There is a classic mythology regarding retinopathy of prematurity (ROP). One story line in this mythology is that, if we could only develop new therapies for this blinding disease, we could save many infants from a lifetime of blindness. The reality, however, is that most of these babies go blind not for lack of treatment, but because they were not examined in a timely fashion, for a variety of reasons. When infants are screened, and those needing treatment are identified and treated in a timely manner, intervention is highly effective in preserving vision.1

Telemedicine offers a unique opportunity to eliminate or greatly reduce blindness from ROP. We know whom to screen for ROP, and we know when to screen. We also know that, with the care of experienced specialists, blindness from ROP is preventable in up to 99% of babies receiving treatment for ROP.2 This suggests that we must change our paradigm, our goal, from seeking new treatments to ensuring all eligible babies are in fact being screened, so that they can be offered appropriate and timely treatment. Telemedicine is poised to help us do that.

Screening for ROP is currently performed in most situations with binocular indirect ophthalmoscopy (BIO). Among the advantages of BIO, it is a familiar modality that provides a 3-D view, and it is easy to manipulate the patient to improve the view. However, BIO also has significant disadvantages that are not often highlighted: It lacks reproducibility and precision, does not facilitate longitudinal tracking, provides no hard copy, requires interpretation (zone, stage, and presence of plus disease), and is not useful for education or conducive to use in studies. In addition, there can be gaps in continuity in the event an experienced screener is unavailable.

Screening Crunch

Currently in the United States there is an ROP screening crunch due to the confluence of several factors. In 2006 there was a major revision of recommendations for screening,3 which led to increased eligibility for screening, increased frequency of screening, and more intensive exams per patient. In the same year, a survey by the American Academy of Ophthalmology of members who provided ROP screening (www.aao.org) found that only 77% of those currently providing the service intended to continue doing so, for the most part due to financial issues.

Taken together, these changes meant that the number of infants to be screened increased by a third, with each infant needing more examinations, but at the same time there was a decrease in the number of ophthalmologists with sufficient knowledge, experience, and willingness to provide the screening.

Promise of Telemedicine

With an increase in the demands of screening and a reduction in the work force to carry it out, the pool of existing talented, experienced ROP screeners must be better leveraged to accomplish the job. Telemedicine allows the opportunity to achieve this.

The Photo-ROP trial,4,5 the design of which included a direct comparison of telemedicine with bedside BIO, found the 2 technologies to be essentially equivalent. The Photo-ROP results highlighted the fact that BIO provides interpretations, and, while in practice these interpretations are always assumed to be correct, this was not always the case in the study.

An ongoing multicenter trial, Telemedicine Approaches to Evaluating Acute-phase ROP, or e-ROP (http://clinicaltrials.gov/ct2/show/NCT01264276), is also comparing remote evaluations to the so-called “gold standard” of BIO. The choice of comparator is unfortunate, because another storyline in the mythology of ROP is that all screeners are equivalent, but this is not the case. In reviewing medicolegal literature, one can identify cases in which, on the same day, the referring physician identifies zone III, stage 1 disease, and the treating physician calls it a zone I, stage 3 eye. Both interpretations are presumed to be correct, but obviously one is wrong.

The advantages of photographic telemedicine screening are the exact inverse of the disadvantages of BIO. Remote photographic assessment offers reproducibility, precision, the ability to track patients longitudinally, the ability to produce hard copy for the medicolegal record, and amenability to educational purposes and studies. 6 Photographic telemedicine leverages screeners and allows continuity of screening. It allows one to characterize zone, stage, and plus disease based on the optics of the camera rather than a drawing.

There are disadvantages to photoscreening. The cameras require a significant investment by each neonatal intensive care unit (NICU) involved in a remote screening program, and use of the camera requires training. The medium provides only 2-D images. And there is a bias in the community toward the more traditional bedside BIO. Fortunately, the new joint statement screening guidelines support the use of photoscreening with the proviso that all babies have 1 binocular indirect ophthalmoscopy examination prior to termination of screening.7

SUNDROP Network

The Stanford University Network for Diagnosis of ROP (SUNDROP)8 is a hub-and-spoke, store-and-forward telemedicine screening program. The central hub at the university is a single remote screener who reads images generated by cameras (RetCam; Clarity Medical Systems) at each of 6 peripheral NICUs. The protocol for photographs, based on the Photo-ROP trial,5 includes an iris view and 5 views of the fundus in each eye, a total of 12 standard photographs for a patient's 2 eyes.

