Retinopathy of prematurity

KEY POINTS

  • Retinopathy of prematurity (also called ROP) is an eye disease that affects many premature babies.

  • ROP is one of the most common causes of vision loss in children. It can lead to lifelong vision problems and blindness.

  • ROP doesn’t have any signs or symptoms when it first develops in a baby. It can only be found during an eye exam. 

  • ROP can develop quickly. Ask your baby’s provider about eye exams at every checkup.

  • If your baby has severe ROP, early treatment gives your baby the best chance of having healthy vision.

What is retinopathy of prematurity? 

Retinopathy of prematurity (also called ROP) is an eye disease that affects many premature babies. A premature baby is one who is born too early, before 37 weeks of pregnancy. ROP happens when a baby’s retinas don’t fully develop. The retina is the nerve tissue that lines the back of the eye. ROP usually affects both eyes.  

About 14,000 to 16,000 babies born in the United States each year have ROP. Most babies with ROP have a mild case and don’t need treatment. But babies with severe ROP can have vision problems or blindness. About 400 to 600 babies each year become legally blind from ROP. 

If your baby has ROP, she needs treatment right away. ROP can develop quickly. If not carefully checked by your baby’s health care provider, ROP can take away your baby’s vision. Take your baby to all his checkups and eye exams so his provider can check him every time for ROP. 

If your baby has ROP or other health conditions, visit our online community at shareyourstory.org. Share is our online community of families of premature babies and babies with other health conditions. You can connect with them for support throughout your baby’s treatment.

What causes ROP?

As your baby’s eyes start to develop during pregnancy, blood vessels form in the back of the eye to give the retina oxygen and nutrients it needs to grow. During the last 12 weeks of pregnancy, your baby’s eyes develop quickly. When your baby is born, most of the blood vessels in the retina are nearly grown. The retina usually finishes growing in the first few weeks after birth.

If a baby is born too early, the blood vessels in his retinas may stop growing or they may not grow correctly. The blood vessels may grow abnormally from the retina into the back of the eye. These blood vessels are fragile and can leak. The can cause bleeding in the eye, and scar tissue can form. If the scars shrink, they may pull the retina loose from the back of the eye. This is called retinal detachment. Retinal detachment is the main cause of vision problems and blindness in ROP. 

Some things make a baby more likely than others to have ROP. These are called risk factors. Having a risk factor doesn’t mean for sure that your baby will have ROP. But it may increase his chances. We know that the smallest and sickest babies are at higher risk for ROP than larger, healthier babies. Risk factors for ROP include:

  • Anemia. This is when the body doesn’t have enough healthy red blood cells to carry oxygen to the rest of the body.
  • Birthweight. Babies who weigh less than 3 pounds at birth are more likely to have ROP than babies who weigh more at birth.  
  • Breathing problems, including apnea and respiratory distress syndrome (also called RDS). These problems are common in premature babies because the lungs and the part of the central nervous system (brain and spinal cord) that controls breathing may not be full developed. Apnea is when a baby’s breathing stops for 15 to 20 seconds or more. Apnea may lead to a drop in a baby’s oxygen level (also called oxygen desaturation) and a slow heart rate (also called bradycardia). RDS is a breathing problem that’s common in premature babies. It’s caused by a lack of surfactant, a slippery substance that keeps small air sacs in a baby's lungs from collapsing.
  • Heart disease or slow heart rate. Heart disease includes conditions that affect the heart and blood vessels.  
  • Infection and sepsis. Sepsis is the body’s extreme response to infection.
  • Premature birth. All premature babies are at risk for ROP. Premature babies born before 30 weeks of pregnancy are most likely to have it.
  • Blood problems, including low blood oxygen, high carbon dioxide levels in the blood, low blood acidity or having a blood transfusion. Your baby’s provider measures blood oxygen, carbon dioxide and blood acidity in your baby’s blood with a blood oxygen level test. If the levels in the blood are out of balance (not in the right amounts), it can mean your baby’s lungs aren’t working well. A blood transfusion is when new blood is put in in the body. Low blood oxygen, high carbon dioxide levels and low blood acidity may be signs of RDS. 

How do you know if your baby has ROP?

