Retinopathy of Prematurity: A Text and Atlas Raj Vardhan Azad
Aggressive posterior ROP 25
Childhood blindness 6
causes 7, 8
Congenital cataracts 105
Cryotherapy vs laser 50, 70
Familial exudative vitreoretinopathy 104
Genetics in ROP 100
Norrie's disease gene 101
International classification of ROP 18
Iridex diode laser indirect ophthalmoscopy 51
Laser treatment for ROP 51
postoperative procedures 60
Light ROP study 100
Norrie's disease 105
Peripheral retinal ablation 50
Persistent hyperplastic primary vitreous 105
PhotoROP 100
Plus disease 19
Pre-plus disease 25
Prethreshold ROP 21
Regressed ROP 30 RetCam (Retinal camera)
applications 108
benefits 109
features 108
limitations 110
procedure 109
Retinal detachment 19
Retinal vascular development 10
Retinoblastoma 105
Retinopathy of prematurity 2
incidence and natural course 42
risk factors 28
Retrolental fibroplasia 2
ROP etiopathogenesis
classical theory 14
growth factors 15
spindle cell theory 15
Rush disease 18
Screening of ROP 44
follow-up examination 46
immature 47
mature 47
method 44
instruments 45
place 44
preparation of the child 44
procedure 45
special criteria 44
Sequelae of ROP
anterior segment changes 80
changes in ocular dimensions 80
disk drag fold and macular heterotopia 84
post-laser regressed ROP 83
refractive errors 80
regressed ROP 80
spontaneously regressed ROP 81, 82
strabismus and motility defects 80
Stop-ROP study 100
Surgery for retinal detachment 86
advanced stage of ROP 88
combined lensectomy and vitrectomy 87
scleral buckling 86
vitrectomy with lens conservation 87
Telemedicine 110
Threshold disease 22
Vasculogenesis 10
defective 15
Visual rehabilitation 96
closed circuit television training 96
eye safety issues 97
medicolegal issues 98
psychosocial factors 97
refractive care 96
ROP awareness program 97
special low vision concerns 96
Chapter Notes

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Retinopathy of Prematurity (ROP): History and IntroductionCHAPTER ONE

Retinopathy of prematurity (ROP) is a disease affecting the retina of premature infants (Figs 1.1A to C). Its key pathologic change, retinal neovascularization, has several features in common with the other proliferative retinopathies such as diabetic and sickle cell retinopathy. Each of these proliferative retinal vascular disorders appears to be associated with local ischemia and the subsequent development of neovascularization.
zoom view
Figs 1.1A to C: Premature children before examination
Historical Perspective
  • First identified by Terry1 in 1942, Terry's original reports designated the condition retrolental fibroplasia based on his impression that the primary change involved a proliferation of the embryonic hyaloid system which incorporated the retina. He studied the unilateral pathological specimens (most likely to be PHPV) and provided details which he thought may be identical with bilateral cases of retrolental fibroplasia.
  • The term ROP was coined by Heath in 1951. He was much more precise in his descriptions and his histopathological studies offered rare insight into the disease processes as it was then understood.
  • In 1951, Campbell suggested that toxic effects of uncontrolled oxygen to new-borns were responsible for the epidemic.2 She suggested avoiding use of oxygen and use only if the child is cyanosed.
  • The incrimination of oxygen as the principal cause of ROP was made in the 1950s.3,4 Subsequent studies showed high levels of oxygen obliterated blood vessels of the neonatal retina. Controlled use of oxygen although lowered the proportion of blindness (In USA alone from 50% in 1950 to 4% in 1965) but it increased the neonatal deaths due to atelectasis and respiratory distress secondary to hyaline membrane disease.5,6
  • 3During the late 1970s and 1980s, resurgence of ROP was noted and called the second epidemic due to increased survival of very low birth premature infants (750-999 gm).7-9
  • In 1981, Phelps8 estimated the incidence of ROP associated with an increase in survival rates of infants with birth weights less than 1,000 gm.
  • In 1983, International Classification of Retinopathy of Prematurity was devised under the leadership of John Flynn.10
  • In 1970s the Japanese physicians treated the ridge and the adjacent avascular retina.11 Hindle and Leyton, in Canada, treated the ridge, and the retina anterior and posterior to it. McPherson, Hittner, and Kretzner advocated cryotherapy of the ridge and adjacent posterior avascular zone to destroy the spindle cells, the presumed source of angiogenic factor.12
  • In Asian countries like India, due to lopsided health care, i.e. due to extremes of health care, state of the art tertiary health care neonatal center and almost defunct neonatal primary health care centers, both first and second epidemic exist, and may be called as the third or mixed epidemic.
  • However, more recent reports indicate a decline in the incidence, severity and progression to threshold disease in developed countries.13-17