Retinopathy of prematurity: Altered development

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Retinopathy of prematurityAltered development

• A disorder with a uniquely American heritage

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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

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RLF National Cooperative Study
Relative Risk 3.6 ( 95 CI 1.7 7.75)
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Normal oxygen values
Avery et al. Neonatology Pathophysiology and
Management of the Newborn, 4th ed. Pg 130, table
11-2.
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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

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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

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The goal supply blood to the maturing retina
http//www.tsbvi.edu/Outreach/seehear/winter98/ICR
OP.gif
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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

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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

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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. 1987105 906-912.
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When ROP develops How bad is it ?

• Plus Disease very tortuous vessels implying
  high blood flow bad
• Rush Disease Plus disease in zone 1

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Stage One
http//ropard.org/ The Association for
Retinopathy of Prematurity and Related Diseases
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Stage Two
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Stage Three
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Stage Four
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Stage Five
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When ROP develops where is it?
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ROP A disease that can regress
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ROP A disease that can regress
Pediatrics. 200511615 23.
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Incidence inversely proportional to gestational
age at birth
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Incidence inversely proportional to gestational
age at birth
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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

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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

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Prevention of severe diseaseCyrotherapy outcome
at 5 ½ years
Arch Opthalmol. 1996224417-424
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Earlier treatment of disease in Zone One
Arch Opthalmol. 2003 1211684-96
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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 gt95 when breathing
  supplemental O2 must be avoided as much as
  possible
• Sola, A, et al. Acta Paediatrica. 96(6)801-812,
  June 2007.

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Limit oxygen exposure
Chow et al. Pediatrics. 2003111339-45
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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)

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Other ophthalmologic sequelae
Cats B. and Tan K. J Ped Opthamal Strabismus.
1989271-75
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Myopia related to ROP
Quinn GE et al.Opthalmology 1998 1051292-1299
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Myopia related to ROP
Quinn GE et al.Opthalmology 1998 1051292-1299