Retinopathy of prematurity is a bilateral disorder of abnormal retinal vascularization in premature infants, especially those of lowest birth weight. Outcomes range from normal vision to blindness. Diagnosis is by ophthalmoscopy. Treatment of severe disease may include cryotherapy, laser photocoagulation, or bevacizumab; other treatment is directed at complications (eg, retinal detachment).
The inner retinal blood vessels start growing about midpregnancy, but the retina is not fully vascularized until term. Retinopathy of prematurity (ROP) results if these vessels continue their growth in an abnormal pattern, forming a ridge of tissue between the vascularized central retina and the nonvascularized peripheral retina. In severe ROP, these new vessels invade the vitreous. Sometimes the entire vasculature of the eye becomes engorged (plus disease).
Susceptibility to ROP correlates with the proportion of retina that remains avascular at birth. In neonates weighing < 1 kg at birth, ROP occurs in 47 to 80%, and severe ROP occurs in 21 to 43%. The percentage is higher when many medical complications exist (eg, infection, intraventricular hemorrhage [see Intraventricular and/or intraparenchymal hemorrhage], bronchopulmonary dysplasia [see Bronchopulmonary Dysplasia (BPD)]). Excessive (especially prolonged) O2 therapy increases the risk. However, supplemental O2 is often needed to adequately oxygenate the infant even though a safe level and duration of O2 therapy have not been determined.
Diagnosis is made by ophthalmoscopic examination, done by an ophthalmologist, which shows a line of demarcation and a ridge in mild cases and proliferation of retinal vessels in more severe cases.
Screening ophthalmoscopy is done in all infants weighing < 1500 g or < 30 wk gestation at birth. Because disease onset is usually at 32 to 34 wk gestational age, screening begins at 31 wk. Ophthalmologic examinations continue every 1 to 3 wk (depending on the severity of the eye disease) until infants have growth of vessels into the periphery (equivalent to term). Because significant ROP is rare in appropriately managed infants weighing > 1500 g at birth, alternative diagnoses should be considered in these infants (eg, familial exudative retinopathy, Norrie disease).
Abnormal vessel growth often subsides spontaneously but, in about 4% of survivors weighing < 1 kg at birth, progresses to produce retinal detachments and vision loss within 2 to 12 mo postpartum. Children with healed ROP have a higher incidence of myopia, strabismus, and amblyopia. A few children with moderate, healed ROP are left with cicatricial scars (eg, dragged retina or retinal folds) and are at risk of retinal detachments later in life; rarely, glaucoma and cataracts can also occur.
In severe ROP, cryotherapy or laser photocoagulation to ablate the peripheral avascular retina reduces the incidence of retinal fold and detachment. Retinal vascularization must be followed at 1- to 2-wk intervals until the vessels have matured sufficiently. If retinal detachments occur in infancy, scleral buckling surgery or vitrectomy with lensectomy may be considered, but these procedures are late rescue efforts with low benefit.
Patients with residual scarring should be followed at least annually for life. Treatment of amblyopia and refractive errors in the first year optimizes vision. Infants with total retinal detachments should be monitored for secondary glaucoma and poor eye growth and referred to intervention programs for the visually impaired.
Bevacizumab is a new antivascular endothelial growth factor monoclonal antibody that can stop the progression of ROP. Compared to laser therapy, bevacizumab has a lower rate of recurrence and fewer structural abnormalities. When disease did recur, it recurred months later; long-term ophthalmology follow-up is required. Concerns regarding systemic absorption and possible infection coupled with the need for optimal dose and timing of follow-up are reasons why this drug has remained a 2nd-line therapy that can be used to treat severe disease or in conjunction with laser therapy.
After a preterm birth, O2 should be supplemented only as needed to avoid swings in O2 as both hyperoxia and hypoxia increase ROP risk. Vitamin E and restricted light are not effective.
Last full review/revision January 2015 by James W. Kendig, MD; Ursula Nawab, MD
Content last modified January 2015