1. Bilateral Eviscerations-Retinopathy of Prematurity
Dr Caroline Graham
Stoke Mandeville Hospital
Aylesbury

2. Clinical History 24 year old female
Traveller-limited history available
Born 15 weeks prematurely
Blind since birth

3. Clinical Summary Small eyeballs Enophthalmos Hypotonia
Corneal scarring with band keratopathy and neovascularisation
Bilateral, painful, phthisical eyes therefore bilateral eviscerations
Non-functioning pituitary adenoma;no treatment

4. Macroscopic appearance
Left:cornea 15 mm diameter with central opacity and some white tissue 15 mm diameter
Right: cornea 15 x 11 mm with a central and peripheral opacity and some firm haemmorhagic tissue, apparently calcified, 12 mm in diameter.

5. Left eye

6. Left eye

7. Left eye

8. Left eye

9. Left eye

10. Left eye

11. Left eye

12. Right eye

13. Right eye

14. Right eye

15. Right eye

16. Right eye

17. Summary of histology Left
Cornea-irregular thickness, neovascularisation, amyloid
Lens-calcified, wrinkled capsule
Massive gliosis and calcification
Drusen

18. Summary of histology Right
Cornea-irregular thickness, band keratopathy, neovascularisation
Lens-calcified and ossified with wrinkled capsule
Gliosis, calcification, ossification

19. Summary
Mostly non-specific changes of phthisis bulbi but in keeping with ROP ie no normal surviving retina, gliosis, drusen
Amyloid is of interest-I think secondary to damaged eye but amyloid can be associated with ROP.
Any ideas from the floor?

20. Retinopathy of Prematurity
Vasoproliferative retinopathy
Occurs in infants with an immature, incompletely vascularised retina
Ranges from minimal sequelae which do not affect vision to bilateral, irreversible blindness

21. Retinal vascularisation
Retinal vascularisation begins at about the 4th month of gestation with a vasculogenic wave
Vascularisation reaches the nasal periphery by the 8th month of gestation and the temporal periphery by about 1 month post-term
ROP develops at the interface between the vascularised retina and non-vascularised periphery

22. Risk factors for ROP Low birthweight
ROP develops in 32% of infants with a birthweight of 1000 grams or less.
Incidence falls to 7% if the birthweight is between 1001 and 1500 grams

23. Risk factors for ROP Retinal maturity
The less well developed the retinal vasculature the more severe is the ROP
Oxygen and angiogenic growth factors
Oxygen tension causes capillary obliteration and vascular endothelial death in the immature retina
Severity of ROP is related to the duration and amount of oxygen exposure
Subsequent exposure to normal levels of oxygen results in opening of the vascular network and the hypoxic retina produces GFs

24. Risk factors for ROP
ROP can occur in infants who are full term or who have not received oxygen therapy
?in utero injury to the genetic factors controlling vascularisation
Can occur in full term infants because temporal retina not fully vascularised until 8 weeks after birth and therefore susceptible to damage

25. Stages of ROP Stage 1 Demarcation line
A thin, tortuous grey-white line develops between the vascularised and avascular retina

26. Stages of ROP Stage 2 Ridge
The demarcation lines becomes an elevated ridge of tissue
Appears pink because of the formation of capillaries seen as abnormally branched vascular tufts

27. Stages of ROP
Stage 3 Ridge with extraretinal fibrovascular proliferation
Proliferating fibrovascular tissue breaks through the ILM and erupts onto the retinal surface and vitreous
Retinal blood vessels posterior to the demarcation line become dilated and tortuous and there are retinal and vitreous haemorrhages
Mild, moderate and severe forms

28. Stages of ROP Stage 4 Subtotal retinal detachment
Extraretinal proliferation causes tractional retinal detachment
Starts at periphery and spreads centrally

29. Stages of ROP Stage 5 Total retinal detachment
In extreme cases the retina is totally detached and pulled into folds
Together with the extraretinal tissue is drawn forward to lie against the lens (retrolental fibroplasia)

30. Clinical Course
ROP usually undergoes complete regression if the stage is less than 2+
Sign of regression is the growth of vessels peripheral to the ridge
Later stages associated with abnormal ocular growth; myopia; retinal pigmentation; dragging of the retina; retinal holes, folds, detachment; glaucoma; synechiae; haemorrhage; scarring; fibrosis; phthisis bulbi

31. Prevention
Titration of oxygen levels to level at which systemic complications caused by hypoxia and ocular complications caused by hyperoxia might be avoided

32. Treatment
Laser
Vitrectomy for removal of retrolental mass

33. Molecular stuff VEGF – it’s complicated
Insulin growth factor I (IGF-I). Hellstrom hypothesized that IGF-I plays a role in ROP

34. Thank you
Miss Ramona Khooshabeh
Luciane & Richard