Narciso F. Atienza, Jr. MD, MBA, FPCS, FPAO
EYE PHYSICIAN AND SURGEON
FELLOW, PHILIPPINE COLLEGE OF SURGEONS
FELLOW, PHILIPPINE ACADEMY OF OPHTHALMOLOGY
DIPLOMATE, PHILIPPINE BOARD OF OPHTHALMOLOGY
SUBSPECIALTY IN DISEASES AND SURGERY OF THE RETINA, VITREOUS, AND MACULA and OCULAR ONCOLOGY
FELLOW, PHILIPPINE COLLEGE OF SURGEONS
FELLOW, PHILIPPINE ACADEMY OF OPHTHALMOLOGY
DIPLOMATE, PHILIPPINE BOARD OF OPHTHALMOLOGY
SUBSPECIALTY IN DISEASES AND SURGERY OF THE RETINA, VITREOUS, AND MACULA and OCULAR ONCOLOGY
What is Retinopathy of Prematurity.
Retinopathy of Prematurity, previously known as retrolental fibroplasia is a condition found mostly in infants born premature where new vessels grow inside the eye causing retinal detachment and eventual blindness in infants. This happens because the vessels inside the eye of this infants were not given time to fully develop. When infants are born prematurely, the eye then tries to develop faster causing these new abnormal vessels to grow. The new vessels are not as well formed as the same vessels that would grow in normal infants. These new vessels may then cause traction on the retina, and may detach that part of the retina when traction has become too extensive.
Which babies are at risk of developing ROP?
Factors that may affect the development of ROP are. 1) Gestational age when the baby was born, 2) Low birth weight. 3) Exposure to oxygen. There has been several studies that state that the more premature or pre-term a baby is, the increased risk he/she has in developing this condition. The lower the birth weight of the baby, the greater the chances of having ROP. Since advancement in pediatric neonatology has enabled pediatricians to save more premature babies than before, more patients are seen with ROP. Premature babies when recuperating are usually exposed to oxygen levels that can stimulate the growth of these vessels.
Which babies should be screened for ROP?
Though not all premature babies are at risk of developing ROP, all babies weighing less than 1500 grams (3 pounds, 5 ounces) or born at or before 34 weeks of gestation are advised to be screened and have eye examinations, since these babies are at risk of developing ROP. Any babies weighing less than 1250 grams (2 pounds, 12 ounces) or born at less than 30 weeks of gestation are at greater risk of developing ROP. It is also thought that babies who are very sick at birth and who require oxygen might be at risk of developing ROP even if they weigh more than the above-mentioned limits and if they are older than noted above. These babies are also examined for ROP.
What are the different stages of ROP?
Retinopathy of prematurity is classified according to the severity of the changes of the blood vessels and the region of the retina into which the vessels have grown. The severity is referred to as the "Stage" and the retinal regions as "Zones".
Stages are as follow:
Immature vessels Normal but incomplete growth
Stage 1 Mildly abnormal growth
Stage 2 Moderately abnormal growth
Stage 3 Severely abnormal growth
Stage 4 Partially detached retina
Stage 5 Completely detached retina
Zones are as follow:
Zone I Earliest growth of the central retina which includes the macula
Zone II Middle region of growth in which most ROP occurs
Zone III Last region of growth
The "immature vessels" stage actually occurs in all infants, and does not necessarily lead to ROP. Also, when ROP regresses (goes away), the vessels may go through this stage again until they complete their growth.
Stage 1 is a mild abnormality of the retinal vessel growth and does not require treatment.
Stage 2 is a moderate abnormality of the retinal vessel growth and also does not require treatment.
Stage 3 is a severe abnormality of the retinal vessel growth in which the blood vessels grow toward the center of the eye instead of following their normal growth pattern along the surface of the retina. When a certain degree of Stage 3 is present and when "plus disease" develops, treatment is considered. "Plus disease" indicates that the blood vessels of the retina have become enlarged and twisted. This is an indication of worsening of the disease. Plus disease can occur with almost any stage and its presence alone is not sufficient to require treatment.
Stage 4 involves a partial detachment of the retina. Stage 4A indicates that the macula is still attached. No surgery is generally required for these cases. Stage 4B indicates that the macula is detached and surgery is suggested in some cases.
Stage 5 involves a complete detachment of the retina and surgery for these cases should be considered very carefully as prognosis in operated cases usually have poor porgnosis even in the best of hands.