Your baby gets an eye exam for ROP if he:

  • Is born before 30 weeks
  • Weighs less than 3 pounds at birth
  • Is born after 30 weeks or weighs 3 to 4.5 pounds at birth and has risk factors for ROP

For the eye exam, a pediatric ophthalmologist checks your baby’s vision. This is a doctor who identifies and treats eye problems in babies and children. Your baby gets her first eye exam 4 to 9 weeks after birth. She may be in the newborn intensive care unit (also called NICU), or she may be home by this time. Babies born at 27 weeks or later usually have their first eye exam when they’re 4 weeks old. Babies born before 27 weeks usually have the exam later. This is because the more premature a baby is, the longer it takes to develop serious ROP. This is why it’s important to take your baby to every eye exam, even after you take your baby home from the NICU.

If your baby’s first eye exam shows that the blood vessels in both retinas have developed normally, she doesn’t need a follow-up exam. If your baby’s eye exam shows that she has ROP and her provider thinks she needs treatment, she starts treatment within 72 hours. Early treatment gives your baby the best chance of having healthy vision. 

How is ROP treated?

Treatment for ROP depends on how severe your baby’s condition is. Providers use stages to define the seriousness of ROP. Stage 1 is the least severe, and stage 5 is the most severe. Any stage can get worse (sometimes really quickly) and need treatment.

  • Stage 1. Mildly abnormal blood vessel growth. Many babies at stage 1 get better without treatment and go on to have healthy vision.  
  • Stage 2. Moderately abnormal blood vessel growth. Many babies at stage 2 don’t need treatment and go on to have healthy vision.
  • Stage 3. Severely abnormal blood vessel growth. Some babies at stage 3 get better without treatment. Others may develop a condition called plus disease. This is when the retina’s blood vessels get big and twisted. Plus disease is a sign that ROP is getting worse, but treatment can help prevent retinal detachment.  
  • Stage 4. Partially detached retina. Babies with stage 4 ROP need treatment because part of the retina pulls away from the inside wall of the eye.
  • Stage 5. Completely detached retina. In stage 5, the retina is completely pulled away from the inside wall of the eye. Without treatment, a baby can have severe vision problems or blindness. 

Most babies with ROP have stage 1 or 2 and get better on their own without treatment. Take your baby to all his checkups and eye exams so his provider can make sure he’s getting any treatment he needs for ROP.

Babies who need treatment for ROP usually have surgery. The most common types of surgery for ROP are:

  • Laser surgery (also called laser therapy or photocoagulation). Your baby’s provider uses laser beams of light to burn and scar the sides of the retina. This is the most common surgery.
  • Cryotherapy (also called freezing). Your baby’s doctor uses a metal probe to freeze and scar the sides of the retina. 

These surgeries can slow down or stop the growth of abnormal blood vessels and help prevent pulling on the retina. They can cause some loss of side vision (also called peripheral vision), but they can help save a baby’s central vision. Central vision is when you can see what’s straight ahead of you.

If your baby has stage 4 or 5 ROP and his retina is partially or completely detached, he may get these types of surgery:

  • Scleral buckle. Your baby’s provider puts a silicone band around the white of your baby’s eye (called the sclera). This band helps push the eye in so that the retina stays along the wall of the eye. The buckle is removed later as the eye grows. If it isn’t removed, a child can become nearsighted. This means he has trouble seeing things that are far away.
  • Vitrectomy. Your baby’s provider removes the clear gel in the center of your baby’s eye (called the vitreous) and puts saline (salt) solution in its place. Your baby’s provider can then take out scar tissue, so that the retina can relax and doesn’t pull. Only babies with stage 5 ROP have this surgery.

Many babies with ROP don’t need treatment. Even with treatment, some babies with ROP may have vision loss. And even if treatment works, babies with ROP are more likely than other babies to have some eye problems later in life including:

  • Nearsightedness (also called myopia)
  • Crossed eyes (also called strabismus)
  • Lazy eye (also called amblyopia)
  • Glaucoma. This is a group of diseases that damage the eye’s optic nerve. The optic nerve connects the retina to the brain. Glaucoma can lead to vision loss and blindness.

This is why it’s so important to make sure your baby gets all his checkups and eye exams. Ask your baby’s provider about eye exams at every checkup to help make sure ROP and other vision problems related to premature birth are diagnosed and treated as early as possible.

More information

Last reviewed: September, 2018

See also: Premature babies, Common conditions treated in the NICU