Retinopathy of prematurity (ROP), is a disease of the eye affecting prematurely born babies generally

having received neonatal intensive care, in which oxygen therapy is used due to the premature
development of their lungs. It is thought to be caused by disorganized growth of retinal blood vessels
which may result in scarring and retinal detachment. ROP can be mild and may resolve spontaneously,
but it may lead to blindness in serious cases. Thus, all preterm babies are at risk for ROP, and very low
birth-weight is an additional risk factor. Both oxygen toxicity and relative hypoxia can contribute to the
development of ROP.

pathophysiology

During development, blood vessels grow from the central part of the retina outwards. However, in
premature babies it is incomplete. If blood vessels grow normally, ROP does not occur. If the vessels
grow and branch abnormally the baby develops ROP. These abnormal blood vessels may grow up from
the plane of the retina and may bleed inside the eye. When the blood and abnormal vessels are
reabsorbed, it may give rise to multiple band like membranes which can pull up the retina, causing
detachment of the retina and eventually blindness before 6 months.

Patients with ROP, particularly those who have developed severe disease needing treatment are at
greater risk for strabismus, glaucoma, cataracts and shortsightedness (myopia) later in life and should be
examined yearly to help prevent or detect and treat these conditions.

Stages

ROP is classified in five stages, ranging from mild (stage I) to severe (stage V):

Stage I — Mildly abnormal blood vessel growth. Many children who develop stage I improve with no
treatment and eventually develop normal vision. The disease resolves on its own without further
progression.

Stage II — Moderately abnormal blood vessel growth. Many children who develop stage II improve with
no treatment and eventually develop normal vision. The disease resolves on its own without further
progression.

Stage III — Severely abnormal blood vessel growth. The abnormal blood vessels grow toward the center
of the eye instead of following their normal growth pattern along the surface of the retina. Some infants
who develop stage III improve with no treatment and eventually develop normal vision. However, when
infants have a certain degree of Stage III and “plus disease” develops, treatment is considered. “Plus
disease” means that the blood vessels of the retina have become enlarged and twisted, indicating a
worsening of the disease. Treatment at this point has a good chance of preventing retinal detachment.

Stage IV — Partially detached retina. Traction from the scar produced by bleeding, abnormal vessels
pulls the retina away from the wall of the eye.
Stage V — Completely detached retina and the end stage of the disease. If the eye is left alone at this
stage, the baby can have severe visual impairment and even blindness.

Most babies who develop ROP have stages I or II. However, in a small number of babies, ROP worsens,
sometimes very rapidly. Untreated ROP threatens to destroy vision.

Signs and symptoms

Many of the signs of retinopathy of prematurity happen deep inside the eye, which means you won’t be
able to see them just by looking at your child. Only an ophthalmologist , using special instruments to
examine your child’s retina.

An infant with severe ROP might develop visible complications, such as nystagmus (abnormal eye
movements) and leukocoria (white pupils) White pupils, called leukocoria

Abnormal eye movements, called nystagmus

Crossed eyes, called strabismus

Severe nearsightedness, called myopia

. However, these are also general signs of vision trouble — if your child has any of these, you should see
an ophthalmologist right away.

Diagnosis

All premature babies who may be at risk for ROP are carefully monitored and examined for the
condition beginning a few weeks after birth, before they're discharged from the hospital. The only way
to determine if babies have ROP is to examine the inside of their eyes for abnormalities in the retina.

Ophthalmologists trained in the diagnosis and treatment of ROP will examine your baby's eyes. During
this exam, your baby's pupils will be dilated with eye drops so the retina can be studied. If any signs of
ROP are detected, follow-up evaluations will be recommended over the following weeks.

riskfactors

Premature babies are the number-one risk group for retinopathy of prematurity. In general, the smaller
and more premature the infant, the more likely he or she is to develop ROP, and the more likely to need
treatment.

Babies considered most at risk for ROP have:

a gestational age of 30 weeks or less, compared with 38 to 42 weeks for a full-term infant (“gestational
age” means the amount of time since the baby was conceived)

a birth weight of 1,500 grams (3.3 pounds) or less, which is 2,000 grams (about 4.4 pounds) less than for
a typical full-term infant
Other possible risk factors for ROP include:

anemia

infection

transfusions

breathing difficulties

heart disease

ethnicity (ROP occurs slightly more often in Caucasian children)

ROP has also been linked to supplemental oxygen, something often given to preemies. But excessive
oxygen levels are largely a thing of the past, thanks to modern-day improvements in monitoring
systems.

Causes

Researchers are still working to understand the mechanism behind retinopathy of prematurity

Studies led by Children’s Hospital Boston ophthalmologist Lois Smith, MD, PhD, suggest that ROP might
be caused by the early cutoff of chemicals that babies receive from their mother in the womb, including
insulin-like growth factor I (IGF-I) and vascular endothelial growth factor (VEGF). By the fourth month of
pregnancy, the fetal retina has begun to develop vascularization. Such formation of blood vessels
appears to be very sensitive to the amount of oxygen supplied, either naturally or artificially.

However, it’s possible there are other chemicals are at play, and research is ongoing.

treatments

The most effective proven treatments for ROP are laser therapy or cryotherapy. Laser therapy “burns
away” the periphery of the retina, which has no normal blood vessels. With cryotherapy, physicians use
an instrument that generates freezing temperatures to briefly touch spots on the surface of the eye that
overlie the periphery of the retina. Both laser treatment and cryotherapy destroy the peripheral areas of
the retina, slowing or reversing the abnormal growth of blood vessels. Unfortunately, the treatments
also destroy some side vision. This is done to save the most important part of our sight — the sharp,
central vision we need for “straight ahead” activities such as reading, sewing, and driving.

Both laser treatments and cryotherapy are performed only on infants with advanced ROP, particularly
stage III with “plus disease.” Both treatments are considered invasive surgeries on the eye, and doctors
don’t know the long-term side effects of each.

In the later stages of ROP, other treatment options include:

Scleral buckle. This involves placing a silicone band around the eye and tightening it. This keeps the
vitreous gel from pulling on the scar tissue and allows the retina to flatten back down onto the wall of
the eye. Infants who have had a sclera buckle need to have the band removed months or years later,
since the eye continues to grow; otherwise they will become nearsighted. Sclera buckles are usually
performed on infants with stage IV or V.

Vitrectomy. Vitrectomy involves removing the vitreous and replacing it with a saline solution. After the
vitreous has been removed, the scar tissue on the retina can be peeled back or cut away, allowing the
retina to relax and lay back down against the eye wall. Vitrectomy is performed only at stage V.