What happens if the ROP does not go away?
Most of the time, Stages 1, 2, and even some Stage 3 cases may go away without treatment. In a small number of babies, for reasons not well understood as yet, ROP worsens and can develop into a sight-threatening condition. This occurs only about 10% of the time, in which case treatment is usually recommended by the doctor.
What treatments are there for ROP?
Starting in the late 1980's, treatments became available for ROP. The first treatments for ROP were "cryotherapy" or freezing treatments. A freezing probe was held onto the outside of the eye to freeze the peripheral retina (side of the retina). This caused the ROP to go away in many cases, and reduced the chances of retinal detachments and blindness by about 50% as compared to babies who had no treatment. Not all babies responded favorably to this treatment. More recently, lasers have been used for treatment of ROP, which appears to work better, but not all babies respond to this treatment either. Laser treatment is less painful, and causes fewer problems than the freezing treatments, but both treatments are accepted, and are still in use today.
Our treatment of choice is laser. The purpose of the treatment is to create scar tissue on the peripheral retina. This has been shown to eliminate ROP progression in many cases. The treated part of the retina will be scarred and will no longer work. The goal of the treatments is to save as much as possible of the central retina, where the best vision is located. Some of the peripheral retina and, therefore, some of the side vision will likely be lost after these treatments. It is important to keep in mind that the central retina, where the reading vision, straight ahead vision, and most of the color vision are located, is the most important part of the retina to save.
In recent years, anti-VEGF injections have been done on premature babies with ROP. Though there have been anecdotal reports of success of this treatment, this has NOT been collaborated by large scale randomized clinical trials, and anti-VEGF injections may have the potential risk of systemically affecting the infant, which can cause severe adverse effects which can be life-threathening.
How is the laser treatment done?
The laser treatment can be performed with the baby in the crib in the neonatal intensive care unit (NICU). The baby may be given medication to make it sleepy and comfortable. The baby's heart rate and breathing is monitored during the entire procedure. The laser beam is directed through the pupil to treat the side part of the retina. The procedure is similar to the examination with the addition of laser. The treatment usually takes 30-45 minutes per eye. Afterwards, the baby's eye may be red, and the eyelids may be red and a little swollen. Eye drops are used for about one week. The redness and swelling usually goes away in a few days but may take a few weeks to completely disappear. A follow-up exam is usually performed 2-3 weeks after the laser treatment.
What happens if the laser treatment does not work?
The biggest concern, if the ROP laser treatments do not work to halt the scar tissue growth, is the development of retinal detachment. Often, only part of the retina detaches. If only the peripheral retina detaches, no further treatments should be performed, since these peripheral detachments may remain the same or go away without treatment. If the center part of the retina or the entire retina detaches, then surgery is recommended to try to reattach the retina. This surgery involves removing the scar tissue inside the eye to help the retina to reattach.
Surgery is not recommended for distortion of the retina or for scar tissue that is not causing a detachment affecting the central vision. These cases are referred to as Stage 4A if the macula is not detached. Cases in which the center of the retina becomes detached are referred to as Stage 4B. Many Stage 4B cases have a thin fold through the macula and do not need surgery. Some Stage 4B cases that do not have a thin fold through the macula may benefit from surgery.
How is surgery for ROP done?
Surgery for ROP is usually the LAST resort for this problems. Depending on the stage, your pediatric retina surgeon may opt to do either a scleral buckle, a vitrectomy or both. He may even use some other materials to help flatten out the retina, such as intraocular gases, and heavy silicone oil. The objective of the laser is to try to attach the central part of the retina. If this is successful, then your baby MAY HAVE a chance to regain some vision back, which is usually only ambulatory vision (counting fingers, hand movement) but not vision not good enough to read or even drive.
Is the surgery always successful?
NO. Due to the very complex nature of this disease, success rate may depend on how early or late the patient is seen, how scarred the retina is, and how long the retinal detachment has been present. This also depends on the overall health of your baby, as a complicated NICU course will also ultimately affect the outcome of the surgery.
