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Retinopathy of Prematurity

Babies born prematurely, prior to 34 weeks of gestation (6 weeks before the due date), face a higher likelihood of encountering various health complications in comparison to full-term infants (37-40 weeks of gestation). One such risk is Retinopathy of Prematurity (ROP), a condition affecting the developing blood vessels in the eye’s retina. ROP stands as the foremost cause of childhood blindness among premature infants born prematurely or with low birth weight. In India, babies weighing less than 2000 grams (2 kgs) or born 6 weeks before the due date (34 weeks of gestation) are susceptible to ROP.
rop

Risk factors for developing Retinopathy of Prematurity (ROP)

The primary factors increasing the likelihood of ROP are being born prematurely (most prevalent in infants born over 12 weeks early) and having a low birth weight (common in 11% – 56% of preterm infants with low birth weight). A baby’s eye development primarily occurs in the final three months of gestation in the womb. When a baby is born prematurely, the retinal development remains unfinished, and there’s a chance that the blood vessels supplying oxygen to the eye may not develop normally, resulting in ROP.
Apart from premature birth and low birth weight, numerous other factors can elevate the vulnerability of these infants to ROP. These encompass extended oxygen usage (for lung function), sepsis (blood infection), blood transfusion, jaundice, inadequate weight gain, reduced platelet count, anemia (low hemoglobin), and intraventricular hemorrhage (brain bleeding), among others.
If your baby was born prematurely, you might be worried about ROP impacting their eyesight. Narayana Nethralaya advises an ROP assessment a few weeks after birth (initial screening before 30 days or sooner) for premature infants and those weighing under 2 kgs at birth, before hospital discharge. Early detection and prompt treatment can enhance the likelihood of maintaining vision.

Signs and Symptoms of Retinopathy of Prematurity (ROP)

The retina plays a crucial role in vision, and any harm to it can result in vision loss or blindness. Regrettably, when ROP initially develops in a newborn, there are no external visible indications. Consequently, many parents fail to have their infants screened promptly. In later ROP stages, a white reflex (known as leukocoria) appears at the center of the pupil (the circular black part of the eye). Unfortunately, by the time signs become apparent (around 4 to 6 weeks of age), it’s already too late to rescue functional vision. An untreated infant with advanced ROP will experience diminished vision, reduced eye contact with parents, abnormal eye shaking (nystagmus), and often delayed developmental milestones as well.

Retinopathy of Prematurity (ROP) Screening

ROP
ROP screening in premature babies serves the goal of promptly identifying the condition, enabling efficient intervention to avert potential vision impairment. As signs of ROP are absent during its initial phases, making predictions about its development in preterm infants is unfeasible for doctors. This underscores the significance of screening. According to India’s 2018 national ROP guidelines, all infants born weighing under 2000 grams and/or born prematurely (with a gestation period of fewer than 34 weeks) should have their inaugural ROP screening completed within 30 days of birth.
Continuous monitoring for ROP is necessary for every premature baby, extending beyond hospital discharge due to the condition’s potential delayed onset. Our team employs specialized imaging devices to conduct screenings either in the NICU of the maternity hospital or at our facility if the baby has been discharged. Should any indications of ROP become apparent, our team of experts in ROP will arrange subsequent assessments to track the baby’s progress and ascertain the appropriate timing for intervention.

Diagnosis of Retinopathy of Prematurity (ROP)

ROP

Diagnosing ROP requires the expertise of an ophthalmologist skilled in managing this condition. At Sai Deep Eye Cilinic Pediatric Retina division, advanced technology is available to identify ROP in premature infants. Our ophthalmologists possess the necessary training to diagnose ROP and will meticulously assess the retina of your preterm baby for any irregularities. This examination, which is painless and brief, typically takes only a few minutes.

Diagnosis of Retinopathy of Prematurity (ROP)

Diagnosing ROP requires the expertise of an ophthalmologist skilled in managing this condition. At Narayana Nethralaya’s Pediatric Retina division, advanced technology is available to identify ROP in premature infants. Our ophthalmologists possess the necessary training to diagnose ROP and will meticulously assess the retina of your preterm baby for any irregularities. This examination, which is painless and brief, typically takes only a few minutes.

