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All ophthalmologists should be familiar with ROP and its treatments
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 Richard L. Lindstrom |
Retinopathy of prematurity is an especially poignant topic for me. This past summer, a relative from Wisconsin, after a complicated pregnancy, gave birth to a baby at 22 weeks’ gestation. I had recommended they seek treatment at the Medical College of Wisconsin, and she and her baby received outstanding care. The neonatal intensive care unit saved the baby, and the department of ophthalmology managed the baby’s ROP. This event made me realize that every ophthalmologist is best informed about the current diagnosis and therapy of ROP, so that they can counsel patients and their families as needed. So, while I am far from an expert on this topic, I will share a few thoughts from my personal experience.
First, infants at risk for ROP are generally those born before 30 weeks’ gestation or weighing less than 1,500 grams at birth. They are tiny infants, and it is an amazing testament to the care available in a modern neonatal intensive care unit that so many survive. However, this increased survival of premature infants also means more ROP. I learned that while too much oxygen can be a problem, so can too little, and many factors other than well-managed oxygen therapy play a role in the development of ROP.
All infants in the high-risk group require screening for ROP, which can be done at 4 to 6 weeks after birth using indirect ophthalmoscopy or, in some remote locations, with photographs using the Clarity RetCam, which are then reviewed by an expert — a good example of telemedicine. Examinations are recommended every 1 to 3 weeks thereafter, depending on the disease stage and the therapy initiated.
The pathophysiology is related to the embryology of the retina. The blood vessels of the retina begin to develop at about 12 weeks and do not complete their development until the baby is near full term 6 months later. VEGF plays a role in the development of the normal retinal vasculature, and this has led to recent use of anti-VEGF compounds such as Avastin (bevacizumab, Genentech) in the therapy of ROP. The classic therapy remains laser photocoagulation and/or cryotherapy. Combinations of these three therapeutic modalities are currently under study, and there is controversy as to the ideal therapy.
The disease is staged at examination from stage 1 to 5, and there is also plus disease, in which vessels are particularly dilated and tortuous. Those interested can look up the stages in a textbook, but very simply, stage 1 disease starts with an avascular periphery in which retinal vascular development has not occurred, transitions to an elevated ridge at the junction between vascular and avascular retina at stage 2, then to fibrovascular proliferation at stage 3, subtotal retinal detachment in stage 4 and total retinal detachment in stage 5. In stages 4 and 5, when retinal detachment is present, vitrectomy and scleral buckling surgery are often necessary.
Management by experts is ideal, often a pediatric ophthalmologist and a vitreoretinal specialist working together, and therapy is lifelong, as many patients develop severe vision loss, secondary extreme myopia, cataract, glaucoma, strabismus and nystagmus. While there are many medical issues to deal with, from vision loss to cosmesis, there is also a great need for compassionate psychological support and counseling. Parents and families are very traumatized and stressed by the experience, and the natural progression of grieving, from denial to anger, then bargaining, depression and hopefully finally healthy acceptance, is to be expected. During the anger stage, many families turn to lawyers, and it was interesting to me that when I Googled ROP, one of the sponsored links was for a law group specializing in ROP lawsuits.
In the modern world, this is an important management issue for physicians routinely engaged in the care of these challenging patients and their families. When interacting with my own relatives, I found it best to only answer questions when asked, and I routinely deferred to the baby’s treating physicians. While I strongly believe from my observation that the baby will at best be 20/200, I have decided not to share this thought with the family. It is best, in my opinion, at this early stage to remain optimistic, as positive surprises and, in my experience after 40 years of ophthalmology, even miracles do occur. There is nothing to be gained by extinguishing hope.
In addition, I found sharing a few uplifting examples of those who have gone on to lead extremely productive lives in the face of severe visual loss from ROP reassuring. Well-known examples include Stevie Wonder and Tom Sullivan, who many of us have met, as he remains heavily engaged with ophthalmology and is an extraordinary partner in supporting the value of increased private and public funding of eye research. There are many others. I hope the application of new therapies such as anti-VEGF agents proves helpful, but when ROP hits close to home, it makes one appreciate not only the quality of care available to our patients, but how blessed those of us are whose children have healthy, normal eyes.