Retinopathy of Prematurity Catholic University of Korea Department of Ophthalmology & Visual Science SHIN Hae park
Retinopathy of Prematurity Overview of ROP Pathogenesis and Risk factor of ROP Clinical features of ROP Treatment of ROP
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
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.
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
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
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 ??
Risk factors of ROP Prematurity < GA 37 weeks LBW < 2500gm, VLBW < 1250gm BW(g) %ROP (all) GA (wks) %ROP (all) 0-500 11 0-23 22 501-750 45 24-25 45 751-1000 42 26-27 45 1001-1250 12 28-29 20 1251-1500 4 30-31 6 1501-1750 0.8 32-33 1 1751-2000 0.4 34-35 0.2 >2000 0 >35 0 <34 weeks, <2000 gm ROP Incidence: MHMC NICU 1997-2001
ROP screening No prophylactic treatment Screening appropriate detection / treatment !!! Who ?? < GA 34 weeks, 2000 gram When ?? postnatal 4-6 weeks, GA 32- 34 weeks Subsequent review : 1~2 weekly interval until retinal vascularization reaches zone 3
ROP screening UK ROP Guideline
Stage1 demarcation line Stage 2 ridge Stage 3 EFP age 4-6 weeks GA 32- 34 weeks Threshold disease Pph. Laser application Complete ablation of pph. retina Producing VEGF Stage 4 partial RD Stage 5 total RD Regressed ROP
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*
STAGE of ROP
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>
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 (+)
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 - 19385116 강 하 람, 21639974 윤일현, 22162506 홍정은아가 - 22268381 김 지 수, 22345334 김 태 은, 22429004 한미희아가
19385116 강 O 람 BHx) 31+6 wks - 1.36kg -PC/SD Prominence of plus sign Shunting from vessels to vessels Significant dilatation and tortuisity of all vessels No classical course stage I23 Featureless demarcation line
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
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)
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
Prognosis of ROP Active ROP usually involutes without progressing to RD. 80%는 자발적으로 소멸, residua도 거의 안 남김.
Prognosis of ROP Progression despite appropriate laser Tx : 10-15 % of cases of threshold ROP : 50 % of AP-ROP Other ocular findings of Regressed ROP - Myopia (근시), anisometropia(부등시), amblyopia (약시) - Strabismus - Retinal detachment
United states 15.58 % Taiwan 13 % 1000명 premature baby 130 ROP (13%) 26명 Threshold (20%) 2.6 + α명 (RD로 진행 10%)
Long-term visual outcomes of laser-treated threshold retinopathy of prematurity: a study of refractive status at 7 years.
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 !!
Thank you for your attention !!!