Published in Glaucoma

Glaucoma Masqueraders: Don't Get Caught By Surprise

This post is sponsored by Carl Zeiss Meditec Professional Education
14 min read

In this course, you will learn which ocular conditions commonly masquerade as glaucoma and how to identify glaucoma masqueraders in practice by utilizing different multimodal ophthalmic imaging.

Glaucoma Masqueraders: Don't Get Caught By Surprise
TABLE OF CONTENTS
WHAT YOU'LL LEARN
Which ocular conditions commonly masquerade as glaucoma
How to identify glaucoma masqueraders in practice

Introducion

Introduction

It is important to see the bigger picture when evaluating and diagnosing a patient as there are many ocular conditions that can masquerade as glaucoma. By evaluating a comprehensive medical history, case history, and utilizing different multimodal ophthalmic imaging, we will be better equipped to diagnose and manage our patients accordingly. This article will walk through different cases to construct a thought process with which to tackle these diseases.

Case 1

Case 1

  • 57 yo BF referred by PCP for glaucoma evaluation.
  • POHx: Latanoprost qhs OU, poor compliance. Pt self discontinued since “she did not feel it was helping”.
  • MHx: HIV (+), unknown viral load
  • BCVA: 20/40 OD/OS
  • Pupils: PERRL (+) Grade 2 RAPD OS
  • IOP: 19/20
  • PACHY OD: 530, OS: 534
  • Gonioscopy: Open (-) neovascularization of the angle, angle recession, peripheral anterior synechiae
  • Additional findings: Anterior Uveitis OS; asymptomatic. Tritan color vision defect, OS
Anterior Uveitis
Figure 1.0: Fundus photos (blur due to cataract) and pachymetry (OD 533um, OS 534um)
Ganglion Cell Analysis
Figure 1.1: Left: OCT RNFL - notice the asymmetry between the RNFL layer of the eyes. Right: Ganglion Cell Analysis - OS shows asymmetry between the superior and inferior portion, respecting the horizontal raphe.
Ganglion Cell Loss
Figure 1.2: Top: 30-2 SITA, OS - dense inferior arcuate defect, and superior nasal step. Bottom: 10-2 SITA, OS - corresponds well with the ganglion cell loss as seen in Figure 1.2. Note: OD visual field not shown, but full without glaucomatous defects.
Conclusion: Still in the gray area, and glaucoma should be put on the differential list. The patient has some outlying data: asymmetry and APD that warrant a closer look at imaging.
Distinct Pallor Temporal
Figure 1.3 Left: Fundus photography, OS. Right: Red-free fundus photography OS - distinct pallor temporal.

Case 1 Discussion

The patient was diagnosed with neurosyphilis. Remember that if you have a puzzling diagnosis that does not look quite right, consider blood work, as well as, neurosyphilis. The ocular manifestations of neurosyphilis are quite diverse:
  • Interstitial keratitis
  • Episcleritis
  • Papillary conjunctivitis
  • Granulomatous or non-granulomatous uveitis
  • Chorioretinitis
  • Retinitis
  • Vitritis
  • Multifocal choroiditis
  • Neuro-retinitis
  • Papilledema
  • Optic atrophy
  • Ocular hypertension
  • Argyll-Robertson pupil
  • Cranial nerve palsies

Case 2

Case 2

  • 52 yo WM
  • BCVA: 20/25 OD, 20/20 OS
  • Pupils: PERRL (-) RAPD
  • IOP: 18/19, Pachymetry: 560/553
  • MHx: unremarkable, + smoking (cigarettes)
OCT RNFL and Ganglion Cell Analysis
Figure 2.0: OCT RNFL and ganglion cell analysis. Notice the asymmetry in the RNFL thickness from OD to OS.
Absolute Defect Anterior Nasal
Figure 2.1: VF 24-2 absolute defect inferior nasal, OD. Recall that glaucoma starts as a relative defect.
Resolution of BRVO
Figure 2.2: Before and after resolution of BRVO.

