Premature babies are at risk of an eye disease called Retinopathy of Prematurity (ROP), which may, in serious cases lead to blindness. This is why premature babies should be regularly screened in line with the 2008 National Guidelines.
In most cases, even if a baby does develop ROP, it will resolve spontaneously and treatment will not be needed. For a small number of babies, ROP worsens, often very rapidly, but with timely treatment the prognosis is good.
Sadly, ROP occasionally gets missed and by the time it is detected, the baby has suffered permanent visual loss.
Janine Collier, Head of the Medical Negligence team at Tees, has specific expertise in helping families whose child has suffered permanent visual impairment because of a failure to detect and treat ROP.
What is retinopathy of prematurity?
Retinopathy of prematurity (ROP), also called retrolental fibroplasia (RLF) and Terry syndrome is where the blood supply to the retina does not develop normally as a consequence of being born prematurely. The retina is the layer of tissue that lines the back of the eye and makes it possible to see.
Your baby’s eyes begin to develop around the 16th week of pregnancy. If he or she is born very early, this process is cut short. The blood vessels in the inner retina do not develop a blood supply until much later in the pregnancy and this process does not complete until the end of the pregnancy. Therefore, if your baby is born prematurely, the inner retina has an incomplete blood supply. The more premature the birth, the less complete the blood supply present.
In most cases, the premature baby’s eyes will develop normally. However, in some cases, the blood vessels in the inner retina do not grow normally. They may grow into other parts of the eye such as the clear gel that fills the space between the lens and the retina of the eyeball and cause bleeding / leaking. The vessels may also change physically, to pull the retina, and, if extensive enough, cause the whole retina to detach from the eye.
Over time, these blood vessels and the associated scar tissue can cause other serious vision problems, such as:
- Crossed eyes (strabismus)
- Increased eye pressure (glaucoma)
- "Lazy eye" (amblyopia)
- Near-sightedness (myopia)
The good news is that with early diagnosis and treatment, most babies will retain a normal structural eye, with good vision.
What are the risk factors for ROP?
The risk of ROP increases with increased prematurity and the smaller your baby is at birth, the greater the chance of her or him having retinopathy of prematurity (ROP). There are other factors which contribute to the risk of ROP, including:
- Bronchopulmonary dysplasia
- Chronic lung disease
- Other inter-current illnesses such as infections, cardiovascular instability
- Poor post-natal weight gain
How do I know if my baby has ROP?
Only an ophthalmologist can tell if your baby has ROP. All infants who are at risk for it should be screened in line with the 2008 National Guidelines. The timing of the first screening examination will depend on your baby’s gestational age at birth.
The baby is given eye drops to make her or his pupils larger ahead of the examination. This helps the doctor see all the parts of the eye better. It doesn't hurt.
As the screening is not an especially comfortable examination, local anaesthetic eye drops are usually used alongside comfort care techniques, such as swaddling, oral sucrose or expressed milk, a dummy / pacifier. Experienced ophthalmologists can usually undertake the examination in 2-3 minutes. Each eye is fully examined to check for ROP.
Screening is usually carried out two-weekly, and, if no cause for concern, stops at around 36 weeks gestational age.
If the ophthalmologist identifies early signs of ROP, the interval may be shorter as the doctor will watch to see if the condition requires treatment, or if it resolves spontaneously. Most babies with ROP will resolve spontaneously.
If my baby has ROP, what treatment will he or she need?
In the UK, approximately 4% of cases of ROP require treatment. The purpose of treatment is to preserve the anatomy of the retina, by preventing retinal detachment.
If the doctor recommends treatment, this is usually given within 48-72 hours.
Most commonly, ROP is treated with laser to the affected part of the retina.
Your baby may suffer some side effects, including inflammation (typically treated with a short course of steroid eye drops and pupil dilating eye drops for 1 to 2 weeks). Rarely, other side effects may occur. Your baby’s doctor should explain these to and discuss these with you prior to treatment.
I am worried about the care my baby is receiving – what should I do?
You might already have tried to talk to doctors about your baby’s condition but sometimes it can feel like you’re on your own. With Tees by your side, you are not. We understand what you're going through, and we're here to give you a voice.
