1. Retinopathy of Prematurity
Catholic University of Korea Department of Ophthalmology & Visual Science SHIN Hae park

2. Retinopathy of Prematurity
Overview of ROP
Pathogenesis and Risk factor of ROP
Clinical features of ROP
Treatment of ROP

3. Retinopathy of Prematurity
Vasoproliferative disorder of the retina which occurs principally in the preterm infant
A significant morbidity in extremely immature infants whose survival rate continues to increase
Only in infants with an immature, incompletely vascularized retina
Outcome spectrum
minimal sequelae with no effect on vision in milder cases
bilateral irreversible total blindness in advanced cases

4. Retinopathy of Prematurity
In 1942, first identified, Retrolental fibroplasia
 became the largest cause of childhood blindness
 In 1950s, relationship between oxygen and ROP
 dramatic decrease in the incidence of ROP
( morbidity, mortality, CP, neurologic disorders..)
 In 1960s, ABGA & O2 monitoring
Improved survival rate of the smallest premature infants, who are at the highest risk of developing ROP
Est. 400 infants blinded each yr; 4300 with serious retinal scars.
ROP is a major challenge to all physicians dealing with premature infants.

5. Pathogenesis of ROP Local ischemia  Retinal neovascularization
Retina of full-term baby
Retina of premature baby
Local ischemia  Retinal neovascularization
Only in immature retina of premature baby

6. Pathogenesis of ROP Normal vasculogenesis in utero
relative hypoxia environment
with physiologic levels of VEGF
At the premature birth,
hyperoxic with decreased levels of VEGF
vessel formation is halted
as the eyes grows, the avascular retina increases in size
peripheral area of hypoxic retina  VEGF
Pathological neovascularization
fibrosis, traction

7. Risk factors of ROP Prematurity Excess O2
: retinal vasoconstriction with vascular closure
(immature, incompletely vascularized retina)
Vitamin E deficiency
apnea, sepsis, transfusion
intraventricular H, RDS, PDA
Ethnicity ??
VEGF polymorphism??
Maternal age ??

8. Risk factors of ROP Prematurity < GA 37 weeks
LBW < 2500gm, VLBW < 1250gm
BW(g) %ROP (all) GA (wks) %ROP (all)
> >35 0
<34 weeks, <2000 gm
ROP Incidence: MHMC NICU

9. ROP screening No prophylactic treatment
Screening  appropriate detection / treatment !!!
Who ?? < GA 34 weeks, 2000 gram
When ?? postnatal 4-6 weeks, GA weeks
Subsequent review : 1~2 weekly interval
 until retinal vascularization reaches zone 3

10. ROP screening
UK ROP Guideline

11. Stage1 demarcation line
Stage 2 ridge
Stage 3 EFP
age 4-6 weeks
GA weeks
Threshold disease
Pph. Laser application
Complete ablation of pph. retina
Producing VEGF
Stage 4 partial RD
Stage 5 total RD
Regressed ROP

12. Clinical features of ROP
LOCATION of ROP
* 3 concentric zones
Zone 1 : an imaginary circle
the radius is twice
the distance from
disc to macula
Zone 2 : from edge of zone 1
radius extends to
nasal ora serrata
Zone 3 : residual temporal
crescent*

13. STAGE of ROP

14. ROP – Extent of disease
* o/c 위치로 표시하거나 30’ sector로 표시(12o/c sector는 12~1o/c) 12 12 I I I 9 3 9 II
III 3
III II
Ora
serrata
Macula 6 6
Optic disc
<Right eye>
<Left eye>

15. Zone II, stage III with plus (+)
Plus disease - engorgement and tortuosity of the blood vessels near the optic nerve.
Also includes growth and dilation of abnormal blood vessels on the surface of the iris, rigidity of the iris, and vitreous haze (exudate along the retinal vessels).
Can accompany any stage, but indicates greater likelihood of progression
Zone I, stage III with plus (+)

16. EXTENT of ROP Rush ROP , Zone I ROP AP-ROP ( aggressive posterior ROP)
Zone 1에 주로 위치(post. location)
Plus disease가 뚜렷히 나타남.
Ill-defined nature of retinopathy
주로 Stage 1~3을 거치지 않고 바로 stage 5까지 진행해버림
(수 주의 기간을 거치지 않고 수일 내로 진행!)
Increased numbers of AP-ROP cases
강 하 람, 윤일현, 홍정은아가
김 지 수, 김 태 은, 한미희아가

17. 강 O 람
BHx) 31+6 wks kg -PC/SD
Prominence of plus sign
Shunting from vessels to vessels
Significant dilatation and tortuisity of all vessels
No classical course stage I23
Featureless demarcation line

18. When to treat ROP Pre-threshold disease Threshold disease
Zone 1, any stage
Zone 2, plus disease, with stage 1, 2
 ROP with increased likelihood of progression to RD, if left untreated
Threshold disease
Stage 3(extraretinal frbrovascular proliferation, EFP) with
5 continuous clock hours or
8 cumulative clock hours in zone 1 or 2, with plus disease
ROP with 50% likelihood of progression to RD, if left untreated
Emergency laser treatment within 3 days

19. Treatment of ROP
Treatment of ROP is directed at treating the underlying pathogenesis by decreasing VEGF levels
 Cryotherapy / laser ablation of peripheral avascular retina that produce s the VEGF
 ant-VEGF therapy (Avastin)

20. Treatment of ROP CRYO-ROP study (1988)
LASER-ROP study (1994) , ETROP study
 current trend : peripheral laser ablation
threshold disease
zone I ROP + plus
Scleral buckling encircling
Vitrectomy

21. Prognosis of ROP
Active ROP usually involutes without progressing to RD.
80%는 자발적으로 소멸, residua도 거의 안 남김.

22. Prognosis of ROP Progression despite appropriate laser Tx
: % of cases of threshold ROP
: 50 % of AP-ROP
Other ocular findings of Regressed ROP
- Myopia (근시), anisometropia(부등시), amblyopia (약시)
- Strabismus
- Retinal detachment

23. United states 15.58 % Taiwan 13 % 1000명 premature baby 130 ROP (13%)
26명 Threshold (20%)
2.6 + α명 (RD로 진행 10%)

24. Long-term visual outcomes of laser-treated threshold retinopathy of prematurity: a study of refractive status at 7 years.

25. Conclusion Appropriate screening : appropriate timing / indication
: stage 3 /threshold disease 를 놓치지 말아야 !!!
Threshold  laser ablation
Zone I ROP, Rush ROP, AP-ROP !!
 rapid progression to RD, atypical course
 50% response to laser
Potentially vision-threatening disease !!

26. Thank you for your attention !!!