The screener looks for all referral-warranted (RW) and/ or treatment-warranted (TW) disease. The camera provides adequate visualization of zone II, and it is good at highlighting plus disease (Figures 1 and 2).

To date, with more than 7 years of data, the SUNDROP telemedicine program has achieved 100% capture of more than 600 premature infants born at 6 centers over 7. Screening sensitivity was 100%, and specificity was in excess of 99.5%, with high negative and positive predictive values.9

The current technology has limitations: Because the camera cannot reliably and reproducibly photograph zone III, it is not possible to discharge infants from acute phase screening of ROP based on the joint statement screening guidelines.7 Additionally, it is difficult to routinely screen babies using the camera upon discharge from the NICU because the babies have grown in size and strength.

In the future we hope to automate the system so that the camera automatically syncs with the remote server. We would also like to institute a tracking system similar to what is used with hearing tests.

Another exciting possibility is to expand beyond ROP and do universal screening of newborns, which is currently occurring in Brazil and China. (See Universal Eye Screening in Healthy Neonates.) The institutional review board at Stanford recently approved our evaluation of the efficacy of such an effort in our community. A single screening could lead to early identification of cataract, vitreous hemorrhage, congenital glaucoma, coloboma, hamartoma, retinoblastoma, and nerve anomalies, possibly resulting in improved functional outcomes.

Conclusion

Screening is vital for the prevention of blindness from ROP. Telemedicine is potentially a highly effective screening tool that allows the leveraging of skilled ROP screeners. We look forward to expanded use of remote screening in other centers, modeled on the successful SUNDROP program.

Darius M. Moshfeghi, MD, is an Associate Professor of Ophthalmology at Stanford University and Founder and Director of the SUNDROP Network. He states that he has no financial relationships to disclose. Dr. Moshfeghi can be reached at +1 650 721-6888; or at dariusm@stanford.edu.

  1. Cryotherapy for Retinopathy of Prematurity Cooperative Group. Multicenter Trial of Cryotherapy for Retinopathy of Prematurity: ophthalmological outcomes at 10 years. Arch Ophthalmol. 2001;119:1110-1118.
  2. Drenser KA, Trese MT, Capone A Jr. Aggressive posterior retinopathy of prematurity. Retina. 2010;30(4 Suppl):S37-40.
  3. Section on Ophthalmology American Academy of Pediatrics; American Academy of Ophthalmology; American Association for Pediatric Ophthalmology and Strabismus. Screening examination of premature infants for retinopathy of prematurity. Pediatrics 2006;117:572-576. Erratum in: Pediatrics. 2006;118:1324.
  4. Photographic Screening for Retinopathy of Prematurity (Photo-ROP) Cooperative Group; Balasubramanian M, Capone A Jr, Hartnett ME, et al. The Photographic Screening for Retinopathy of Prematurity Study (Photo-ROP): study design and baseline characteristics of enrolled patients. Retina. 2006;26(Suppl): S4-S10.
  5. Photographic screening for retinopathy of prematurity (Photo-ROP) cooperative group. The photographic screening for retinopathy of prematurity study (Photo-ROP): primary outcomes. Retina. 2008;28(Suppl):S47-S54.
  6. Chiang MF, Melia M, Buffenn AN, et al. Detection of clinically significant retinopathy of prematurity using wide-angle digital retinal photography: a report by the American Academy of Ophthalmology. Ophthalmology. 2012;119(6):1272-1280.
  7. Fierson WM; American Academy of Pediatrics Section on Ophthalmology; American Academy of Ophthalmology; American Association for Pediatric Ophthalmology and Strabismus; American Association of Certified Orthoptists. Screening examination of premature infants for retinopathy of prematurity. Pediatrics. 2013;131(1):189-95. doi: 10.1542/peds.2012-2996.
  8. Murakami Y, Jain A, Silva RA, Lad EM, Gandhi J, Moshfeghi DM. Stanford University Network for Diagnosis of Retinopathy of Prematurity (SUNDROP): 12-month experience with telemedicine screening. Br J Ophthalmol. 2008;92(11):1456-1460.
  9. Moshfeghi DM. Update on the Study of Telemedicine for ROP. Paper presented at: American Academy of Ophthalmology Annual Meeting, Retina Subspecialty Day; November 9-10, 2012; Chicago, IL.