Anatomic success rates (retina has been re-attached) for patients who were seen very early in the disease have been reported to as high as 60% by some authors, but this goes down dramatically when delay in the surgery has been made. Majority of patients with an unsuccessful surgery will have shrinkage of the eye in due time
Anatomic success DOES NOT translate that the baby can see when he/she grows up, as there have been a lot of instances when the retina will re-detach despite the first successful surgery. Many patients who keep their retina attached do not also see when they grow up as the retina has already been scarred during the development. However, patients may still keep ambulatory vision good enough for them to move around without assistance
In the event that the retina has been re-attached successful with a scleral buckle alone, or combined with a vitrectomy, a second operation in necessary in 6 months to remove the scleral buckle because the buckle itself will prevent the normal growth of the eye.
Retinopathy of Prematurity, previously known as retrolental fibroplasia is a condition found mostly in infants born premature where new vessels grow inside the eye causing retinal detachment and eventual blindness in infants. This happens because the vessels inside the eye of this infants were not given time to fully develop. When infants are born prematurely, the eye then tries to develop faster causing these new abnormal vessels to grow. The new vessels are not as well formed as the same vessels that would grow in normal infants. These new vessels may then cause traction on the retina, and may detach that part of the retina when traction has become too extensive.
Which babies are at risk of developing ROP?
Factors that may affect the development of ROP are. 1) Gestational age when the baby was born, 2) Low birth weight. 3) Exposure to oxygen. There has been several studies that state that the more premature or pre-term a baby is, the increased risk he/she has in developing this condition. The lower the birth weight of the baby, the greater the chances of having ROP. Since advancement in pediatric neonatology has enabled pediatricians to save more premature babies than before, more patients are seen with ROP. Premature babies when recuperating are usually exposed to oxygen levels that can stimulate the growth of these vessels.
Which babies should be screened for ROP?
Though not all premature babies are at risk of developing ROP, all babies weighing less than 1500 grams (3 pounds, 5 ounces) or born at or before 34 weeks of gestation are advised to be screened and have eye examinations, since these babies are at risk of developing ROP. Any babies weighing less than 1250 grams (2 pounds, 12 ounces) or born at less than 30 weeks of gestation are at greater risk of developing ROP. It is also thought that babies who are very sick at birth and who require oxygen might be at risk of developing ROP even if they weigh more than the above-mentioned limits and if they are older than noted above. These babies are also examined for ROP.
What are the different stages of ROP?
Retinopathy of prematurity is classified according to the severity of the changes of the blood vessels and the region of the retina into which the vessels have grown. The severity is referred to as the "Stage" and the retinal regions as "Zones".
Stages are as follow:
Immature vessels Normal but incomplete growth
Stage 1 Mildly abnormal growth
Stage 2 Moderately abnormal growth
Stage 3 Severely abnormal growth
Stage 4 Partially detached retina
Stage 5 Completely detached retina
Zones are as follow:
Zone I Earliest growth of the central retina which includes the macula
Zone II Middle region of growth in which most ROP occurs
Zone III Last region of growth
The "immature vessels" stage actually occurs in all infants, and does not necessarily lead to ROP. Also, when ROP regresses (goes away), the vessels may go through this stage again until they complete their growth.
Stage 1 is a mild abnormality of the retinal vessel growth and does not require treatment.
Stage 2 is a moderate abnormality of the retinal vessel growth and also does not require treatment.
Stage 3 is a severe abnormality of the retinal vessel growth in which the blood vessels grow toward the center of the eye instead of following their normal growth pattern along the surface of the retina. When a certain degree of Stage 3 is present and when "plus disease" develops, treatment is considered. "Plus disease" indicates that the blood vessels of the retina have become enlarged and twisted. This is an indication of worsening of the disease. Plus disease can occur with almost any stage and its presence alone is not sufficient to require treatment.
Stage 4 involves a partial detachment of the retina. Stage 4A indicates that the macula is still attached. No surgery is generally required for these cases. Stage 4B indicates that the macula is detached and surgery is suggested in some cases.
Stage 5 involves a complete detachment of the retina and surgery for these cases should be considered very carefully as prognosis in operated cases usually have poor porgnosis even in the best of hands.
What happens if the ROP does not go away?
Most of the time, Stages 1, 2, and even some Stage 3 cases may go away without treatment. In a small number of babies, for reasons not well understood as yet, ROP worsens and can develop into a sight-threatening condition. This occurs only about 10% of the time, in which case treatment is usually recommended by the doctor.
What treatments are there for ROP?
