Transcript Retinopathy of prematurity - Emory Department of Pediatrics

Retinopathy of prematurity:
Altered development
A disorder with a uniquely
American heritage
Early History
• Silverman, WA. Retrolental Fibroplasia: A
Modern Parable. Monographs in
Neonatology. 1980
• Dr. Stewart Clifford, Boston pediatrician
discovers first case -1941
• Dr. Harry Messenger, Boston
ophthalmologist coined the term RLF
RLF National Cooperative Study
Group
Scarring
RLF
No
Scarring
RLF
39
Total
Routine
8
Curtailed
20
405
425
Total
28
444
472
Relative Risk = 3.6 ( 95% CI 1.7 – 7.75)
47
Normal oxygen values
P02
Hb sat
(%)
Oxygen
content
Hb g/dl
Uterine
vein
(mom)
40
Umbilical Umbilical
artery
vein
15
27
76
30
68
12.2
6.4
14.5
12
16
16
Avery et al. Neonatology: Pathophysiology and Management of the
Newborn, 4th ed. Pg 130, table 11-2.
Retinal vascular development
(ontogeny)
• The choroidal vessels can supply the thin
retina via diffusion
• The retinal nerve cells (photoreceptors)
develop from the optic nerve to the
periphery
• Additional blood supply develops as the
retinal nerve cell layer becomes thicker
Ontogeny of the retinal vascular
bed
• Inner vascular plexus
– Within the nerve fiber layer
– Capillaries appear around the 16th week of
gestation and reach the ora serrata at about
32 – 36 weeks gestation nasally and
temporally just before term
– Vasculogenesis
The goal – supply blood to the
maturing retina
http://www.tsbvi.edu/Outreach/seehear/winter98/ICROP.gif
Ontogeny of the retinal vascular
bed
• Outer vascular plexuses
– Develops later in gestation and continues to
develop postnatally
– Capillaries arise as cellular buds from the
innermost vessels
– Angiogenesis
When ROP develops –
How bad is it ?
• Stage One – A line of demarcation
between the vascular and avascular retina
• Stage Two – The line comes a ridge
• Stage Three – The ridge is associated with
neonvascularization entering the vitreous
When ROP develops –
How bad is it ?
• Stage Four – Subtotal detachment of the
retina
– IV – A is extrafoveal detachment
– IV – B the detachment includes the fovea
• Stage Five – Total Detachment
– The old retrolental fibroplasia
An International Classification of Retinopathy of Prematurity. Arch Ophthalmol.
1987;105: 906-912.
When ROP develops –
How bad is it ?
• Plus Disease – very tortuous vessels
implying high blood flow; bad
• Rush Disease – Plus disease in zone 1
Stage One
http://ropard.org/ The Association for Retinopathy of
Prematurity and Related Diseases
Stage Two
Stage Three
Stage Four
Stage Five
When ROP develops where is it?
ROP – A disease that can regress
BW < 1250 gm
1981 -1985
n = 185
1985 – 1988
n = 226
Mild ROP
86 (46%)
118 (52%)
Moderate ROP
24 (13%)
35 (15%)
Severe ROP
10 (5%)
12(5%)
ROP – A disease that can regress
Stage one Stage two
Stage three
Birth weight
< 750 gm
18%
34%
48%
750 – 999
30%
38%
31%
1000 - 1250
51%
31%
18%
Pediatrics. 2005;116:15 – 23.
Incidence inversely proportional to
gestational age at birth
Incidence inversely proportional to
gestational age at birth
ETROP
CRYO-ROP
< 27 wks
89%
83%
> 27- 31 wks
52%
55%
> 32 wks
14%
30%
Prevention of severe disease
• Primary – decrease the number of infants
born at the gestations with highest risk
• Secondary
– An agent that will prevent the retinal blood
vessel drop out after birth in very premature
infants
– Limit the vasoproliferative phase
– Safe oxygen administration
Prevention of severe disease
• Cryotherapy and laser therapy limit the
vasoproliferative phase by destroying the
avascular retina once THRESHOLD has
been reached
• Intravitreal bevacizumab (Avastin)
injection
Prevention of severe disease
Cyrotherapy outcome at 5 ½ years
Treated Eye
Control Eye
Normal
103 (49%)
68 (33%)
Total retinal
detachment
Blind
46 (22%)
80 (39%)
70 (31%)
106 (48 %)
Arch Opthalmol. 1996;224:417-424
Earlier treatment of disease in
Zone One
Early Treatment Conventional
Treatment
Normal
213 (64%)
200 (62%)
Blind or Low
vision
33 (10%)
47 (15%)
Arch Opthalmol. 2003; 121:1684-96
Limit excessive oxygen exposure
• Conclusion: Inappropriate oxygen use is a neonatal
health hazard associated with aging, DNA damage and
cancer, retinopathy of prematurity, injury to the
developing brain, infection and others. Neonatal
exposure to pure O2, even if brief, or to pulse oximetry
>95% when breathing supplemental O2 must be avoided
as much as possible
– Sola, A, et al. Acta Paediatrica. 96(6):801812, June 2007.
Limit oxygen exposure
Chow et al. Pediatrics. 2003;111:339-45
Screen
• All infants with birth weights less than
1500 grams or gestational age less than
32 weeks
• Begin at 4 to 6 weeks
• Continue until mature (vascularized to the
periphery)
Other ophthalmologic sequelae
Regressed
ROP
No ROP
Strabismus
31%
19%
Myopia > 3 D
16%
2%
Decreased
vision
31%
5%
Cats B. and Tan K. J Ped Opthamal & Strabismus. 1989:271-75
Myopia related to ROP
Quinn GE et al.Opthalmology 1998; 105:1292-1299
Myopia related to ROP
Quinn GE et al.Opthalmology 1998; 105:1292-1299