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Retrolental Fibroplasia (R.L.F.)/Retinopathy of Prematurity (R.O.P.)

(Originally called retrolental fibroplasia; now called retinopathy of prematurity.)

DESCRIPTION: One of the most devastating, baffling, and controversial ocular conditions involving young children; first reported in the 1940's and theorized to be caused by excess oxygen administered to premature infants; current theories suggest that the cause may not yet be known, since it has also begun to occur in full-term infants who did not receive oxygen. Among the causative theories are: too much light, too soon, in hospital neonatal units; Vitamin E deficiency; multiple factors associated with prematurity. Ocular involvement follows a pattern of initially constricted ocular blood vessels, followed by vascular dilation and fibrovascular proliferation into the vitreous (thus, the term "retrolental fibroplasia" - literally, fibrous retinal vessel tissue behind the lens). In some cases, there is partial or complete regression of the condition, with little ocular damage. In other cases, the retina is pulled away and blindness ensues. Occasionally, the condition is unilateral. Myopia and strabismus are common among children who retain useful vision, and there is a possibility of inlater life. Glaucoma, uveitis, cataract, or phthisis bulbi (shrunken blind eye) may also occur months or years later.

TREATMENT: Careful monitoring of retinal status in premature infants; if vessel development appears abnormal, removal of oxygen support usually allows spontaneous regression of the condition. (If there are respiratory or other problems requiring the use of oxygen for the survival of the infant, it may be impossible to control the deterioration of the ocular condition).

Other complicating eye conditions should be monitored.

Educational adaptations include a variety of optical aids (hand magnifiers, hand telescopes, CCTV). Myopic corrections are usually indicated, as well as high level of illumination. If visual acuity is too low to use magnification effectively, adaptations will change accordingly.

IMPLICATIONS: There is some evidence, though inconclusive, that children with RLF/ROP have a higher than normal incidence of social and/or emotional problems (particularly autistic-like behaviors). Research has not yet established a definite link, but it is thought that some as yet undefined factor associated with prematurity may be responsible.

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