We've handled many medical claims and have a particular expertise in supporting families with babies and young children, so our team of lawyers really do have the practical experience to support you. We're persistent, and we'll fight to get answers for you.
Baby A was born prematurely at 25 weeks and 4 days gestation by caesarean section. He suffered respiratory distress, was intubated and transferred to the NICU for a period of intensive care and specialist support. He remained ventilated for 9 days, spent 22 days in Intensive Care and 7 days in the high dependency unit. During his admission, he received antibiotic treatment for sepsis, insulin for hyperglycaemia and two blood transfusions.
Baby A’s parents understood that he was at risk of Retinopathy of Prematurity (ROP). However, his parents also understood that their baby would be regularly screened, so that if there were signs of ROP, he could receive treatment, significantly reducing the risk of loss of vision.
Five weeks after birth, Baby A had his first screening for ROP. At the time of the exam, Baby A was still small and in an incubator. When screened, each eye examination took around 10 minutes. Parents were advised that he would be screened every two weeks.
Baby A was transferred to a Special Care Baby Unit at a local hospital. Parents felt that the care at the local hospital was very different to what they had experienced at the previous hospital. They felt the staff treated them as over anxious young parents and did not encourage them to be a part of their baby’s care, which was unlike their experience at the previous hospital.
Whilst at the local hospital, Baby A was screened twice for ROP over a six-week period. Prior to being discharged, the Ophthalmologist spent an extended period of time examining Baby A’s eyes. During the exam the Ophthalmologist cut the white part of Baby A’s eye. After a time the despite the Ophthalmologist being unable to get a clear view, Baby A was discharged from hospital.
At a paediatric follow up the following month, Baby A’s parents told the Paediatrician that they were worried about his eyes as he would not look, follow or track. The Paediatrician said that it was just because he was a young premature baby and that they should not worry.
One month later, Baby A was referred to Great Ormond Street (GOSH) for assessment. Baby A’s parents were told that he had suffered significant and severe visual loss, equivalent of Stage 5 ROP / retinal detachment in the left eye and Stage 4a ROP in the right eye.
Baby A had surgery on the right eye at GOSH. The estimate is that post-operation he may have 2% vision in his good right eye since having the operation. It was not possible to offer Baby A any treatment for his left eye.
The hospital that treated Baby A has since undertaken a full investigation and a review of the care that he received.
As a result of the review, the hospital has changed their process and procedure for ROP screening. The Ophthalmologist no longer undertakes ROP screening and the Paediatrician no longer reviews premature babies.
Baby C was born at 24 week’s gestation, weighing c. 600 grams. She suffered several complications because of her extreme prematurity including Respiratory Distress Syndrome, Chronic lung disease, Hypertension, a patent ductus arteriosus, sepsis, hyperglycaemia and necrotizing enterocolitis.
Due to her prematurity and low birth weight, C was at high risk of suffering from ROP.
Baby C was examined by an ophthalmologist at, 7 weeks old, 8 weeks old, 9 ½ weeks old
On all occasions, it was noted that there was no ROP.
At ten and a half weeks of age, Baby C was again examined. The ophthalmologist found and recorded a “definite progression of ROP stage 3 zone 2 in both eyes, + disease”.
Treatment by both laser and cryotherapy was undertaken, but, the disease being so extensive now, was unsuccessful.
ROP behaves in a highly predictable manner and, we were, therefore, able to infer that at the time of the examination when C was 9 ½ weeks of age, it is implausible that there was no ROP present. The examination must, therefore, have been substandard. With a competent examination, C would have been referred for urgent laser treatment and, on the balance of probabilities, she would have retained good functional vision in both eyes.
Baby C lost all vision in her left eye, and has a shrunken eye. She has lost most useful vision in her right eye. She is at risk of retinal detachment, retinal degeneration, the need for surgical treatment of the band keratopathy, glaucoma and shrinkage of the right eye.
C also suffers from learning, behavioural and social difficulties because of her extreme prematurity. Her visual impairment has compounded her other developmental problems.