Case 2 Discussion

This patient had a branch retinal vein occlusion (BRVO) of the right eye. The occlusion starts very close to the optic nerve head (Figure 2.2), and branches off to the peripheral retina with damage to capillary beds as seen by the deeper hemorrhages. Due to the size and extent of damage, there’s been a lot of retinal ischemia.
Cases like this one can be glaucoma masqueraders when the patient walks into your office after resolution of the condition. You want to be able to pick up a history of retinal vein occlusion so the patient doesn’t get misdiagnosed with a different condition. With careful analysis of the fundus photo post-resolution, you can see the arterials were attenuated. A fundus autofluorescence image (Figure 2.3) also makes the tiny collateral vessels pop out. It is important to use different imaging techniques to assess the overall picture, and to evaluate from quadrant to quadrant to pick up on subtle signs.
Fundus autofluorescence
Figure 2.3: Fundus autofluorescence, OD. Cursor is pointed at collateral vessels.
Swelling of the Retina
Figure 2.4: OCT angiography, OD. Top left: 3 day presentation after RVO. The black area denotes swelling of the retina. You can see collateralization near the macula to drain and perfuse the retina. And identify that there are at least 5 disc diameters of capillary dropout (clinical sign of BRVO). Top middle: 2 months after the initial presentation. Note the capillary dropout. Top right: Along the superior arcade. Although there is no capillary bed there, notice the microvascular changes.
Recall that the average patient with vein occlusions is over 50 years of age. BRVO does not come from the same etiology as a CRVO or an HRVO. BRVOs are primarily ischemic and retinal. CRVO will affect the optic disc, iris, and angle, and will present with different sizes of capillary non-perfusion. This table will help outline the difference between the three types:
Capillary Nonperfusion Table
This patient was diagnosed with ischemic BRVO with macular edema. The patient was young at 52 years of age. If a patient doesn’t fit the norm for vein occlusion (older, hypertensive, diabetic, hyperlipidemia), make sure to think of differential diagnosis. Blood work is important to order to rule out an infectious nature, and throw syphilis as mentioned in case #1 as a possible outlier.
In the last couple of years, literature advises to check homocysteine levels. Increased homocysteine promotes arteriosclerosis which leads to thrombosis. The etiology of vein occlusions is thrombosis. Patients with elevated homocysteine levels have a higher change of cerebral vascular problems, peripheral vascular disease, and 5x increased risk of stroke. This patient’s blood work showed normal results overall, besides homocysteine and cholesterol levels being elevated. If these levels are caught early, they can be easily treated with topical supplementation. For vein occlusions, it is important to evaluate for homocysteine levels in blood work.
This patient was managed with oral folic acid to normalize his homocysteine levels. He was also treated with 2 avastin injections over a three month period.

Case 3

Case 3

  • 52 yo, BM comes in for a second opinion.
  • POHx: Glaucoma (dx 2016)
  • Medications: Latanoprost qhs OU, Cosopt bid OS, Brimonidine 0.15% tid OU
  • Surgery: s/p laser procedure OS, 1 yr ago
  • Tmax: unknown
  • PMHx: situs inversus
  • FOHx: glaucoma - brother
  • BCVA: OD 20/20, OS 20/25
  • Pupils: PERRLA, + RAPD OS grade 3
  • IOP: 12 mmHg OU
  • Pachymetry: 494 OD, 490 OS
OCT RNFL
Figure 3.1: OCT RNFL - fairly symmetric RNFL within 10um. The temporal quadrants on each eye are flagged, but fairly symmetric. One of the ways to pick up whether a patient has situs inversus is to analyze their RNFL thickness. Note that the two peaks move a little nasal.
Ganglion Cell Analysis 2
Figure 3.2: GCC analysis. There is a large difference between the eyes. Before thinking that the patient has such a severe dropout in OS, keep in mind the limitations of the scan. A ganglion cell analysis is looking at the internal limiting membrane and ganglion cells. The ILM is 50um, so if you notice numbers that fall below this value, it means that the segmentation is wrong. Note also the difference between the outer retinal layer of OD vs OS.
Visual Field Reports
Figure 3.3: Visual field reports. A 24-2 and 10-2 were performed on OD due to a central depressed point. However, the fields were unreliable for both eyes.

