Transcript Bilateral Eviscerations-Retinopathy of Prematurity

Bilateral EviscerationsRetinopathy of
Prematurity
Dr Caroline Graham
Stoke Mandeville Hospital
Aylesbury
Clinical History
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24 year old female
Traveller-limited history available
Born 15 weeks prematurely
Blind since birth
Clinical Summary
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Small eyeballs
Enophthalmos
Hypotonia
Corneal scarring with band
keratopathy and neovascularisation
Bilateral, painful, phthisical eyes
therefore bilateral eviscerations
Non-functioning pituitary adenoma;no
treatment
Macroscopic appearance
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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.
Left eye
Left eye
Left eye
Left eye
Left eye
Left eye
Left eye
Right eye
Right eye
Right eye
Right eye
Right eye
Summary of histology
Left
 Cornea-irregular thickness,
neovascularisation, amyloid
 Lens-calcified, wrinkled capsule
 Massive gliosis and calcification
 Drusen
Summary of histology
Right
 Cornea-irregular thickness, band
keratopathy, neovascularisation
 Lens-calcified and ossified with
wrinkled capsule
 Gliosis, calcification, ossification
Summary
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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?
Retinopathy of
Prematurity
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Vasoproliferative retinopathy
Occurs in infants with an immature,
incompletely vascularised retina
Ranges from minimal sequelae which
do not affect vision to bilateral,
irreversible blindness
Retinal vascularisation
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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 postterm
ROP develops at the interface between the
vascularised retina and non-vascularised
periphery
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
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
Risk factors for ROP
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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
Stages of ROP
Stage 1 Demarcation line
 A thin, tortuous grey-white line
develops between the vascularised
and avascular retina
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
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
Stages of ROP
Stage 4 Subtotal retinal detachment
 Extraretinal proliferation causes
tractional retinal detachment
 Starts at periphery and spreads
centrally
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)
Clinical Course
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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
Prevention
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Titration of oxygen levels to level at
which systemic complications caused
by hypoxia and ocular complications
caused by hyperoxia might be avoided
Treatment
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Laser
Vitrectomy for removal of retrolental
mass
Molecular stuff
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VEGF – it’s complicated
Insulin growth factor I (IGF-I).
Hellstrom hypothesized that IGF-I
plays a role in ROP
Thank you
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Miss Ramona Khooshabeh
Luciane & Richard