Retinopathy of Prematurity (ROP): Full Form, Stages, Treatment

Retinopathy of Prematurity, i.e., full form of ROP, is also known as retrolental fibroplasia and Terry syndrome. ROP is an eye condition that occurs in babies born prematurely (early). It affects the retinal layer of the eye, which interprets light such that it helps us see. 

ROP, in mild cases, usually resolves on its own. However, if untreated in severe cases, it might even progress to blindness. Hence, babies born prematurely should be under the constant supervision of obstetricians and ophthalmologists. Continue reading to know about the ROP symptoms, causes, risk factors, prevetive measures, diagnosis, treatment and complications.

Retinopathy of prematurity is an eye condition wherein blood vessels grow abnormally in the retinal layer of the eye. The retina is situated in the posterior part of the eyeball and is the most important layer in the eye that is responsible for vision. In ROP, the abnormal blood vessels grow near this layer resulting in several vision problems.

Babies born prematurely are at highest risk for suffering from this disease. Premature babies are those that are born before their estimated delivery date, i.e., before completing 38 weeks of pregnancy. Since they do not spend enough time developing inside the mother's womb, they become more prone to many conditions, one of which is retinopathy of prematurity.

India is the leading country with the highest number of premature births in the world. (1) This may increase the risk of developing ROP among the Indian population.

The main pathology in retinopathy of prematurity lies in the blood vessels of the eye. The development of ROP eye occurs through several stages. There are five ROP stages, starting from mild to severe. These stages are:

1. Stage 1, Mild ROP: In this stage, there is a thin white line appears at the border of the retina and also mild abnormal blood vessel growth in the retina. The blood vessels may be dilated or twisted, but they do not affect the baby's vision.
2. Stage 2, Moderate ROP: In this stage,  the line becomes thicker, and a small ridge or fold may appear. More abnormal blood vessel also grow in the retina. The blood vessels are still confined to a small area of the retina and do not affect the baby's vision.
3. Stage 3, Severe ROP: In this stage, there is a significant amount of abnormal blood vessel growth in the retina. The blood vessels extend into the central part of the retina and can cause bleeding, scarring, and detachment of the retina. This can lead to vision loss if not treated promptly. 
4. Stage 4, Advanced ROP: In this stage, the abnormal blood vessels have caused a partial detachment of the retina, which can lead to severe vision loss or blindness.
5. Stage 5, End-Stage ROP: In this stage, there is a complete detachment of the retina, which can cause permanent vision loss or blindness.

ROP is a disease that affects the internals of the eyeball; hence there are almost no signs and symptoms one can notice, especially in the early stages of the disease. In children with severe ROP eye, one can notice the following signs and symptoms:

1. Unusual movements of the eye like they may wander or shake, called nystagmus
2. Inability of the eye to follow objects
3. Trouble identifying faces
4. Cross eyes, called strabismus
5. Severe nearsightedness, called myopia
6. White appearance of the pupils, called leukocoria

The blood vessels of the ROP eye retina start growing in the fourth month of pregnancy and grow until the ninth month. In babies born before the nine months are completed, the blood vessels may haven't developed properly. This can lead to the abnormal formation of blood vessels, also known as retinal neovascularization.

This abnormal proliferation of blood vessels in relation to the retina may lead to retina detachment. The retina is a layer of cells in the eyeball's posterior part. It consists of special nerve cells that help perceive light and images. Any problem with this layer can cause vision problems and blindness in severe cases. In ROP, it is the blood vessels which affect the retina. 

Several factors can increase the risk of abnormal proliferation of blood vessels. These are as follows:

1. Premature birth and low birth weight are the biggest risk factors for ROP.
2. Oxygen therapy, such as supplemental oxygen, prolonged mechanical ventilation, etc., is given for breathing problems.
3. Infections (same)
4. Maternal risk factors 
5. High blood pressure during pregnancy
6. Maternal diabetes mellitus
7. Usage of medications during pregnancy (e.g. beta blockers, antihistaminics)
8. Advanced age
9. Smoking and consumption of tobacco products

ROP is a dangerous condition, which if not treated on time, can lead to fatal life-long complications. Prevention of ROP eye can be done by preventing premature births. In order to do that, one must remember the following things: 

1. First and foremost, ensure that the baby is delivered in a tertiary care hospital with a neonatal intensive care unit attached to it.
2. Continuous monitoring of oxygen therapy is done so as to prevent the deleterious effects of too much oxygen in the retinal vessels. This is done by measuring the oxygen levels in the retinal vessels.
3. Steroids are given to mothers before delivery. Steroids help premature babies develop and become mature, thereby reducing the risk of ROP.
4. Consult a doctor before taking any medication during pregnancy. One should inform the doctor diligently about all the meds she is on.
5. Avoid smoking or consuming tobacco products as it may lead to low birth weight babies or intrauterine growth restriction (the growth of the foetus is restricted in the uterus).
6. Avoid late pregnancies. Pregnancy at an advanced age is not only associated with preterm or low birth weight babies but also chromosomal abnormalities (example down's syndrome).

If a baby is at risk for developing ROP, the diagnosis of the same is made collectively on the basis of the patient's and the mother's history, clinical examination, and investigations.

Patient History

Various factors can put a baby at an increased risk of developing ROP. The mother is asked about the history of following things before the delivery of the baby:

1. High blood pressure 
2. Infections 
3. Diabetes mellitus 
4. Taking medications such as beta-blockers or antihistaminics 
5. Smoking or chewing tobacco products 

The doctor also inquires about the history of the baby by asking about the following questions:

1. The time of delivery of the baby
2. Mode of delivery of the baby
3. Weight at birth
4. Any oxygen therapy taken for breathing problems
5. After taking a detailed history of the mother and the baby, the doctor performs a clinical examination on the baby.

