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Retrolental fibroplasia

Retinopathy of prematurity (ROP), also known as retrolental fibroplasia (RLF), is a disease of the eye that affects prematurely born babies. It is thought to be caused by disorganised growth of retinal blood vessels resulting in scarring and retinal detachment. more...

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ROP can be mild and may resolve spontaneously, but may lead to blindness in serious cases. Oxygen toxicity may contribute to the development of ROP.

International Classification of Retinopathy of Prematurity (ICROP)

The system used for described the findings of ROP is entitled, The International Classification of Retinopathy of Prematurity (ICROP). ICROP "demarcated the location of the disease into zones (1, 2, and 3) of the retina, the extent of the disease based on the clock hours (1-12), and the severity of the disease into stages (0-5)" .

Symptoms and prognosis

In preterm infants, the retina is often not fully formed. ROP occurs when abnormal tissue forms between the central and peripheral retina. There are 5 progressive stages to ROP. Stage 1 is mild and may resolve on its own without severe vision loss; stage 5 is severe and usually results in retinal detachment.

Multiple factors can determine how fast a patient progresses through the stages, including overall health, birth weight, the stage of ROP at initial diagnosis, and the presence or absence of "plus" disease. "Plus" disease occurs when the abnormal vessels in the retina invade other areas of the eye, greatly increasing the risk of retinal detachment.

The abnormal vessel growth often subsides spontaneously, but can progress to retinal detachment and vision loss in patients with extremely low birth weight. Patients with ROP are at greater risk for glaucoma, cataracts and myopia later in life, and should be examined yearly to help prevent and treat these conditions.

Treatment

Cryotherapy
"Indirect laser"
scleral buckle and/or vitrectomy may be considered for severe ROP with retinal detachment

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Insight into preemies' blindness - premature babies:retinopathy of prematurity - Brief Article
From Science News, 7/7/01 by

About 5 percent of premature babies suffer vision losses, including blindness, due to a condition known as retinopathy of prematurity. The damage occurs when excessive growth of blood vessels and other tissue pulls the newborn's retinas away from the walls of their eyes. Now, researchers have linked the condition to low concentrations of a substance called insulin-like growth factor 1 (IGF-1).

Previous research established that IGF-1 concentrations are higher in full-term babies than in preemies, says Lois E. H. Smith of Harvard Medical School in Boston. She and her colleagues also knew that IGF-1 is crucial for the survival of cells that make up blood vessels. So, they looked at the effect of various concentration of IGF-1 and found that the concentrations associated with premature birth aren't high enough to ensure survival of blood vessel cells in laboratory cultures.

To see if these lab results reflect a link between IGF-1 and retinopathy, Smith and her colleagues at the Queen Silvia Children's Hospital in G6teborg, Sweden, measured IGF-1 concentrations in the blood of 41 infants born 5 to 12 weeks premature. In babies of the same gestational age, those with the lower IGF-1 concentrations were more likely to develop retinopathy.

"Measurement of this growth factor might help researchers predict which premature infants are most at risk of developing the disease," Smith says. "Ultimately, replacing IGF-1 might allow doctors to prevent this tragic complication."

"This is potentially a good way to treat a serious problem of thousands and thousands of infants," said Ross Clark of the University of Auckland in New Zealand. He noted that IGF-1 might have beneficial side effects since it's involved in the normal growth of the lungs and gut, which often remain underdeveloped in preemies.

Enticing as IGF-1 treatment sounds, Clark cautions that preemies are so small that it may be hard to monitor the growth factor's concentration in blood. Says Clark: "Treating premature infants with drugs raises serious concerns."

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