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ORIGINAL STUDY Clinical Applicability of the International Classification of Disease and Related Health Problems (ICD-9) Glaucoma Staging Codes to Predict Disease Severity in Patients With Open-angle Glaucoma Anjali S. Parekh, MD,* Ali Tafreshi, BS,* Syril K. Dorairaj, MD,w and Robert N. Weinreb, MD* to provide more specific detail with regard to disease Purpose: The aim of the study was to determine how the Interna- diagnosis and severity in a particular patient. The purpose tional Classification of Disease and Related Health Problems of this characterization would be to allow third party (ICD-9) Glaucoma Staging Codes (GSC) relate to visual field payers and insurers to better understand the needs of global indices and average retinal nerve fiber layer (RNFL) thick- a given patient, profile the complexity of a physician’s ness in patients with primary open-angle glaucoma (POAG). practice, and appropriately allocate resources.2 Another Methods: Over a 6-week period, charts of consecutive patients were suggested benefit of this system is the potential to do prospectively reviewed. Included patients had optic nerve head meaningful research based on a more defined and specific damage consistent with glaucoma as well as reliable functional and Medicare database.2 After refinements required by the imaging tests. Patients were divided into early-stage, moderate- government’s ICD-9 Coordination and Maintenance stage, and severe-stage glaucoma using the ICD-9 GSC guidelines. Committee, the GSC were approved for implementation on Relationships between mean deviation (MD), pattern SD (PSD), 3 average RNFL thickness, and the staging system were evaluated. October 1, 2012. Many staging systems have been proposed during the Results: A total of 616 patient charts were evaluated and 135 past 40 years.4 However, patients with glaucoma present patients met the inclusion criteria. Of the 135 patients, both eyes with varying severity along a continuum, and a widely (270 eyes) were evaluated and the worse eye (by MD) was included accepted system of staging has not been adopted.5 Under- in the analysis. Using the ICD-9 GSC guidelines as a basis for standing how this new system relates to clinically diagnosed defining severity, MD was significantly lower in the severe-stage compared with the moderate-stage group (P < 0.01) and in the disease severity is of significant importance, as resources, moderate-stage when compared with the early-stage group reimbursements, and research will likely be influenced by (P = 0.020). PSD was significantly higher in the severe-stage this system. compared with the moderate-stage group (P < 0.01) and in the This study aimed to determine how the ICD-9 GSC moderate-stage compared with the early-stage group (P < 0.01). relate to visual field global indices and average retinal nerve Average RNFL thickness was not significantly lower in the severe- fiber layer (RNFL) thickness, widely used measures of stage compared with the moderate-stage group (P = 0.05); how- functional and structural testing in clinical practice. ever, it was significantly lower in the severe-stage and moderate- stage groups compared with the early-stage group (P < 0.01). Conclusions: ICD-9 GSC showed a significant relationship between METHODS worsening visual field indices and RNFL loss with increasing dis- Subjects ease severity in patients with POAG. This was an observational cross-sectional study that Key Words: glaucoma staging codes, open-angle glaucoma, visual included patients from a glaucoma specialist’s practice at field the Shiley Eye Center (University of California, San Diego). Participants were evaluated with a comprehensive clinical (J Glaucoma 2014;23:e18–e22) examination along with imaging and functional tests. The University of California San Diego Human Subjects Committee approved all the protocols, and methods he overall burden of glaucoma is likely to intensify in described attended to the tenets of the Declaration of Tthe future because of the increasing aging population Helsinki. and numbers of patients at elevated risk for the disease.1 To Over a 6-week period, the charts of consecutive prepare for this possibility, a new glaucoma staging system patients were prospectively reviewed. Each subject’s chart was developed by an American Glaucoma Society work- underwent evaluation by 2 reviewers and the following group. The Glaucoma Staging Codes (GSC) were designed information was compiled and recorded: patient age, race, review of medical history, best-corrected visual acuity, slit- lamp biomicroscopy, intraocular pressure (IOP) using Received for publication August 1, 2013; accepted September 25, 2013. From the *Hamilton Glaucoma Center, University of California, La Goldmann applanation tonometry, gonioscopy, dilated Jolla, CA; and wMayo Clinic, Jacksonville, FL. fundoscopy, stereoscopic optic disc photography, optic Disclosure: The authors declare no conflicts of interest. nerve head optical coherence tomography (OCT), and Reprints: Anjali S. Parekh, MD, Hamilton Glaucoma Center, Uni- standard automated perimetry (SAP). To be included, all versity