Clinical Examination & Investigations

Infants at risk of developing ROP must undergo an eye exam after a few weeks of birth. An ophthalmic examination is performed by the ophthalmologist. This is commonly known as the ROP test or ROP eye test. In mild cases, a demarcation line and a ridge is usually noted by the doctor. In advanced cases, abnormal proliferation of blood vessels is visualised while performing the ROP eye test.

Apart from this ROP test, the doctor may perform routine investigations to rule out any other systemic disorders. These may include:

1. CBC (Complete blood count)
2. Blood sugar levels
3. Thyroid assay
4. Magnetic Resonance Imaging (MRI) of the head 
5. Ultrasonography 

How to prepare for the doctor’s consultation? 

Before taking your baby for a doctor’s appointment you must prepare for the appointment. Following things can be kept in mind while preparing for the consultation

1. Make an appointment with the doctor in advance.
2. Visit the OPD on the scheduled time and date for the examination.
3. Complete the formalities necessary at the doctor's clinic.

What to expect from the doctor? 

1. Ask about the medical history of both the child and the mother. 
2. Clinical examination on the child and look for the signs of ROP. 
3. Perform ROP eye test

What questions should the patient ask the doctor?

One can ask the following questions before birth/ during pregnancy:

1. How to prevent premature birth?
2. How to prevent hypertension in pregnancy?
3. How to prevent diabetes during pregnancy?

The following questions should be asked after delivery:

1. When do I have to come for the ROP eye test?
2. Why should I come for the ROP eye tests?
3. What should I expect during the ROP eye test?
4. What if I do not come for the follow-up tests for ROP?
5. What will happen if the child is not treated for ROP?
6. What are the treatment options for ROP?
7. Why is surgery needed?
8. What would be the entire cost of the entire treatment?
9. How long will it take to treat ROP?
10. Can ROP be managed without surgery?
11. How to prevent the progression of ROP eye?

The treatment for ROP depends upon the stage of the disease. Early stages (stages 1 and 2) do not require any specific treatment except for regular eye examinations. Frequent eye examinations are a must to keep the disease in check since the abnormal blood vessels can begin to grow at any time.

ROP treatment without surgery 

Retinopathy of prematurity treatment options without surgery are fairly limited. As such there is no treatment required for early stages which is identified after performing tests and analysing retinopathy of prematurity radiology features.

For advanced cases, the doctor may suggest the following treatment:

1. Anti-VEGF therapy: These injections consist of medications known as anti VEGF drugs. The primary function of this drug is to stop the abnormal proliferation and growth of the blood vessels. These injections are directly given into the eye and is performed under anaesthesia.

ROP treatment with surgery

For advanced stages, surgical treatment is the only retinopathy of prematurity treatment modality that is required. The following procedures can be done to treat ROP:

1. Laser Photocoagulation: This is the treatment of choice in most cases and has largely superseded cryotherapy in the management of ROP. It is better tolerated by infants than by other surgical interventions.
2. Vitrectomy: It refers to the removal of the vitreous humour, a gel-like fluid, from the eyeball and replacing it with saline. Vitrectomy helps prevent total blindness in advanced cases of ROP.
3. Scleral buckling: Scleral buckling involves wrapping an elastic band which is typically composed of silicone, over the eye's rim. The band is wrapped around the eye's sclera (the white part of the eye), causing it to "buckle" or push inward. As a result, the ruptured retina is pushed up against the eye's outer border and continues to do so.

The retinopathy of prematurity treatment approach and selection of procedures depend on the patient’s health condition and the treating doctor's opinion.

ROP is usually asymptomatic in initial stages but can cause severe complications when not treated on time. It can cause several eye complications, such as: 

1. Myopia: loss of far vision.
2. Amblyopia: also known as lazy eye, decreased eyesight.
3. Strabismus: the eyes don't align in the same direction
4. Nystagmus: fast, uncontrollable eye movements.
5. Cataracts: the clouding of the eye's normally clear lens leading to vision disturbance/ loss.
6. Retinal breaks: this occurs due to the abnormal development of the peripheral retina, which can lead to thinning of the peripheral retina, retinal holes, tears, and detachments.
7. Retinal detachment: part or the whole of the retina pulls away from the connective tissue. It is an emergency situation 
8. Blindness

When to See a Doctor? 

One must see a doctor when the baby shows any of the following signs:

1. Unable to follow an object with the help of their eyes.
2. Unable to notice any movements made in front of the eyes.
3. Unable to see or recognise known faces.
4. Irritable.

Since ROP is a condition affecting premature babies, the mother needs to do everything she can to avoid premature birth. Pregnant women should incorporate the following food items and their diet to prevent retinopathy of prematurity: 

1. Dark leafy vegetables such as spinach, kale, broccoli, etc.
2. Fruits rich in vitamin C such as kiwi, oranges, guava, etc.
3. Green beans
4. Calcium rich foodstuff like green vegetables, milk, cheese, soyabean, and fish
5. Water
6. Whole grain cereals, fortified cereals
7. Fibre-rich bread
8. Fish since they are a rich source of Omega 3 fatty acids
9. Seeds like flax seeds, pumpkin seeds, and sunflower seeds are rich in micronutrients and antioxidants

Foods to avoid

1. Processed food items made up of refined flour like cakes, biscuits
2. Baked goods
3. Fried foodstuffs 
4. Junk food

ROP is an eye condition affecting the retinal layer of the eye. It occurs in premature babies born before thirty weeks of pregnancy or those weighing less than 1.5 kgs. It mainly manifests in the form of vision problems such as myopia, strabismus, nystagmus etc., and can further lead to complications such as retinal detachment and blindness. This condition can be managed by appropriate surgical intervention. Regular eye examinations are essential in the management of early stages of ROP. 

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