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Diagnosis and Grading of Papilledema in Patients With

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Diagnosis and Grading of Papilledema in Patients With CLINICAL SCIENCES Diagnosis and Grading of Papilledema in Patients With Raised Intracranial Pressure Using Optical Coherence Tomography vs Clinical Expert Assessment Using a Clinical Staging Scale Colin J. Scott, MD; Randy H. Kardon, MD, PhD; Andrew G. Lee, MD; Lars Frise´n, MD, PhD; Michael Wall, MD Objectives: To compare and contrast 2 methods of quan- tographs correlated well (R=0.85). OCT total retinal thick- titating papilledema, namely, optical coherence tomog- ness and MFS grade from photographs had a similar cor- raphy (OCT) and Modified Frise´n Scale (MFS). relation of 0.87. Comparing OCT RNFL thickness with OCT total retinal thickness, a slope of 1.64 suggests a Methods: Digital optic disc photographs and OCT fast greater degree of papilledema thickness change when retinal nerve fiber layer (RNFL) thickness, fast RNFL map, using the latter. total retinal thickness, and fast disc images were ob- tained in 36 patients with papilledema. Digital optic disc Conclusions: For lower-grade abnormalities, OCT com- photographs were randomized and graded by 4 masked pares favorably with clinical staging of optic nerve pho- expert reviewers using the MFS. We performed Spear- tographs. With higher grades, OCT RNFL thickness pro- man rank correlations of OCT RNFL thickness, OCT total cessing algorithms often fail, with OCT total retinal retinal thickness, and MFS grade from photographs. thickness performing more favorably. Results: OCT RNFL thickness and MFS grade from pho- Arch Ophthalmol. 2010;128(6):705-711 APILLEDEMA, OR OPTIC DISC patient fixation stability during imaging swelling due to raised intra- without eye tracking and the failure of seg- cranial pressure, has been mentation algorithms for defining RNFL graded using the Frise´n thickness and total retinal thickness in eyes Scale.1 This scale uses visual with severe papilledema. Pfeatures of the optic disc and peripapil- Although fundus photographs require lary retina to stage optic disc edema. Its subjective interpretation, they show de- reproducibility has been validated,1 but it tailed topographic relationships of optic is limited by use of an ordinal scale. disc edema that may be difficult to quan- Optical coherence tomography (OCT) tify and document on clinical examina- is a potential tool to quantify changes in the tion. Unlike OCT, photographic artifacts Author Affiliations: degree of papilledema and to monitor the (eg, defocus or incorrect light exposure) Department of Ophthalmology efficacy of treatment interventions. First de- that could interfere with interpretation are and Visual Sciences, Carver scribed in 1991 by Huang et al,2 OCT is a usually obvious. School of Medicine, University cross-sectional imaging technique that OCT is useful in evaluating various oph- of Iowa, and Veterans Affairs Hospital, Iowa City (Drs Scott, quantitatively assesses multiple layers of the thalmic disorders such as band atrophy, Kardon, Lee, and Wall); and retina, allowing measurement of the reti- glaucoma, diabetic retinopathy, cystoid Institute of Neuroscience and nal nerve fiber layer (RNFL) with a reso- macular edema, central serous retinopa- Physiology, Department of lution of approximately 10 µm using time- thy, macular hole formation, optic nerve Clinical Neurosciences, domain variables.3-5 Direct measurements head pits, and ischemic optic neuropa- Sahlgrenska Academy, are calculated by a computer algorithm to thy.12-18 Most of these conditions involve University of Gothenburg, quantify the nerve fiber layer and total reti- RNFL thinning. Few studies evaluated the Gothenburg, Sweden nal thickness. OCT offers several advan- use of OCT in measuring RNFL or total reti- (Dr Frise´n). Dr Lee is now with tages over conventional photographic nal thickening due to papilledema. A 2007 the Department of imaging such as use with small pupil sizes study19 compared the findings of OCT vs Ophthalmology, The Methodist Hospital and the Departments and nuclear cataracts. OCT findings are rea- disc photographs in children with idio- of Ophthalmology, Neurology, sonably reproducible on successive mea- pathic intracranial hypertension. Another 20 and Neurosurgery, Weill Cornell surements in normal eyes and in eyes with study assessed the importance of RNFL Medical College New York, glaucoma.6-11 Despite these advantages, limi- thickness analysis in intracranial hyperten- New York. tations of OCT include the requirement for sion using imaging laser polarimetry. In pa- (REPRINTED) ARCH OPHTHALMOL / VOL 128 (NO. 6), JUNE 2010 WWW.ARCHOPHTHALMOL.COM 705 ©2010 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 10/04/2021 dopapilledema but did not directly compare OCT vs fundus photograph grades. To our knowledge, this is the first study to compare OCT RNFL thickness and OCT total retinal thickness with fundus disc photographs in adults having raised intracranial pressure. Our objectives were to