Retinopathy of prematurity was first reported in 1942, so every ophthalmologist practicing today is familiar with this entity and its relationship to premature delivery and oxygen supplementation. We all remember as residents in ophthalmology struggling in the neonatal intensive care unit of our respective academic medical centers to examine these tiny and vulnerable infants with an indirect ophthalmoscope and scleral depression to make the judgment as to whether or not they were showing signs of ROP that required therapy. The responsible faculty, even 45 years ago when I trained, were pediatric ophthalmologists and retina specialists. It remains so today.
The usual infant at risk weighs less than 1,250 g at birth and has a gestation period of less than 31 weeks. Of the approximately 4 million infants born in the U.S. each year, about 28,000 fit these criteria. Of the 28,000 at risk for ROP, about 15,000 premature infants manifest signs of stage 1 to 5 ROP, as discussed in the accompanying cover story. Of the 15,000 with ROP, 90% have mild enough disease that they do not require treatment. Only 1,500 of these premature infants develop ROP severe enough to require therapy, usually stage 3 disease. This is a very small number. We have close to 1,500 retina specialists in the U.S. and about 750 pediatric ophthalmologists, so that is only one case per retina specialist and perhaps two per pediatric ophthalmologist.
The diagnosis and treatment of ROP in the first years of life are the responsibility of the academic fellowship-trained pediatric and retina specialist, not the comprehensive ophthalmologist. However, in later life, these patients also develop retinal detachments, pathologic myopia, strabismus, amblyopia and glaucoma. As the patient ages, the management of these comorbidities and the requirement for specialty glasses, low vision aids and training for the visually handicapped often require engagement of the comprehensive ophthalmologist, especially in rural communities.
The treatment regimens for stage 3 and above ROP include laser photocoagulation, cryotherapy, anti-VEGF intravitreal injections, and oral propranolol alone or in combination. For severe disease, a vitrectomy and retinal detachment repair may be required. For the premature infant born in a community where no ophthalmologist is available, telemedicine with the RetCam 3 from Natus Newborn Care is proving effective in determining which infants need referral for treatment and which can be followed locally.
The patient with ROP requiring treatment is, in my opinion, extraordinarily difficult to manage, and even among experts, who to treat and how to treat are intensely debated. I can only take my hat off to those who have stepped forward to help these very challenged infants and their often distraught parents.
Disclosure: Lindstrom reports no relevant financial disclosures.