks after birth, whichever is earlierbirth, whichever is earlier

At 3 critical stagesAt 3 critical stages 32-34 wks32-34 wks 35-37 wks35-37 wks 39-42 wks39-42 wks If no ROP- incomplete vascularisation examined If no ROP- incomplete vascularisation examined

every 2 wklyevery 2 wkly Early ROP- (Zone 3 & 2 < than prethreshold)- wklyEarly ROP- (Zone 3 & 2 < than prethreshold)- wkly Prethreshold- twice wkly.Prethreshold- twice wkly. Last screening till complete retinal Last screening till complete retinal

vascularisation- 42-45 wksvascularisation- 42-45 wks

RETCAM FOR ROP RETCAM FOR ROP DOCUMENTATIONDOCUMENTATION

Wide angle digital paediatric retinal Wide angle digital paediatric retinal imaging systemimaging system

Mobile, self contained system for use in Mobile, self contained system for use in nursery, ICU, O.Tnursery, ICU, O.T

Easily used by technicians or nursesEasily used by technicians or nurses Avoids stress & expertise of I/O Avoids stress & expertise of I/O

examination & indentation, but as specific examination & indentation, but as specific and sensitive as I/Oand sensitive as I/O

Useful for diagnosis, F/U & documentation Useful for diagnosis, F/U & documentation

RETCAM FOR ROP DOCUMENTATION

CONCLUSIONCONCLUSION

Timely screening, referral & treatment is key toTimely screening, referral & treatment is key to prevent blindnessprevent blindnessWith,With, ROP screening programsROP screening programs Awareness amongst ophthalmologists & Awareness amongst ophthalmologists &

neonatologistsneonatologists Referral servicesReferral services Advanced vitreoretinal surgical techniquesAdvanced vitreoretinal surgical techniquesVisual outcome of child suffering from ROP will Visual outcome of child suffering from ROP will

bebe brighter!brighter!