Case 3 Discussion

This is not glaucoma due to many factors. There are abnormalities in different areas that do not coincide with glaucomatous damage. The patient is on 4 different medications, and has had some kind of retinal procedure. It is possible to start over with this patient’s medical treatment and see if changes occur due to glaucoma. If not, it opens the door to other options. We can go back over different scans done with this patient and dive a little deeper.
Fundus photo and OCT macula
Figure 3.4: Fundus photo and OCT macula, OS. Note the outer pigment epithelial detachments and atrophy. (full scan of the OCT macula indicates more PED’s) .
Tilt of the Optic Nerve
Figure 3.5: Fundus photo, OD. Note tilt of the optic nerve, and inferior vessels coming out on the nasal side.
Fundus Photo Damaged Macula
Figure 3.6: Fundus photo, OS. With fundus autofluorescence, the extent of damage on the macula is visibly highlighted.
This patient has chronic central serous chorioretinopathy. This correlates with the PED’s seen in the OCT macula. At some point in his life, he had large serous detachments. The descending drip occurs because gravity pulls the fluid down. The filtered image will show stressed areas as hyperfluorescence, and the darker spots as areas where the RPE is no longer functioning (Figure 3.6).
This patient still needs to be evaluated for glaucoma vs ocular hypertension. You can do a medication holiday and reevaluate periodically. This particular patient’s IOP went back up to 28 and 24, which is great to establish Tmax. Instead of having him on three different drops, we can start with one and periodically monitor pressure elevations.
OCT Slice
Figure 3.7: OCT slice, OS showing variation over time. Note how the disease changes with the PEDs even though there is no active serous detachment.
Advanced RPE Analysis
Figure 3.8: Advanced RPE analysis, OS. On the right image, the orange pigmented spot denotes the PED. The outlined spots in yellow indicate subretinal or choroidal illumination - areas of geographical atrophy. Recall that the patient had laser treatment in his OS, which points towards retinal laser instead of SLT that may have been thought of on initial discussion. This scan is great for AMD patients to monitor for changes in disease over time.
Central serous chorioretinopathy, “sick RPE syndrome”, is associated with increased permeability of the choroid resulting in PED and serous retinal detachments. This condition is mostly seen in Type A young males. OCT will show an increased choroidal thickness (normal average is 270um vs 405um seen in CSR).

Case 4

Case 4

  • 52 yo, AF, diagnosed with POAG, moderate stage, OU for 14 years
  • Medications: Cosopt bid OU, Brimonidine bid OD
  • Tmax: 26, 24
  • Pachymetry: 528 OD, 519 OS
  • POHx: degenerative myopia OU, lattice degeneration OU, pigmented atrophic hole OD
  • MHx: unremarkable
Note: there is a higher risk of glaucoma in degenerative myopic patients. It is also harder to examine their optic nerves and cupping because of the tilts.
Oblique Tilted Insertion and PPA
Figure 4.1: Optic nerve photos. Note the oblique/tilted insertion, and PPA. The cups look fairly symmetric.
Visual Fields Showing Defect
Figure 4.2: Visual fields. Top left: superior defect in OD (not as dense compared to the BRVO in Case 2). Bottom: 10-2 also indicates a superior defect., which classifies it as a severe stage. Right: OS, peripheral visual field defect inferior and superior nasal.
OCT and Ganglion Cell Analysis
Figure 4.3: OCT and ganglion cell analysis. There is symmetry in reduction of the RNFL thickness OU that points more towards glaucoma. There is asymmetry in cupping as seen on the RNFL thickness map, but remember that the insertion is not the same for this degenerative myope.
Note: The normal database for OCT scans goes up to -6.00D. This patient has a refractive error of -10.00D. So the red indicators just means that the patient does not fit the normative database.
Nerve Fiber Layer Analysis
Figure 4.4: Comparative scans over a ten year span. The top images are nerve fiber layer thickness, and bottom shows the ganglion cell analysis.