Treatment of Retinopathy of Prematurity (ROP)

The course of treatment hinges on the stage and severity of ROP. Typically, ROP is mild in most cases and resolves spontaneously, causing no harm. Nevertheless, more severe manifestations of ROP necessitate intervention to minimize the risk of retinal detachment and enduring vision impairment or blindness. ROP demanding treatment is referred to as ‘Type 1 ROP,’ and the optimal approach is to initiate treatment within 48 hours of diagnosis for the best results.
Commonly, ROP treatment employs a specialized ROP laser performed by a specialist. This procedure is conducted under topical anesthesia (via eye drops), targeting the retinal area lacking blood supply. This laser treatment yields positive results in about 90% of cases, leading to the disappearance of ROP and the preservation of eyesight. In specific scenarios, injections of a corrective drug directly into the eye (intravitreal) to counter abnormal vessel growth are also utilized. It’s noteworthy that several recipients of injections might later require laser treatment.
For advanced stages like 4 and 5 of ROP, retinal surgery is necessary. This procedure, performed under general anesthesia, is coupled with NICU support.
Failure to administer timely treatment can lead to permanent blindness or profound visual impairment in both eyes for the child. In certain cases, ROP that initially presents as Stage 1 or 2 can progress to the more severe Stage 3 or beyond. Unfortunately, the outcomes following treatment for Stage 5 ROP, even after undergoing surgery, are notably unsatisfactory.

Treatment

Vitrectomy surgery is an effective treatment for age-related macular holes at stage 2 and beyond. In this procedure, the vitreous gel inside the eye is removed, the hole is closed, and the eye is filled with a gas bubble. The gas bubble self-absorbs over a period of 4-6 weeks.

Following the surgery, certain surgeons might suggest adopting a face-down position for the first few days to expedite the hole’s closure. Stage 1 macular holes do not require surgery but necessitate regular check-ups to monitor any progression to subsequent stages. If a macular hole develops in the other eye (contralateral eye), more frequent check-ups for the unaffected eye may be recommended. Macular holes resulting from other causes generally have a less favorable outlook.

