What is retrolental fibroplasia?

Retrolental fibroplasia, also known as retinopathy of prematurity (ROP), is a rare disease in which blood vessels grow abnormally behind the retina. In severe cases this progresses to scar tissue which can lead to problems with the eye. Retrolental fibroplasia occurs in premature babies and has been linked to the high concentrations of oxygen used to maintain their underdeveloped lungs.

In a fetus, blood vessels begin to form in the eye three months after conception and are complete at birth. Prematurity interrupts this development, often causing retinal vascular proliferation. Severe retrolental fibroplasia is marked by this rapid growth, as well as severe scarring and sometimes retinal detachment. This can lead to blindness, reduced vision, and other problems with the eye.

Which premature babies are most at risk of developing retrolental fibroplasia? The risk is proportional to how premature the baby is, which means that the earlier it is born, the greater the chances of developing the disease. Also, the smallest premature babies, regardless of gestational age, are at higher risk. Because retrolental fibroplasia is such a serious disease, most babies born before 34 weeks gestational age, three weeks before full term, are seen by an ophthalmologist.

What are the symptoms of retrolental fibroplasia? Unfortunately, most signs of the disease are not noticeable to the untrained eye. Symptoms include white pupils (leukocoria), abnormal eye movement (nystagmus), severe nearsightedness (myopia), and crossed eyes (strabismus). It is imperative to recovery that this disease be diagnosed and treated early, before severe scarring develops and the retina has detached.

Retrolental fibroplasia treatments include cryotherapy or freezing therapy, surgery to reattach the retina, laser treatment, and low vision support. Laser therapy is used more often than cryotherapy, but should be used before severe scarring and retinal detachment occur. Studies in preterm infants using oral vitamin E have shown a reduction in the incidence of retrolental fibroplasia. Most of the time, abnormal blood vessel growth becomes normal on its own, but about 10% of affected babies will continue to experience abnormal growth and progress to severe retrolental fibroplasia.

Little can be done to prevent retrolental fibroplasia other than avoiding premature labor. Early diagnosis and treatment are key to preventing major damage to the eye. As neonatal intensive care units are becoming more state-of-the-art, clinicians can more effectively monitor the level of oxygen delivered to premature babies, thereby reducing the potential for disease development.

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