Retinopathy of prematurity (ROP) is a developmental disorder of the eye occurring in small, very premature neonates. In some cases, if untreated, it results in tractional retinal detachment and blindness.
Severe ROP must be diagnosed in a timely manner and treated effectively to avoid progression to blindness. This updated policy statement outlines an effective program that can be implemented in all neonatal intensive care units (NICUs) to identify infants at risk for this severe problem and enable timely treatment.
Since 1997, the AAP — in conjunction with the American Academy of Ophthalmology, American Association for Pediatric Ophthalmology and Strabismus, and since 2013, the American Association of Certified Orthoptists — has issued guidance on this topic. The latest version, Screening Examination of Premature Infants for Retinopathy of Prematurity, is available at http://pediatrics.aappublications.org/content/early/2018/11/21/peds.2018-3061 and will be published in the December issue of Pediatrics.
Six recommendations cover which infants are at risk, examination technique, scheduling initial and follow-up examinations, termination of examinations, photo-screening for ROP with remote interpretation, treatment and follow-up, transition of care to secondary hospitals and discharge from hospital care.
Important updates
Several changes from previous versions are noteworthy.
New sections address the method of utilization of digital wide-angle ocular fundus photography with remote interpretation via the internet.
NICUs, especially those remote from university hospital settings, have had the ability to screen for ROP in at-risk babies using this methodology. This statement provides a scheme for this technique to be performed safely within the regular framework of examinations provided for all at-risk premature infants who previously were examined only using bedside indirect ophthalmoscopy by an ophthalmologist. The statement highly recommends continued inclusion of that examination at least once before discharge or treatment for ROP.
Another section discusses alternative treatment for aggressive posterior ROP (AP-ROP) with injection of anti-vascular endothelial growth factor (VEGF) medications and its implications. An important change from previous versions is a large section on the treatment of AP-ROP with intra-vitreal injections of anti-VEGF medications. It is thought that the inappropriate proliferation of extra-retinal blood vessels into the vitreous in AP-ROP is caused by high levels of VEGF within the eye, and there is good laboratory evidence for this mechanism.
Several VEGF antagonist medications have been available for treatment of other conditions of the eye, including proliferative diabetic retinopathy and proliferative sub-retinal maculopathy via intra-vitreal injection of anti-VEGF. Two of these now are used for ROP — bevacizumab (Avastin), the most widely used, and ranibizumab (Lucentis). It has been shown that in many clinical situations these are as effective in causing regression of disease as laser peripheral retinal ablation, the gold-standard therapy for ROP.
The statement recommends consideration of use of these compounds and outlines requirements for their use. However, it notes that the regression caused by anti-VEGF is not the same as that caused by laser ablation. While anti-VEGF causes less retinal damage than laser ablation, it permits a much higher level of recurrence of ROP for a much longer time.
Postscript for pediatricians
The implications of the inclusion of anti-VEGF medications into practice are important for general pediatricians who care for NICU graduates. The recurrences of ROP reported in the literature peak between 45-55 weeks’ gestational age, and some have been reported as late as 70 weeks. This usually is after hospital discharge for most premature infants, so recurrence and possible disease progression to blindness may be occurring after the baby enters the pediatrician’s practice.
Therefore, it is important for pediatricians to be aware of the progressive nature of ROP and of the requirements for scheduled examinations in this statement and do everything in their power to facilitate and encourage parents to attend their baby’s scheduled examinations.
Dr. Fierson, the lead author of the policy statement, has chaired the AAP Subcommittee on Retinopathy of Prematurity since 1990.
