I remember standing by my micropreemie’s isolette, describing everything I saw out the window: the blue sky, the trees, the sunshine. I wanted my son to know what it was like outside the walls of the NICU, but I didn’t know if he would ever be able to see these things for himself. Jaxson had just been diagnosed with severe and aggressive Retinopathy of Prematurity (ROP) and the disease was threatening to take his eyesight.
Jaxson was referred to a Retinopathy of Prematurity (ROP) specialist. The doctor was kind, informative, and very skilled at her job. We trusted her and we knew she would help us make the right decisions to save Jaxson’s eyesight. However, I had a hard time finding information online about ROP that I could understand, especially when I was nervous and stressed out about the diagnosis.
Here’s what I learned…
What is Retinopathy of Prematurity (ROP)?
The retina is the lining of the back of the eye, where images are formed and sent to the brain. Retinopathy of Prematurity (ROP) happens when the retina’s blood vessels grow abnormally. The smaller a baby is at birth, the more likely that baby is to develop ROP, although there are many factors that determine if a baby will develop ROP. (Source)
How serious the ROP is depends on what part of the eye is affected (the zone); how far the disease has progressed (the stage); and whether the blood vessels are enlarged and twisted (plus disease). (Source)
Zones of the Eye
- Zone 1 — near the center of the retina; core vision
- Zone 2 — the middle of the retina
- Zone 3 — the outer portions of the retina; peripheral vision
Stages of ROP
- Stage I — Mildly abnormal blood vessels. Usually requires no treatment.
- Stage II — Moderately abnormal blood vessels. Usually requires no treatment
- Stage III — Severely abnormal blood vessels. The blood vessels grow toward the center of the eye instead of following their normal growth pattern along the surface of the retina. Treatment is usually necessary and has a good chance of preventing retinal detachment.
- Stage IV — Partially detached retina. Scar tissue from the abnormal vessels pulls the retina away from the wall of the eye. Treatment is necessary.
- Stage V — Completely detached retina. Treatment is necessary.
“Plus Disease” means that the blood vessels of the retina have become enlarged and twisted, indicating a worsening of the disease. When Plus Disease is present (any zone or stage), treatment is usually necessary. (Source) Pre-Plus Disease means that the eyes are showing warning signs of developing Plus Disease.
Frequent eye exams are necessary to monitor for progression of the disease.
What Are the Treatment Options for Retinopathy of Prematurity (ROP)?
My son’s disease was severe and aggressive and it was effecting his core vision (Zone 1, Stage III, with Plus Disease). Our ophthalmologist recommended the new-at-the-time treatment of injecting a drug called Avastin into Jax’s eyes. Avastin is often used with cancer patients, but the drug works differently than chemotherapy. Avastin is a tumor-starving (anti-angiogenic) therapy that prevents the growth of blood vessels.
The drug would target and destroy the enlarged and twisted vessels (the Plus Disease) and allow healthy blood vessels to grow.
The Avastin injections were administered bedside in the NICU. The procedure took one minute! While the procedure itself was quick, the process was stressful for me and my baby. After the procedure, I immediately did Kangaroo Care with my son to help comfort him.
The injections cured my son’s Plus Disease. Jaxson showed positive progress immediately when the healthy blood vessels reached Zone 2 of his eye, but then the vessels stopped growing. Our doctor warned us that laser surgery was still a possibility.
Laser therapy “burns away” the periphery of the retina. Another less commonly used treatment option is cryotherapy, where the periphery of the retina is frozen. Both treatments are considered invasive surgeries on the eye. (Source) These treatments can slow or reverse the abnormal growth of blood vessels. Unfortunately, the treatments also destroy some side vision. Treatment is done to save the most important part of our sight – the sharp, central vision we need for “straight ahead” activities like reading and driving.
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 prevents pulling on the scar tissue and allows the retina to flatten back down onto the wall of the eye. Sclera buckles are usually performed on infants with stage IV or V ROP.
- Vitrectomy. Vitrectomy involves removing the the gel-like substance of the eye called 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.
Jaxson’s eyes were carefully monitored for any changes. He got weekly eye exams until he was released from the NICU and they continued after we got home. When my son was 6 months old actual (2 months adjusted), the disease began progressing. The blood vessels in his retina began bleeding and showing signs (again) of Plus Disease. It was time for laser surgery. We were more comfortable with laser surgery at this point because the Avastin injections had cured the disease in Zone 1; he would only need laser surgery in Zones 2 and 3. The laser surgery took approximately 30 minutes and was performed under general anesthesia. Jaxson’s eyes were slightly puffy and red after surgery, but he did not seem bothered by it at all. After the surgery, Jax had monthly follow-ups with his ROP specialist.
Can ROP Cause Other Eye Problems?
Infants with ROP have a higher risk for developing eye problems later in life, such as retinal detachment, myopia (nearsightedness), strabismus (crossed eyes), amblyopia (lazy eye), and glaucoma. In many cases, these eye problems can be treated or controlled. (Source)
My preemie was discharged from his ROP specialist and referred to a pediatric ophthalmologist for continuing care when he was 1 year old actual. We see the pediatric ophthalmologist every 6 months. Jaxson is 2 1/2 years old now and has just begun showing signs of “lazy eye” which could be a lasting effect from the ROP, the injections, or the laser surgery.
Was your preemie diagnosed with Retinopathy of Prematurity (ROP)? What treatments, if any, did your preemie receive? Has your preemie experienced any lasting effects from ROP?
I hope this information helps if your preemie is facing a diagnosis of ROP. I have linked to sources within this post and have provided first-hand information about our experience. This article is not a substitute for medical information. Please talk with your pediatric ophthalmologist for information specific to your preemie.