Retinopathy of prematurity remains the leading cause of blindness in children in the United States. According to the National Eye Institutes website, 400 to 600 children become blind from ROP each year (http://www.nei.nih. gov/health/rop/#3). This represents almost 1 percent of the 60,000 to 80,000 premature infants eligible for ROP screening each year.1 It is more distressing when one considers that effective treatment strategies have existed for 20 years.2-6 In this environment, where treatment is usually effective if initiated early for appropriate levels of disease, the onus is on the medical community to ensure that all eligible patients are offered screening.

Unfortunately, the picture is more complicated. ROP screening remains the single highest risk endeavor in the practice of ophthalmology. In the face of high medicolegal risk with accompanying large financial settlements, many trained practitioners are simply unwilling to provide the necessary screening services. Additionally, these services are not well-compensated, often times being reimbursed by Medicaid. The American Academy of Ophthalmology commissioned a survey of its members who screened for ROP in 2006 and discovered that fully 23 percent of those already screening were planning on discontinuing in the immediate future (aao.org). The top reasons for discontinuing provision of these services revolved around financial issues—medicolegal risk, low reimbursement, lack of hospital support and complexity of patient care scheduling. To compound matters, the screening criteria were revised in 2006.7 These changes included the following: increasing the gestational age from 28 to 30 weeks post menstrual age (PMA), changing the initial screening date to 31 weeks PMA or four weeks chronological age (whichever is later), increasing the screening frequency for high-risk disease, and delineating termination criteria for acute-phase ROP screening.7 The cumulative result of these changes was to increase the number of eligible babies, increase the duration of the screening period, and to increase the number of examinations per baby per period. In fact, using the new criteria and applying it to the 2004 U.S. birth data, approximately 80,000 patients would be eligible for ROP screening, up from 60,000 using the old criteria.1,7 In the face of a 23 percent decrease in eligible and willing screeners and a 33 percent increase in eligible patients, the ROP screening paradigm needs to be re-examined.

The present gold standard for ROP screening is binocular indirect ophthalmoscopy by an ophthalmologist experienced in the sequential changes of ROP.7 This recommendation comes from the Policy Statement on ROP screening from the AAO, the Section on Ophthalmology from the American Academy of Pediatrics, and the American Association for Pediatric Ophthalmology and Strabismus.7 Interestingly, there has never been a study validating the reproducibility or reliability of this technique.

This is not a diabetic screening examination performed in the comfort of the office. Most ROP screening is initiated in the neonatal intensive care unit (NICU). These are small, premature babies with multiple medical problems and are prone to episodes of apnea, desaturation, bradycardia and tachycardia during the examination. The nurse is often right by the elbow gently encouraging the examiner to "move it along." Oftentimes, the parent is at the bedside or just outside the room. It is in this environment that the bedside binocular indirect ophthalmoscopy examination is performed, following which a drawing is produced for each eye depicting the presence or absence of disease (See Figure 1). Fortunately, there is a common language within the framework of the International Classification of ROP to describe the findings.8,9 Still, I find it troubling that this method has not been rigorously investigated.

We know from studies on fluorescein angiograms that there is wide variability in inter-observer as well as intra-observer interpretation of findings.10-12 Typically, fluorescein angiogram reading is not in the same sort of stressful environment as the NICU ROP examination. Additionally, the fluorescein angiogram has the advantage of not being a moving target—while the observers may change in person or time, each frame of the fluorescein angiogram remains fixed for eternity. This is not the case in ROP screening, where each individual will create the drawing that he sees. These drawings, in my experience, tend to focus on the advancing edge of disease. It seems reasonable that there may be great disparity in the depiction of the status of the fundus from one individual to another in this sort of environment.

A growing body of evidence has been published on the utility of telemedicine for ROP screening using the RetCam (Clarity Medical Systems, Pleasanton, Calif.).13-25 Additionally, the PhotoROP trial results were presented at the Retina subspecialty day at the AAO. The PhotoROP trial was a multicenter, prospective trial to evaluate telemedicine screening with the RetCam relative to bedside indirect ophthalmoscopy.26 The preliminary results indicated that remote interpretation of RetCam images had a sensistivity of 100 percent and specificity of 97 percent when compared to bedside binocular indirect ophthamolscopy. Furthermore, the screening center readers recommended treatment two weeks earlier than the bedside screeners, possibly indicating the advantages of longitudinal examination with photographs.