Case 4 Discussion

Figure 4.4 shows lack of change over the course of 10 years. This patient did have elevated eye pressure, but it could just be ocular hypertension. Let’s analyze her defects a little further:
OD with Radial Raster
Figure 4.5: Fundus autofluorescence, OD with radial raster. Note the large cavitation right below the optic nerve head.
Superior and Inferior
Figure 4.6: Fundus autofluorescence, OS. There is a superior and inferior cavitation present.
This condition is called peripapillary choroidal cavitation. Looking back at figure 4.3, the right eye tomogram in the middle picks up the cavitation. The OCT images also show a vast inferior thinning of the right eye corresponding with the large cavitation. In the left eye, there were also signs of superior and inferior nasal steps that correspond with the smaller cavitations (figure 4.2).
Peripapillary choroidal cavitation is generally found in high myopes (average -9.00D, 27.36mm axial length). In 60% of cases, there will be a yellow-orange peripapillary lesion. About 70% of patients present with glaucomatous appearing visual field losses. Glaucoma is a progressive optic neuropathy. Cavitations are not. It is important to monitor these patients and see if there is any progression in her IOP, visual field, OCT scans etc.

Conclusion

Conclusion

There are countless glaucoma masqueraders, but with advanced imaging and diagnostics many of these mimics can be distinguished and diagnosed accordingly. It’s absolutely crucial to supplement diagnostic imaging with comprehensive patient history for accurate diagnosis and appropriate treatment—key elements in delivering excellent patient care.
Patricia Fulmer, OD, FAAO
About Patricia Fulmer, OD, FAAO

Dr. Fulmer is the sole owner of Legacy Vision Center, a cold start, medically focused practice in Huntsville, AL, which launched at the end of 2020. A graduate of the UAB School of Optometry, she completed an ocular disease and primary care residency at the Thomas E. Creek VA Medical Center before returning to Alabama to serve as the Center Director and consultative optometrist for a referral-based, tertiary care facility and then as an associate at a multi-doctor family practice. Her clinical interests include disease management, particularly glaucoma, uveitis, dry eye, and neurology-related cases, as well as educating students and colleagues through lecturing and writing. She is an active member in the American Academy of Optometry and American Optometric Association and currently serves her profession as a member of the Board of Directors of the Alabama Optometric Association and as President of the North Alabama Optometric Association. In her free time, Dr. Fulmer enjoys spending time with her husband and family, spoiling their fur-babies, traveling, attending concerts, and exploring the outdoors while kayaking and hiking. She also loves giving back through volunteering, including serving on the Board for HEALS, Inc, an organization that focuses on providing healthcare to local in-need youth.

Patricia Fulmer, OD, FAAO
Jennifer Gould, OD, MS, FAAO
About Jennifer Gould, OD, MS, FAAO

Dr. Jennifer Gould-Blackmore earned both her Doctor of Optometry and Masters of Vision Science degrees from SUNY Optometry in 2013. She completed an ocular disease residency at East New York Diagnostic and Treatment Center. Clinical areas of interest include glaucoma, retina, and anterior and posterior segment disease. She was formerly an Associate Clinical Professor at SUNY College of Optometry and the Chief of Advanced Care Services for the University Eye Center. Dr. Gould-Blackmore is a fellow of the American Academy of Optometry, a diplomate of the American Board of Optometry, and a board member for the Optometric Glaucoma Society.

Jennifer Gould, OD, MS, FAAO
Huda Minhas, OD
About Huda Minhas, OD

Dr. Huda Minhas received a bilingual Doctorate of Optometry at the Inter-American University of Puerto Rico, with an honors undergraduate degree at the University of Toronto. She currently practices in Sacramento at an MD-OD clinic that specializes in cataract surgery, ocular diseases, and secondary/tertiary care. Her philosophy is that healthy eyes are important to one's overall health, wellness, and well-being. In her spare time, Dr Minhas enjoys weightlifting, fine cuisine, and hiking.

Huda Minhas, OD
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