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

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Presentation on theme: "Bilateral Eviscerations- Retinopathy of Prematurity Dr Caroline Graham Stoke Mandeville Hospital Aylesbury."— Presentation transcript:

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

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

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

4 Macroscopic appearance Left:cornea 15 mm diameter with central opacity and some white tissue 15 mm diameter 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. 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

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12 Right eye

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

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

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

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

21 Retinal vascularisation Retinal vascularisation begins at about the 4 th month of gestation with a vasculogenic wave Retinal vascularisation begins at about the 4 th month of gestation with a vasculogenic wave Vascularisation reaches the nasal periphery by the 8 th month of gestation and the temporal periphery by about 1 month post- term Vascularisation reaches the nasal periphery by the 8 th 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 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. 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 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 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 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 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 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 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 ?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 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 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 The demarcation lines becomes an elevated ridge of tissue Appears pink because of the formation of capillaries seen as abnormally branched vascular tufts 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 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 Retinal blood vessels posterior to the demarcation line become dilated and tortuous and there are retinal and vitreous haemorrhages Mild, moderate and severe forms Mild, moderate and severe forms

28 Stages of ROP Stage 4 Subtotal retinal detachment Extraretinal proliferation causes tractional retinal detachment Extraretinal proliferation causes tractional retinal detachment Starts at periphery and spreads centrally 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 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) 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+ ROP usually undergoes complete regression if the stage is less than 2+ Sign of regression is the growth of vessels peripheral to the ridge 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 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 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 Laser Vitrectomy for removal of retrolental mass Vitrectomy for removal of retrolental mass

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

34 Thank you Miss Ramona Khooshabeh Miss Ramona Khooshabeh Luciane & Richard Luciane & Richard


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