Four years ago I initiated a series of discussions with the administration of Lucile Packard Children's Hospital at Stanford University regarding the feasibility of providing telemedicine services using the RetCam II at their affiliated NICUs. I had been providing these services as a stop-gap after the long-time screeners had discontinued their services for the reasons outlined in the AAO survey. The end result was the Stanford University Network for Diagnosis of Retinopathy of Prematurity. SUNDROP is a community-based telemedicine initiative that relies on individuals in the NICU to identify patients eligible for screening, to photograph the fundus, and to transfer the images to me for interpretation. The basic model is a hub-and-spoke design familiar to the business traveler (See Figure 2). The goals are straightforward: 1) to perform screening examinations for ROP; 2) to identify patients that have a need for bedside indirect ophthalmoscopy; 3) to standardize the screening process within the community, 4) to provide access to subspecialty care to the community hospital; 5) to provide hard-copy objective documentation of the screening examination; and 6) to allow the patient to remain in the community NICU until referral-warranted disease is identified. We borrowed from the PhotoROP trial the concept of five standard fundus photographs in each eye, as well as an anterior segment photo (See Figure 3). Ideally, there are six photographs per eye. In each NICU, several nurses are identified to receive training in the operation of the camera. This training is provided by Clarity using one of their certified ophthalmic photographers. I then generally provide follow-up training as necessary. Once images are received by me, I generate a report within 24 hours outlining the interpretation as well as disposition for next examination.

SUNDROP does not seek to supplant indirect ophthalmoscopy, but rather to streamline the process, identifying the patients with the greatest risk for needing therapeutic intervention. The endpoints are straightforward—identification of referral-warranted ROP (RW-ROP), treatment, or discharge from the NICU. In the SUNDROP network, all babies discharged from the NICU are seen in my outpatient clinic within 72 hours for indirect ophthalmoscopy with scleral depression. Referral-warranted ROP has been classified to include Type 1 or Type 2 ETROP disease, stage 3 disease, plus or pre-plus, and threshold disease.

Presently, four sites are enrolled in SUNDROP. Acceptance of the technique and methodology has been high. Results of the first 12 months of the SUNDROP initiative will be presented at this month's AAO meeting in New Orleans.

There is an increasing burden on the practitioners who provide ROP screening services, both from increased eligible infants as well as declining pool of willing screeners. Telemedicine screening for ROP offers the potential to leverage the skills of those few practitioners remaining who wish to continue providing these services.

 

Dr. Moshfeghi is an assistant professor of ophthalmology in Adult and Pediatric Vitreoretinal Surgery and is co-director of Ocular Oncology at Stanford University. Contact him at 1225 Crane St., Ste. 202, Menlo Park, Calif. 94025. Phone: (650) 323-0231; fax: (650) 323-6385; or e-mail: darius m@stanford.edu. He is on the scientific advisory board, Clarity Medical Systems (maker of the RetCam).

 

1. Martin JA, Hamilton BE, Sutton PD, Ventura SJ, Menacker F, Kirmeyer S. Births: Final Data for 2004. National Vital Statistics Report, 2006; 55(1):1-104.

2. Multicenter trial of cryotherapy for retinopathy of prematurity. Preliminary results. Cryotherapy for Retinopathy of Prematurity Cooperative Group. Arch Ophthalmol 1988;106:471-9.

3. Multicenter trial of cryotherapy for retinopathy of prematurity. Three-month outcome. Cryotherapy for Retinopathy of Prematurity Cooperative Group. Arch Ophthalmol 1990;108:195-204.

4. Multicenter trial of cryotherapy for retinopathy of prematurity. One-year outcome—structure and function. Cryotherapy for Retinopathy of Prematurity Cooperative Group. Arch Ophthalmol 1990;108:1408-16.

