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Retinopathy of Prematurity THIRTY DAYS TO LIGHT!

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Dr Subhadra Jalali

Babies born prematurely with or without infertility treatments need early eye examination, writes Dr Subhadra Jalali, Associate Director, Srimati Kanuri Santhamma Centre for Vitreo Retinal Diseases, L V Prasad Eye Institute, Hyderabad

Dr Subhadra Jalali

Dr Subhadra Jalali
Associate Director, Srimati
Kanuri Santhamma Centre
for Vitreo Retinal Diseases,
L V Prasad Eye Institute,
Hyderabad

Today, more couples are turning to assisted fertilisation to help build families due to numerous advances and improved successes of these techniques. Now, women have great opportunities and they are choosing to develop their education/careers instead of becoming housewives. However, by the time, they focus on having a family, age becomes the major deterrent. There are also couples who stay far away from each other, but still want to have babies. Assisted fertilisation techniques have come as a boon for such people.

Babies born after any type of assisted conception are more likely to be born pre-term, more likely to be of multiple births (twins/triplets/quadruplets) and of low-birth weight. Pre-mature babies including those after Assisted Conception are vulnerable to a serious eye disease called, Retinopathy of Prematurity (ROP). Awareness about this risk is low even amongst those doctors that deliver, or those that take care of these babies after birth, resulting in needless and irreversible blindness.

Retinopathy of Prematurity (ROP) is abnormal blood vessel development in the retina of the eye in a premature infant. The blood vessels of the retina begin to develop three months after conception, and complete their development at the time of normal birth. If an infant is born prematurely, that is anytime less than a month before the expected date of delivery (EDD), this eye development can be disrupted. The vessels may stop growing, or grow abnormally from the retina into the normally clear gel that fills the back of the eye. The vessels are fragile and can leak, causing bleeding inside the eye not visible from outside. This dynamic disease is not present at birth. Although, the retinal vasculature is immature at birth, there is an attached macula that has a potential for good vision. ROP starts only two-three weeks after birth, providing a window period for screening and offering treatment at the right time. All pre-term babies are at risk of ROP and should have one compulsory retinal examination before 30 days of life. Smaller babies should be screened earlier by 20 days of life. Further, close follow- up examinations are needed till the child reaches the EDD, and then once or twice a year at least till the child is five years old.

Scar tissue may develop and pull the retina loose from the inner surface of the eye (retinal detachment) causing irreparable vision loss. Routine use of excess oxygen to treat premature babies is an additional risk factor. If oxygen is accurately monitored, and given in a controlled way, the problem is less likely but not absent, and can occur even when no oxygen was given. The risk of developing ROP also depends on the degree of prematurity and the sickness after birth. Generally, the smallest, low-weight and most sick premature babies have the highest risk, but no premature baby is without risk of ROP, and ROP blindness.

There are five stages of ROP

Stage I: There is mildly abnormal blood vessel growth.
Stage II: Blood vessel growth is moderately abnormal, and in some cases may need early treatment.
Stage III: Blood vessel growth is severely abnormal, and requires early treatment within 72 hours.
Stage IV: Blood vessel growth is severely abnormal, and there is a partially detached retina and some eyes may improve      with urgent surgery.
Stage V: There is a total retinal detachment, and only very few eyes get minimal vision even after advanced treatment.

The blood vessel changes cannot be seen with the naked eye. A detailed retinal examination by a well-trained person is needed to reveal such problems.

Symptoms of severe ROP usually in its incurable advanced stage include

Abnormal eye movements
Crossed eyes
Severe nearsightedness
White-looking pupils (leukocoria)

Early stages of ROP where treatment is effective have no symptoms, and cannot be detected by routine eye examination. Hence, a routine dilated retinal examination within 20 to 30 days of birth is the only way to detect ROP, and prevent this blindness.

Treatment
In ROP, time means vision. The first 20 to 30 days of life are the most critical for ROP management. Early ablation of the avascular retina produces improved visual outcomes. Laser ablation is the standard of care, and is highly successful in more than 85 percent cases. It is safe, if performed properly at the correct stage of disease, usually within three to five weeks of birth. Failed laser cases can be offered immediate use of newer emerging therapies like Avastin (Bevacizumab) injection, or immediate surgery under expert guidance. Late cases can be offered surgery, but results are universally poor and hence the importance of early screening by 20 to 30 days of life. Success in ROP is directly linked to the time and rapidity of treatment. It is a medical emergency, and should be treated as such.

Complications
The severe case of ROP can occur due to extreme prematurity, fluctuating or unmonitored oxygen, sepsis and delayed presentation to the ophthalmologists beyond 25 to 30 days of life. A few cases fail due to inadequately done laser. If there is a poor response to treatment, the child should be referred within 48 hours to an expert who can do more aggressive laser, early surgery, or in selected cases inject an anti-VEGF drug to halt the progression of the disease.
Babies with ROP can have sequelae such as low vision, myopia, anisometropia and squint. Advanced cases can also develop complications like glaucoma and late-onset retinal detachments besides cataract.

“ROP starts only twothree weeks after birth, providing a window period for screening and offering treatment at the right time. All pre-term babies are at risk of ROP, and should have one compulsory retinal examination before day 30 of life”

Expectations
If managed appropriately, most premature infants with ROP recover with no lasting visual problems, but many of them would need spectacles. Many premature infants with slight problems in retinal blood vessel growth have the vessels return to normal without treatment and can recover completely.

Prevention
The most effective prevention of Retinopathy of Prematurity is prevention of premature birth which currently is not possible, and in fact is on the rise partially due to assisted fertilisation techniques that often result in premature and multiple births. Preventing other complications of prematurity (such as neo-natal respiratory distress syndrome) may also help prevent ROP.
ROP is an increasing concern among young parents who are caught unawares with blindness in their premature baby due to poor ROP management. The science and art of preserving vision in these fragile newborns has to be taken out of journals where the scientific information lies in the form of complicated medical studies and has to be placed in the cribs (baby beds) amongst the caregivers. The slogan for all those who handle pre-term babies should be Tees Din Roshni Ke: Thirty Days to Light!

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