trying to cover basics of the disease - includes pathology, presentation , classification, screening, management

1. Presenter – dravikakanathia Moderator – drshahanamazumdar Retinopathy of Prematurity

2. definition Vasoproliferative disorder of the retina which occurs in the preterm babies

3. Historical background First described by Terry in 1941 Originally known as retrolentalfibroplasia Term coined by Heath in 1951 Universally accepted classification in 1984

4. incidence MHMC - NICU

6. Low birth weight

7. Oxygen administration

8. Light exposurePostnatal sepsis Concurrent illnesses Anemia High carbon dioxide levels Seizures Bradycardia Apnea Mechanical ventilation Blood transfusions Intraventricular hemorrhage Multiple prenatal maternal factors including heavy smoking, diabetes, and preeclampsia

9. Pathogenesis When retinal vessels have not yet completed their centrifugal growth from the optic disc to the oraserrata Primitive endothelial cells (spindle cells) form cords that canalize into capillaries and further differentiate into arterioles and venules 16 weeks of gestation – primitive spindle cells gradually grow out over the surface of the retina 29 weeks – reached oraserrata ,spindle cells begin to form blood vessels

10. Pathogenesis cont… Blood vessels reach anterior edge of the retina and stop their progression at the time of birth During vasculogenesis any insult (preterm,increasedoxygen,hypoxia,toxin,shock) interrupts and demarcation line formed Primitive vessels pile up over this line forming ridge Anterior to this line retina has inadequate oxygen supply  new vessels and fibroblasts Fibrous scar  RD

11. ROP pathogenesis and suggested treatments. (a) Retina vessels in the process of their formation and progressive covering of the retina surface. (b) Hyperoxia at this formative stage suppresses VEGF and, consequently, results in regression of newly formed vessels. (c) Upon return to normal air, the ischemic retina upregulates VEGF to high levels, causing excessive formation of leaky vessels. To antagonize VEGF at this stage has been suggested as a strategy to reduce adverse vessel formation. (d) An alternative strategy proposed by Shih et al. (2) is to protect retina vessels from oxygen-induced obliteration through administration of PlGF-1.

12. Classification The International Classification of Retinopathy of Prematurity (ICROP) Based on 1. severity (Stage) 2. anatomical location (Zone) 3. extent (Clock hours)

13. zones Zone I – posterior pole - circle with centre at optic disc having radius twice the distance between disc and macula Zone II – middle zone - zone I to within one disc diameter oraserrata nasally , to equator temporally Zone III – outer zone - superior, inferior , temporal crests of remaining retina

15. staging Stage I – demarcation line Stage II – ridge Stage III – extra retinal vascular proliferation Stage IV – subtotal retinal detachment IV(a) extra foveal IV(b) foveal Stage V – total RD

16. Stage I – demarcation line First pathognomic sign Flat ,grey white , curvilinear separating avascular anterior from vascular posterior

17. Stage I

18. Stage II - ridge Volume , extending out of the retinal plane May change colour from white to pink Small neovascular tufts posterior to ridge (popcorn)

19. Stage II

20. Stage III – extra retinal proliferation Vessel growth into and above the ridge With progressive growth  into vitreous  VH

21. Stage III

22. Stage IV – subtotal retinal detachment

23. Stage IV

25. Open or closed anteriorly or posteriorly leukocoria

26. Stage V

28. Plus disease Progressive vascular disease (ICROP) Tortuous arteries and dilated veins over the posterior pole Marked peripheral vascular shunting Vascular engorgement of the iris Pupil does not dilate Vitreous haze

29. Plus disease

30. Threshold ROP Stage 3+ ROP Zones 1 / 2 5 contiguous / 8 non-contiguous clock hours Cryo – ROP Progression to RD  62% zone 2 90% zone 1 Urgent treatment Cryo / laser therapy in < 72 hours

31. Pre-threshold ROP ETROP (early treatment for ROP cooperation group) Type 1 (high risk) - zone 1 ROP , any stage , + - zone 2 , stage III - zone 2 , stage II/III , + Type 2 (low risk) - zone 1 , stage I/II - zone 2 , stage III

32. Rush disease Aggressive posterior ROP, AP – ROP Zone 1 ROP Signs of plus disease

33. Rush disease

34. Regressed ROP Long standing cases Residual / cicatricial changes – Peripheral retina Posterior pole

35. Peripheral retina Pigmentary changes Thin retina/peripheral folds Vitreous membrane with / without attachement to retina Lattice like degenerations Retinal breaks Retinal detachment

36. Posterior pole Straightening of vascular arcades Dragging of macula and disc

37. Screening guidelines All premature babies BW < 1500 gms All neonates < 32 weeks High risk babies Exposed to oxygen Multiple blood transfusion Sepsis RDS Apnoea Pneumonitis Neonatalogist, temperature controlled room

38. Screening… No feeding immediately before examination Dilatation Tropicamide 1% and phenylephrine 2.5% - twice at 15 min intervals Cyclopentolate – paralytic ileus Phenylephrine – hypertension and intraventricularhaemorrhage Tropicaime – relatively safer Washing hands and wearing gloves prior to examination

39. Screening… Anterior segment examined first Retinal periphery evaluated from nasal to temporal quadrant Look for plus disease signs If mature retina : no follow up If immature retina : follow up till vessels reach temporal and nasal periphery ROP : record zone/quadrant/clock hours for each follow up till maturity or reaches threshold when intervention required

41. Screening… First screening is done at 32 weeks of post conceptual age Or 4-5 weeks after birth (whichever is earlier) Thumb rule is within 4 weeks all babies should have been examined

42. Screening… Requirements : Binocular indirect ophthalmoscope Paediatric wire speculum 20 D / 28 D lens Paediatric depressor/cotton swab/malleable aluminium wire Topical anaesthetic Can be examined by RETCAM 120

44. management Control of excessive light (light – rop) Vitamin E supplements (IM, 10 mg/kg) Cryo therapy Photocoagulation Scleral buckling for stage IV Viterectomy for stage IV and V Anti VEGF

46. Topical/local/general anaesthesia

47. Cryo-ROP

49. Recommendations of ablative therapy (ETROP) Type 1 pre threshold disease – ablation Type 2 - observation

51. Dilated pupil via 20D / 28D lens

52. Complications – anterior segment ischemia, cataract, burns of iris/cornea

53. Advantages – lesser pain, portability, few systemic effects

56. Stage IV a scleral buckling usually preferred

57. Visual prognosis is poor and main aim is to maintain ambulatory vision

59. Thank you