of California, San Diego, 9500 Gilman Drive, La Jolla, CA 92093 (e-mail: [email protected]). patients had glaucomatous optic neuropathy diagnosed by Copyright r 2013 by Lippincott Williams & Wilkins the presence of neuroretinal rim thinning, excavation, or DOI: 10.1097/IJG.0000000000000033 RNFL defects in addition to reliable functional and e18 | www.glaucomajournal.com J Glaucoma Volume 23, Number 1, January 2014 J Glaucoma Volume 23, Number 1, January 2014 Clinical Applicability of the ICD-9 GSC ICD-9 Staging Definitions Each patient’s visual field was evaluated, and the ICD- 9 GSC3,6 were used to determine disease severity. Two graders evaluated each visual field and staged both eyes of each patient; however, as defined by the system, the worse eye was used to classify each patient’s level of disease and was used for analysis. If a patient had 2 eyes that were equally damaged, the worse of the 2 eyes, based on involvement of fixation, or poorer global indices was marked as the eye for analysis. The worse eye was the only eye included in the analyses. Disease severity was graded as follows3,6: Mild or Early-stage Glaucoma It is defined as optic nerve abnormalities consistent with glaucoma but no visual field abnormalities on any white-on-white visual field test, or abnormalities present only on short–wavelength-doubling perimetry (Fig. 1). Moderate-stage Glaucoma It is defined as optic nerve abnormalities consistent with glaucoma and glaucomatous visual field abnormalities in 1 hemifield, not within 5 degrees of fixation (Fig. 2). Severe-stage Glaucoma It is defined as optic nerve abnormalities consistent with glaucoma and glaucomatous visual field abnormalities in both hemifields, and/or loss within 5 degrees of fixation in at least 1 hemifield (Fig. 3). FIGURE 1. Mild-stage or early-stage glaucoma: no visual field abnormalities on any white-on-white visual field test or abnor- Statistical Analysis 3 malities present only on short–wavelength-doubling perimetry. Continuous variables were compared between the groups using 1-way analysis of variance (ANOVA) with imaging tests as described below. Patients must have undergo visual field testing and OCT imaging within 1 year of the clinical examination. Subjects with coexisting cornea, retinal disease, uveitis, or nonglaucomatous optic disc neuropathy were excluded from the study. Patients underwent SAP with 24-2 Swedish Interactive Threshold Algorithm (Carl Zeiss Meditec Inc., Dublin, CA). Only reliable tests with r33% fixation losses and false negatives and <15% false positives were included. For each test, visual field index, mean deviation (MD), and pattern SD (PSD) were recorded. Visual fields were reviewed to identify the presence of artifacts such as lid and rim artifacts, fatigue effects, inattention, or inappropriate fixation. In addition, they were reviewed for the presence of nonglaucomatous abnormalities such as homonymous hemianopia. RNFL imaging was carried out using Cirrus SD-OCT (software version 4.5; Carl Zeiss Meditec Inc.). Cirrus uses a superluminescent diode scan with a center wavelength of 840 nm and an acquisition rate of 27,000 A-scans per second at an axial resolution of 5 m. The protocol used for RNFL thickness evaluation, the optic disc cube, is based on a 3-dimensional scan of a 6Â6mm2 area centered on the optic disc, where information from a 1024 (depth)Â200Â200-point parallelepiped is collected. Then, a 3.46-mm-diameter circular scan is automatically placed around the optic disc, and the information about para- papillary RNFL thickness is obtained. To be included, all images were reviewed for noncentered scans and had to have signal strength >6, the absence of movement artifacts, FIGURE 2. Moderate-stage glaucoma: visual field abnormalities and good centering around the optic disc. in one hemifield, and not within 5 degrees of fixation.3 r 2013 Lippincott Williams & Wilkins www.glaucomajournal.com | e19 Parekh et al J Glaucoma Volume 23, Number 1, January 2014 FIGURE 3. Severe-stage glaucoma: glaucomatous visual field abnormalities in both hemifields, and/or loss within 5 degrees of fixation in at least one hemifield.3 Tukey honestly significant difference (HSD) test for post There was no significant difference between the groups in hoc analysis. In the event, significant differences between any of the other characteristics (Table 1). the groups were found in ANOVA; analysis of covariance MD was significantly lower in the severe-stage glau- (ANCOVA) was applied to adjust for age, central corneal coma group compared with the moderate-stage group thickness (CCT), and race. Linear regression analysis was (P < 0.01) and the moderate-stage compared with the used to evaluate the relationship between RNFL thickness early-stage group (P = 0.020; ANOVA with Tukey HSD and SAP indices MD and PSD across groups; R2 and the test). PSD was significantly higher in the severe-stage corresponding P-values were calculated for each parameter. glaucoma group compared with the moderate-stage glau- Statistical analyses were made using JMP software version coma group (P < 0.01) and in the moderate-stage group 10.0.0 (SAS Institute, Cary, NC).