Retinopathy of Prematurity
Retinopathy of prematurity (ROP) is abnormal blood vessel development in the retina of the eye in infants that are born too early (premature).
The blood vessels of the retina (in the back of the eye) begin to develop about 3 months into pregnancy. In most cases, they are fully developed at the time of normal birth. The eyes may not develop properly if a baby is born very early. The vessels may stop growing or grow abnormally from the retina into the back of the eye. Because the vessels are fragile, they can leak and cause bleeding in the eye.
Scar tissue may develop and pull the retina loose from the inner surface of the eye (retinal detachment). In severe cases, this can result in vision loss.
In the past, the use of too much oxygen in treating premature babies caused vessels to grow abnormally. Better methods are now available for monitoring oxygen. As a result, the problem has become less common, especially in developed countries. However, there is still uncertainty about the right level of oxygen for premature babies at different ages. Researchers are studying other factors besides oxygen which appear to influence the risk of ROP.
Today, the risk of developing ROP depends on the degree of prematurity. Smaller babies with more medical problems are at higher risk.
Almost all babies who are born before 30 weeks or weigh less than 3 pounds (1500 grams or 1.5 kilograms) at birth are screened for the condition. Some high-risk babies who weigh 3 to 4.5 pounds (1.5 to 2 kilograms) or who are born after 30 weeks should also be screened.
In addition to prematurity, other risk factors may include:
- Brief stop in breathing (apnea).
- Heart disease.
- High carbon dioxide (CO2) level in the blood.
- Infection.
- Low blood acidity (pH).
- Low blood oxygen.
- Respiratory distress.
- Slow heart rate (bradycardia).
- Transfusions.
The rate of ROP in most premature infants has gone down greatly in developed countries over the past few decades due to better care in the neonatal intensive care unit (NICU). However, more babies born very early are now able to survive, and these very premature infants are at the highest risk for ROP.
The blood vessel changes cannot be seen with the naked eye. An eye exam by an ophthalmologist is needed to reveal such problems.
There are five stages of ROP:
- Stage I: There is mildly abnormal blood vessel growth.
- Stage II: Blood vessel growth is moderately abnormal.
- Stage III: Blood vessel growth is severely abnormal.
- Stage IV: Blood vessel growth is severely abnormal and there is a partially detached retina.
- Stage V: There is a total retinal detachment.
An infant with ROP may also be classified as having “plus disease” if the abnormal blood vessels match pictures used to diagnose the condition.
Symptoms of severe ROP include:
- Abnormal eye movements.
- Crossed eyes.
- Severe nearsightedness.
- White-looking pupils (leukocoria).
Babies who are born before 30 weeks, weigh less than 1,500 grams (1.5 kilograms or about 3 pounds) at birth, or are high risk for other reasons should have retinal exams.
In most cases, the first exam should be within 4 to 9 weeks after birth, depending on the baby’s gestational age.
- Babies born at 27 weeks or later most often have their exam at 4 weeks of age.
- Those born earlier most often have exams later.
Follow-up exams are based on the results of the first exam. Babies do not need another exam if the blood vessels in both retinas have completed normal development.
Parents should know what follow-up eye exams are needed before the baby leaves the nursery.
The goal of treatment is to reduce your eye pressure. Treatment depends on the type of glaucoma that you have.
OPEN-ANGLE GLAUCOMA
- If you have open-angle glaucoma, you will probably be given eye drops or receive laser glaucoma treatment.
- You may need more than one type of eye drops. Most people can be treated with eye drops.
- Most of the eye drops used today have fewer side effects than those used in the past.
- Laser treatment uses a painless laser to open the channels where fluid flows out.
- You also may be given pills to lower pressure in the eye.
If drops alone or laser treatment do not work, you may need other treatment:
- If drops and laser treatment do not work, you may need surgery. Your eye doctor will open a new channel so fluid can escape. This will help lower your eye pressure.
- Recently, new implants have been developed that can help treat glaucoma in people having cataract surgery.
ACUTE ANGLE GLAUCOMA
An acute angle-closure attack is a medical emergency. You can become blind in a few days if you are not treated.
- You may be given eye drops, pills, and medicine given through a vein (by IV) to lower your eye pressure.
- Some people also need an emergency operation, called an iridotomy. The eye doctor uses a laser to open a new channel in the iris. Sometimes this is done with surgery. The new channel relieves the attack and will prevent another attack.
- To help prevent an attack in the other eye, the same procedure will often be performed on the other eye. This may be done even if it has never had an attack.
CONGENITAL GLAUCOMA
- Congenital glaucoma is almost always treated with surgery.
- This is done using general anesthesia. This means the child is asleep and feels no pain.
SECONDARY GLAUCOMA
If you have secondary glaucoma, treating the cause may help your symptoms go away. Other treatments also may be needed.
Most infants with severe vision loss related to ROP have other problems related to early birth. They will need many different treatments.
About 1 out of 10 infants with early changes will develop more severe retinal disease. Severe ROP may lead to major vision problems or blindness. The key factor in the outcome is early detection and treatment.
Complications may include severe nearsightedness or blindness.
The best way to prevent this condition is to take steps to avoid premature birth. Preventing other problems of prematurity may also help prevent ROP.
Retrolental fibroplasia; ROP.
References
Fierson WM; American Academy of Pediatrics Section on Ophthalmology; American Academy of Ophthalmology; American Association for Pediatric Ophthalmology and Strabismus; American Association of Certified Orthoptists. Screening examination of premature infants for retinopathy of prematurity. Pediatrics. 2019;143(3):e20183810. PMID: 30824604 pubmed.ncbi.nlm.nih.gov/30824604/.
Olitsky SE, Marsh JD. Disorders of the retina and vitreous. In: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Nelson Textbook of Pediatrics. 21st ed. Philadelphia, PA: Elsevier; 2020:chap 648.
Sun Y, Hellström A, Smith LEH. Retinopathy of prematurity. In: Martin RJ, Fanaroff AA, Walsh MC, eds. Fanaroff and Martin’s Neonatal-Perinatal Medicine. 11th ed. Philadelphia, PA: Elsevier; 2020:chap 96.
Thanos A, Drenser KA, Capone Jr A. Retinopathy of prematurity. In: Yanoff M, Duker JS, eds. Ophthalmology. 6th ed. Philadelphia, PA: Elsevier; 2023:chap 6.17.
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