5. McNamara JA, Tasman W, Vander JF, Brown GC. Diode laser photocoagulation for retinopathy of prematurity. Arch Ophthalmol 1992;110:1714-16.

6. Hunter DG, Repka MX. Diode laser photocoagulation for threshold retinopathy of prematurity. A randomized study. Ophthalmology 1993;100:238-44.

7. Screening examination of premature infants for retinopathy of prematurity. Pediatrics 2006;117:572-6.

8. An international classification of retinopathy of prematurity. The Committee for the Classification of Retinopathy of Prematurity. Arch Ophthalmol 1984;102:1130-4.

9. The international classification of retinopathy of prematurity revisited. Arch Ophthalmol 2005;123:991-999.

10. Friedman SM, Margo CE. Choroidal neovascular membranes: Reproducibility of angiographic interpretation. Am J Ophthalmol 2000;130:839-41.

11. Holz FG, Jorzik J, Schutt F, et al. Agreement among ophthalmologists in evaluating fluorescein angiograms in patients with neovascular age-related macular degeneration for photodynamic therapy eligibility (FLAP-study). Ophthalmology 2003;110:400-5.

12. Kaiser RS, Berger JW, Williams GA, et al. Variability in fluorescein angiography interpretation for photodynamic therapy in age-related macular degeneration. Retina 2002;22:683-90.

13. Lorenz B, Bock M, Muller HM, Massie NA. Telemedicine based screening of infants at risk for retinopathy of prematurity. Stud Health Technol Inform 1999;64:155-63.

14. Schwartz SD, Harrison SA, Ferrone PJ, Trese MT. Telemedical evaluation and management of retinopathy of prematurity using a fiberoptic digital fundus camera. Ophthalmology 2000;107:25-8.

15. Roth DB, Morales D, Feuer WJ, et al. Screening for retinopathy of prematurity employing the retcam 120: sensitivity and specificity. Arch Ophthalmol 2001;119:268-72.

16. Yen KG, Hess D, Burke B, et al. The optimum time to employ telephotoscreening to detect retinopathy of prematurity. Trans Am Ophthalmol Soc 2000;98:145-50; discussion 50-1.

17. Yen KG, Hess D, Burke B, et al. Telephotoscreening to detect retinopathy of prematurity: Preliminary study of the optimum time to employ digital fundus camera imaging to detect ROP. J AAPOS 2002;6:64-70

18. Ells AL, Holmes JM, Astle WF, et al. Telemedicine approach to screening for severe retinopathy of prematurity: A pilot study. Ophthalmology 2003;110:2113-7.

19. Mehta M, Adams GG, Bunce C, et al. Pilot study of the systemic effects of three different screening methods used for retinopathy of prematurity. Early Hum Dev 2005;81(4):355-60.

20. Chiang MF, Starren J, Du YE, et al. Remote image based retinopathy of prematurity diagnosis: A receiver operating characteristic analysis of accuracy. Br J Ophthalmol 2006;90:1292-6.

21. Mukherjee AN, Watts P, Al-Madfai H, et al. Impact of retinopathy of prematurity screening examination on cardiorespiratory indices: a comparison of indirect ophthalmoscopy and retcam imaging. Ophthalmology 2006;113:1547-52.

22. Shah PK, Narendran V, Saravanan VR, et al. Screening for retinopathy of prematurity—a comparison between binocular indirect ophthalmoscopy and RetCam 120. Indian J Ophthalmol 2006;54:35-8.

23. Wu C, Petersen RA, VanderVeen DK. RetCam imaging for retinopathy of prematurity screening. J Aapos 2006;10:107-11.

24. Chiang MF, Keenan JD, Starren J, et al. Accuracy and reliability of remote retinopathy of prematurity diagnosis. Arch Ophthalmol 2006;124:322-7.

25. Chiang MF, Jiang L, Gelman R, et al. Interexpert agreement of plus disease diagnosis in retinopathy of prematurity. Arch Ophthalmol 2007;125:875-80.

26. 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(7 Suppl):S4-10.