Starting in the late 1980's, treatments became available for ROP. The first treatments for ROP were "cryotherapy" or freezing treatments. A freezing probe was held onto the outside of the eye to freeze the peripheral retina (side of the retina). This caused the ROP to go away in many cases, and reduced the chances of retinal detachments and blindness by about 50% as compared to babies who had no treatment. Not all babies responded favorably to this treatment. More recently, lasers have been used for treatment of ROP, which appears to work better, but not all babies respond to this treatment either. Laser treatment is less painful, and causes fewer problems than the freezing treatments, but both treatments are accepted, and are still in use today.
Our treatment of choice is laser. The purpose of the treatment is to create scar tissue on the peripheral retina. This has been shown to eliminate ROP progression in many cases. The treated part of the retina will be scarred and will no longer work. The goal of the treatments is to save as much as possible of the central retina, where the best vision is located. Some of the peripheral retina and, therefore, some of the side vision will likely be lost after these treatments. It is important to keep in mind that the central retina, where the reading vision, straight ahead vision, and most of the color vision are located, is the most important part of the retina to save.
In recent years, anti-VEGF injections have been done on premature babies with ROP. Though there have been anecdotal reports of success of this treatment, this has NOT been collaborated by large scale randomized clinical trials, and anti-VEGF injections may have the potential risk of systemically affecting the infant, which can cause severe adverse effects which can be life-threathening.
How is the laser treatment done?
The laser treatment can be performed with the baby in the crib in the neonatal intensive care unit (NICU). The baby may be given medication to make it sleepy and comfortable. The baby's heart rate and breathing is monitored during the entire procedure. The laser beam is directed through the pupil to treat the side part of the retina. The procedure is similar to the examination with the addition of laser. The treatment usually takes 30-45 minutes per eye. Afterwards, the baby's eye may be red, and the eyelids may be red and a little swollen. Eye drops are used for about one week. The redness and swelling usually goes away in a few days but may take a few weeks to completely disappear. A follow-up exam is usually performed 2-3 weeks after the laser treatment.
What happens if the laser treatment does not work?
The biggest concern, if the ROP laser treatments do not work to halt the scar tissue growth, is the development of retinal detachment. Often, only part of the retina detaches. If only the peripheral retina detaches, no further treatments should be performed, since these peripheral detachments may remain the same or go away without treatment. If the center part of the retina or the entire retina detaches, then surgery is recommended to try to reattach the retina. This surgery involves removing the scar tissue inside the eye to help the retina to reattach.
Surgery is not recommended for distortion of the retina or for scar tissue that is not causing a detachment affecting the central vision. These cases are referred to as Stage 4A if the macula is not detached. Cases in which the center of the retina becomes detached are referred to as Stage 4B. Many Stage 4B cases have a thin fold through the macula and do not need surgery. Some Stage 4B cases that do not have a thin fold through the macula may benefit from surgery.
How is surgery for ROP done?
Surgery for ROP is usually the LAST resort for this problems. Depending on the stage, your pediatric retina surgeon may opt to do either a scleral buckle, a vitrectomy or both. He may even use some other materials to help flatten out the retina, such as intraocular gases, and heavy silicone oil. The objective of the laser is to try to attach the central part of the retina. If this is successful, then your baby MAY HAVE a chance to regain some vision back, which is usually only ambulatory vision (counting fingers, hand movement) but not vision not good enough to read or even drive.
Is the surgery always successful?
NO. Due to the very complex nature of this disease, success rate may depend on how early or late the patient is seen, how scarred the retina is, and how long the retinal detachment has been present. This also depends on the overall health of your baby, as a complicated NICU course will also ultimately affect the outcome of the surgery.
Anatomic success rates (retina has been re-attached) for patients who were seen very early in the disease have been reported to as high as 60% by some authors, but this goes down dramatically when delay in the surgery has been made. Majority of patients with an unsuccessful surgery will have shrinkage of the eye in due time
Anatomic success DOES NOT translate that the baby can see when he/she grows up, as there have been a lot of instances when the retina will re-detach despite the first successful surgery. Many patients who keep their retina attached do not also see when they grow up as the retina has already been scarred during the development. However, patients may still keep ambulatory vision good enough for them to move around without assistance
In the event that the retina has been re-attached successful with a scleral buckle alone, or combined with a vitrectomy, a second operation in necessary in 6 months to remove the scleral buckle because the buckle itself will prevent the normal growth of the eye.