com- pare and contrast OCT and Modified Frise´n Scale (MFS) grading with the peripapillary RNFL in quantifying optic disc edema. METHODS Digital optic disc photographs of the right or left eye were se- lected for review if corresponding OCT images of the RNFL were Grade 0 Grade 1 available and if photographs and OCT images were of sufficient quality. For OCT analysis, the circular image had to be cen- tered on the nerve, the signal strength had to be at least 5 (range, 1[lowest]-10 [highest]), and the proprietary algorithm used by the manufacturer (OCT3; Zeiss Meditech, Dublin, California) had to successfully delineate the borders of the RNFL on manual inspection of the images. The proprietary algorithm creates a best- fit estimate of the inner and outer borders of the RNFL and the total retinal thickness to calculate the mean thickness, as dem- onstrated by the overlayed white lines of the B-scan in Figure 1. Algorithm failure occurred when the borders of the retinal lay- ers delineated by the software were qualitatively incompatible with the borders inferred by experts (C.J.S. and R.H.K.). For fun- dus photographs, the optic nerve had to be in focus to allow evalu- ation of the RNFL at the disc border. Analysis was monocular rather than binocular because of some poor photographic qual- ity or failure to capture the entire extent of the z-axis by OCT Grade 2 Grade 3 due to peripapillary elevation and cropping of the B-scan in 1 of 2 eyes. Furthermore, use of both eyes would violate a principle of parametric statistics, as the results of one eye are not inde- pendent of the other eye. Although the relationship between eyes could be accounted for with nested analysis of variance, using both eyes in some patients would have complicated our statis- tical analysis. Patients retrospectively identified as having raised intracranial pressure at some point in their clinical course from January 2004 to March 2008 were included in the study, and the imaging studies chosen were from the patient’s first presenta- tion to our clinic for evaluation. While most cases were idio- pathic, the specific etiology was not addressed, as the pathogen- esis of disc edema pathogenesis of papilledema was secondary to raised intercranial pressure in all cases is static and should not affect comparison of disc appearance with OCT values. No other cause of visual loss or optic disc edema was present such as op- tic nerve compression, inflammatory optic neuropathy, or is- Grade 4 Grade 5 chemic optic neuropathy. Monocular digital photographs were randomized and graded by 4 masked expert reviewer neuro- Figure 1. Representative fundus photographs of each Modified Frise´n Scale ophthalmologists (3 from the University of Iowa and 1 from the grade as summarized in Table 1. A representative optical coherence University of Gothenburg, Sweden). Photographs were viewed tomography image is below each disc grade. Overlayed white lines of the ϫ optical coherence tomography image represent borders of the retinal nerve at 1600 1200 resolution (SyncMaster 213T; Samsung, Ridge- fiber layer thickness (upper and middle lines) and the total retinal thickness field Park, New Jersey). (upper and lower lines) estimated using the algorithm (OCT3; Zeiss We used an adaptation of a clinical staging scale initially de- Meditech, Dublin, California). Modified Frise´n Scale key features (Table 1) are scribed by one of us (L.F.) (Table 1 and Figure 1).1 The first 2 well demonstrated. grades are primarily dependent on changes surrounding the op- tic disc such as decreased translucency and opacification of the tients with retinal vein occlusions and inflammatory op- peripapillary nerve fiber layer (the term blurring is avoided be- tic neuropathies, Menke et al21 found that OCT RNFL cause it has many meanings, including lack of image focus). Grade thickness was increased compared with that in control sub- 0 represents no optic disc edema, and grades 1 through 5 rep- 22 resent increasing severity of optic disc edema. We modified the jects. Hoye et al used OCT to demonstrate subretinal Frise´n Scale by identifying a key feature for each grade, with the macular edema in papilledema, and OCT has been used other criteria considered minor. For grade 1, the key feature is to measure RNFL thickness in patients with optic neuri- the presence of a C-shaped halo of opacification of the RNFL at tis.23,24 Other studies25-27 compared OCT (as well as auto- the region of the optic disc border; a gap remained at the loca- mated perimetry) RNFL thickness in papilledema and pseu- tion of the maculopapular bundle. Rarely, a thin smooth-edged, (REPRINTED) ARCH OPHTHALMOL / VOL 128 (NO. 6), JUNE 2010 WWW.ARCHOPHTHALMOL.COM 706 ©2010 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 10/04/2021 ring-shaped peripapillary light reflex may be present in normal anatomy; when present, it is a continuous reflex. Therefore, patho- Table 1. Modified Frise´n Scale logic designation requires irregularity and breakup of any ring- shaped peripapillary reflex (grade 1 in Figure 1).
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