Fig. 5.1
This is a copy of a FocusROP report, which can include images of the retina with Zone 1 drawn or montaged images
The FocusROP (2.0) software is a smart software image-handling program that attempts to eliminate two areas of human error. The Focus program takes images that are captured by the NICU nurse—a pupil image, a posterior pole image, a temporal nasal image, superior and inferior image. The images are uploaded in a pattern that is familiar to the ophthalmologist and seen in Fig. 5.1. Images are then montaged into a single image that needs to be read or analyzed in addition by the ROP Tool. The algorithm in the FocusROP program determines whether or not a bedside examination is required or if a repeat photographic examination is required.
The errors that are possible are first, the nurse uploading the images into the program to be sent to the FocusROP website. The FocusROP and montaging software is capable of identifying images that were loaded into the wrong location and correcting that to allow proper montaging. The individual images are also available to the reader since the reader wants them in that fashion. The reader can also manipulate the brightness, contrast, and color hue to maximize reading images. The other most common doctor error is that the examination schedule is made too long and the computer program can be set to a time frame that is unable to be changed and is of an appropriate examination schedule given the findings. These systems, coupled with the use of the ROP Tool to give an indication of plus disease [29, 30], allow a very safe examination technique for retinopathy of prematurity screening.
It would seem that this type of examination is becoming the standard and the interpretations could be done initially by physicians and non-physician readers, information sent perhaps to the neonatologist who could in fact make the diagnosis based on the information given to them in a safe and reliable fashion, reducing the number of ophthalmology examinations that are needed in the NICU. The role of the non-physician reader has been validated by the EROP Study [31].
Retinopathy of prematurity (ROP) management has changed greatly over the last 20 years and continues to evolve. Currently, with appropriate screening that allows appropriate treatment intervention, the failure rates resulting in blindness for retinopathy of prematurity are reduced to a level of 1–2%, with 90% success of laser [10, 32–35], and 90% success of early lens-sparing vitrectomy [36–40]. The key however to this treatment advantage is appropriate screening. As less than 20% of premature infants with the risk of ROP end up with treatment, it is possible that physicians may be encountering disease at an infrequent rate. Therefore, it may be that the detection of disease is reduced by this infrequent nature of the severe manifestations of retinopathy of prematurity. In many areas of medicine, telemedicine and computer-driven programs are being looked at and used for supplying the physician with information that allows them to make a diagnosis. An example is cardiograms are now often analyzed by computer program giving the physician information that they can use to interpret the patient’s cardiogram [41].
It has been known for many years that plus disease is the key change in the fundus which triggers a treatment event [25, 42–44]. A computer program referred to as the “ROP Tool” is a program that gives a number that can be helpful in the determination of plus disease [45]. Plus disease, defined as dilatation and tortuosity in the posterior pole, is something that can be captured, most often quite well, with photographic documentation [29, 46]. The photographs can be analyzed and receive a value based on dilatation and tortuosity. Dilatation is very photographically dependent; whereas, tortuosity can be interpreted by a non-physician reader quite readily [31].
In order to test the hypothesis that the ROP Tool program can match the need for referral-warranted ROP or treatment-warranted ROP, a validation study was designed. Fundus photographs of 335 eyes were read by three experts using the FocusROP software, and then compared to quantitative analysis by the ROP Tool. A scale (0–4) was used by the readers to grade tortuosity. If any photograph was >0, the images were classified as a case. In total, the study included 84 cases and 251 controls. The analysis performed well for identifying tortuosity, but was unable to accurately distinguish dilation in cases from controls. The area under the tortuosity line in a receiver operator curve was 0.918 (95% CI), demonstrating that the ROPtool has a high discriminatory power in differentiating tortuosity between cases and controls (see Fig. 5.1). As a clinical tool, this study demonstrates that the software can provide a sensitive assessment of the need for examination of a child by an ROP expert, potentially reducing cost while delivering quality care.
Virtual Pediatric Retinal Reading Center
The complexity of some forms of pediatric retinal diseases is contributed to by the fact that many of these diseases are rather rare and require a multitude of imaging and diagnostic techniques that are not always easily available to every physician. For that reason, we have established a Pediatric Retinal Reading Center, which can be used to seek advice relative to more complex pediatric retinal issues. These issues include retinopathy of prematurity, pediatric retinal vascular diseases such as familial exudative vitreoretinopathy, persistent fetal vasculature syndrome, Norrie disease, Coats’ disease, and other retinal vascular anomalies. Oncology may also be added to this system allowing lesions such as retinoblastoma, retinal hamartomas, and vascular tumors to be evaluated by a group of expert ophthalmologists. This type of electronic consultation is becoming more and more popular and available in a fee-for-service type of package that generates a usable report that can be shared with patients. The access to the Pediatric Reading Center will be available in the first quarter of 2015. The reading center itself may contain some technologies not available in other locations such as use of the ROPtool as has been discussed previously in this chapter. The ability to montage images can allow further analysis of lesion measurements. It also can integrate imaging techniques such as wide-field fluorescein angiography, color fundus photography, optical coherence tomography, and ultrasound. These image sets in the future may also be integrated with genetic findings as more and more genetic information becomes available relative to pediatric retinal diseases. This type of information, since it is available via the Internet, can be delivered to the doctor in a very time efficient fashion after the physician becomes a registered user of the Pediatric Retinal Reading Center service.
The Pediatric Retinal Reading Center can also be used for national and international randomized prospective controlled clinical trials as images can be analyzed and returned to treatment centers in a very rapid fashion globally. A worldwide distribution of information can be gathered that should allow in-depth analysis of what can be very useful information data sets on some diseases that are rare enough that no single physician may be able to accumulate in a meaningful number of patients. The imaging should allow reliable diagnostic information and in some circumstances, the Pediatric Retinal Reading Center may be able to allow entry of vascular active diseases such as retinopathy of prematurity with a description of activity that assures that equally active eyes are being entered into randomized therapeutic selections.
In all, the Pediatric Retinal Reading Center should bring a service to physicians who deal with pediatric retinal diseases and allow them to not function in isolation, but with the support of several other physicians interested in pediatric retinal disease worldwide.
In summary, ROP screening in the twenty-first century involves the team approach of NICU nursing, physician readers, involvement of parents in the long-term care of their child and use of digital imaging and smart software to quantitate the exam. All of these features supply better patient care, mitigates malpractice issues for physicians and hospitals and brings the screening for ROP truly into the twenty-first century using the Internet, which now is so common to manage our banking and social networking, and can also be used to give us ideal patient screening for retinopathy of prematurity. This paradigm results in better medicine, better documentation, and less expense than currently exists for ROP screening.
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