ROP - RETINA - Macular Hole

Frequently Asked Questions

WHAT IS ROP?
Retinopathy of Prematurity (ROP) is an ocular ailment that impacts the retina, which is the nerve tissue within the eye. It predominantly afflicts premature infants who have a lower birth weight. In India, infants born weighing under 2000 grams (2 kgs) or delivered six weeks before their expected due date (34 weeks of gestation) are susceptible to ROP. After the surgery, the patient has the option to decide between lying down or sitting in one position with the support of a headrest. This step is crucial as it ensures a proper gas sealing effect on the macular hole.
WHAT CAUSES ROP?
ROP develops due to the incomplete establishment of the retina’s blood supply when a premature infant is born. Following birth, these blood vessels are expected to grow, but this progression is insufficient in around 30-40% of infants, leading to the formation of a dense “barrier.” In roughly 5-10% of cases, this barrier expands and inflicts permanent damage on the retina. Apart from premature birth, various other factors elevate the risk of ROP in these infants. These include extended usage of oxygen, occurrences of sepsis (blood infection), blood transfusions, episodes of jaundice, inadequate weight gain, low platelet levels, anemia (reduced hemoglobin), instances of intraventricular hemorrhage (brain bleeding), among others.
HOW DOES ROP AFFECT THE EYES/VISION?
Roughly 40% of premature newborns are at risk of developing ROP, while the remaining 60% undergo natural retinal blood vessel development without requiring intervention. Among the 40% affected by the condition, about 5-10% experience severe forms necessitating treatment to counteract the formation of the dense barrier. Failing to provide timely treatment could result in complete retinal damage (retinal detachment), leading to permanent blindness or profound visual impairment in both eyes for the child.
SIGN AND SYMPTOMS OF ROP
Regrettably, during the initial phases of ROP, there are no observable external indicators. This absence of visible cues often leads to a delay in parents seeking timely screening for their infants. In more progressed stages, a white reflection termed ‘leukocoria’ emerges at the center of the pupil, the round dark part of the eye. Regrettably, by the time these signs become evident, it’s already too late to salvage useful vision. A prematurely born child with untreated advanced ROP will likely exhibit impaired vision, limited eye contact with parents, abnormal eye movements (nystagmus), and frequently experience delayed developmental milestones as well.
STAGES OF ROP
ROP progresses through five distinct stages. The classification of these stages is based on the evaluation of the barrier. A slender barrier characterizes Stage 1. Stage 2 is identified by the thickening of the barrier in both height and width. Stage 3 marks a critical juncture where abnormal blood vessels and tissue develop on the barrier and extend beyond it, resulting in bleeding and leakage. Stage 4 is characterized by an elevation of the barrier, causing the detachment of the retina from its usual position. This detachment initially occurs at the periphery of the retina and subsequently in the central visual area known as the macula. Stage 5 materializes when the retina becomes completely detached. An additional severe form of ROP known as ‘Aggressive Posterior ROP’ (APROP) bypasses stages 1 to 3 and directly enters stages 4 and 5. Diagnosing and treating APROP are especially challenging due to their distinct nature.
WHAT IS ROP SCREENING?
In India, the national ROP guidelines from 2018 propose that all infants weighing under 2000 grams at birth and/or born before 34 weeks of gestation should have their initial ROP screening performed within the first 30 days of life. Subsequent follow-up appointments will be determined based on the findings from the initial assessment. On average, a baby will need to attend 3-5 appointments to receive a “ROP-free” status, assuming no disease develops during any of the sessions. These appointments are typically spaced 1-3 weeks apart. If a baby is diagnosed with a need for treatment (such as ROP stage 3, ROP stage 2 with plus, or APROP), intervention should ideally occur within 48 hours to achieve the best possible outcome. Normally, you will be prescribed medications like Tylenol or similar pain relievers. However, if these medications do not provide relief, it is important to inform your doctor. Also, mild or even intense redness is a common occurrence, but it will gradually diminish over time. To prevent any issues, it is advisable to avoid high altitudes or elevations that might cause the gas bubble to expand beyond its normal size. This expansion could potentially lead to eye damage. Therefore, it is better to refrain from flying until the gas bubble is completely absorbed.
HOW IS ROP DIAGNOSED?
Diagnosing ROP necessitates the expertise of an ophthalmologist specializing in ROP management. To visualize the babies’ retinas, their pupils are dilated using eye drops. Traditionally, a tool known as an indirect ophthalmoscope was employed by specialists to examine and document their findings on paper. A modern approach involves capturing retinal images using specialized cameras (wide-field, contact, infant cameras), providing an objective report. The 2018 Indian national guidelines have officially recognized imaging as an alternative method for ROP screening. These images can be shared via telemedicine platforms or forwarded to specialists for additional opinions. Furthermore, these images prove valuable in communicating with parents and tracking the baby’s condition over time.
ROP TREATMENT
ROP requiring treatment is referred to as ‘Type 1 ROP,’ and it’s advisable to initiate treatment within 48 hours of diagnosis. The preferred approach for ROP treatment is laser treatment, considered the “gold standard.” This procedure is conducted by a specialist using topical anesthesia in the form of eye drops. In laser treatment, the retina’s portion that lacks proper blood supply (located in front of the barrier) is targeted. Alternatively, injections of a drug into the eye (intravitreal) that can reverse abnormal vessel growth are also employed in specific cases. It’s noteworthy that many babies who receive injections might later require laser treatment as well. For the more advanced stages of ROP, particularly stages 4 and 5, retinal surgery is essential. This procedure is performed under general anesthesia and requires support from the Neonatal Intensive Care Unit (NICU).
ROP FOLLOW-UP
It’s crucial to bear in mind that all premature infants, including those without ROP, need ongoing monitoring even after their condition appears to be “normal.” This is due to their increased vulnerability to low vision, refractive errors (possibly necessitating eyeglasses before their first birthday), cortical vision impairment, and squint when compared to full-term babies (born at ‘9 months’). Given their preverbal stage, specialized tools and assessments are necessary to evaluate the vision of these young infants.
DOES ROP CAUSE BLINDNESS?

The majority of ROP instances do not lead to blindness. When diagnosed promptly and if those needing treatment are identified and addressed in a timely manner, more than 90-95% of infants have favorable outcomes. The outcomes of APROP are less predictable and hinge on various factors. Unfortunately, the outcomes following treatment for Stage 5 ROP are significantly unfavorable, even after undergoing surgical intervention.

Stage 4 is characterized by an elevation of the barrier, causing the detachment of the retina from its usual position. This detachment initially occurs at the periphery of the retina and subsequently in the central visual area known as the macula. Stage 5 materializes when the retina becomes completely detached. An additional severe form of ROP known as ‘Aggressive Posterior ROP’ (APROP) bypasses stages 1 to 3 and directly enters stages 4 and 5. Diagnosing and treating APROP are especially challenging due to their distinct nature.

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