Woman presents with peripapillary hemorrhages
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A 76-year-old woman was referred from optometry for glaucoma evaluation after bilateral optic nerve hemorrhages were found on a routine eye exam. The patient’s ocular history included mild hyperopia and mild dry macular degeneration.
Five years prior, the patient saw a neuro-ophthalmologist for subjective visual disturbances that included 5 seconds of “whiteout” of her bilateral central visual field while exercising. On exam at that time, she was found to have an acute posterior vitreous detachment (PVD) in the right eye, as well as nasal and superior optic nerve hemorrhages. OCT of the retinal nerve fiber layer (RNFL), Humphrey visual field (HVF) and inflammatory markers were within normal limits. In the setting of an acute PVD and the less typical nasal location of the larger hemorrhage, it was felt that this was a possible hemorrhagic PVD. The patient was lost to ophthalmology follow-up since that visit. Her medical history consisted of osteoporosis, anxiety and depression.
Examination
On examination, the patient’s best corrected visual acuity was 20/25-2 in the right eye and 20/25 in the left eye with mild hyperopic correction. Her pupils were 6 mm in scotopic conditions and constricted to 4 mm in photopic conditions without relative afferent pupillary defect in either eye. IOPs by Goldmann applanation were 16 mm Hg and 17 mm Hg in the right and left eyes, respectively, and extraocular movements were full. On gonioscopy, the angle structures were not visible, and the irises had a steep approach; the angle opened to the scleral spur with dynamic gonioscopy, and the trabecular meshwork had mild to moderate pigmentation. Pachymetry values were 578 µm on the right and 576 µm on the left. There were moderate nuclear sclerotic and cortical cataracts in both eyes and no transillumination defects on retroillumination of the irises. Optic nerve examination revealed a moderate cup with a thin superior rim and a flame-shaped hemorrhage superotemporally in the right eye (Figure 1a) and a moderate cup with a thin superior rim and another flame-shaped hemorrhage superotemporally in the left eye (Figure 1b). There was no optic nerve edema.
Source: Chelsea Gottschalk, MD, and Astrid C. Werner, MD
Imaging
As part of the glaucoma evaluation, the patient underwent OCT RNFL as well as HVF testing of both eyes. The OCT-RNFL demonstrated superior and inferior thinning of the right optic nerve and superior thinning of the left optic nerve (Figure 2a). On OCT ganglion cell complex (GCC) testing, there was thinning bilaterally (Figure 2b). HVF 24-2 testing revealed an inferior paracentral scotoma and superior arcuate scotoma in the right eye (Figure 3a) and an inferior Bjerrum scotoma in the left eye (Figure 3b).
Imaging from 5 years before presentation demonstrated a normal OCT RNFL and diffuse GCC thinning of unclear significance (Figures 4a and 4b). A fundus photo from the prior visit demonstrated the previous optic nerve hemorrhage (Figure 5).
What is your diagnosis?
See answer below.
Optic nerve hemorrhages
The constellation of findings that includes bilateral optic nerve hemorrhages, superior and inferior optic nerve thinning on OCT-RNFL with visual field defects that respect the horizontal midline, as well as normal IOPs points to normal-tension glaucoma as the likely diagnosis; however, angle-closure glaucoma cannot be ruled out as the gonioscopy did demonstrate occludable angles.
Because there were no peripheral anterior synechiae on gonioscopy, the patient denied subacute angle closure symptoms and there were optic nerve hemorrhages (which are more classically associated with normal-tension glaucoma), normal-tension glaucoma (NTG) was determined to be the primary diagnosis rather than an acute or chronic angle-closure mechanism. The photos and imaging from 5 years prior support this diagnosis, as there was another documented optic nerve hemorrhage in the right eye; however, glaucoma testing at this time was essentially normal. While the prior optic nerve hemorrhage was thought to be from a hemorrhagic posterior vitreous detachment, taken in context with the current testing, it is more likely that this optic nerve hemorrhage represented early glaucomatous change. Optic nerve hemorrhages in glaucoma, known as Drance hemorrhages, classically affect the superotemporal and inferotemporal quadrants, making a nasal hemorrhage misleading in this patient.
Workup and management
The patient was asked about risk factors for glaucoma and normal-tension glaucoma specifically and stated that she had been hit in the left eye 30 years prior, denied any steroid use, and denied a history of diabetes, migraine, nighttime antihypertensive use, sleeping on her arm and sleep apnea. She did endorse a history of Raynaud’s phenomenon and stated that her blood pressure measurements tended to run in the low 100s mm Hg to 120s mm Hg systolic.
As for management, a prophylactic laser peripheral iridotomy was recommended to the patient given the narrow angles and presence of glaucomatous optic neuropathy. However, the patient opted to observe. Topical IOP-lowering medication was recommended, and she was started on latanoprost in both eyes. Four weeks after the initiation of latanoprost, IOPs were measured as 11 mm Hg and 12 mm Hg in the right and left eyes, respectively, a 30% reduction. The patient will be monitored closely with serial examinations, OCT RNFL and HVF tests. She was also recommended to keep a blood pressure log to screen for nocturnal hypotension and to be screened for anemia.
Discussion
The differential diagnosis of an optic nerve hemorrhage includes glaucoma, hemorrhagic PVD, hypertension, diabetes, vein occlusion and ischemic optic neuropathy. An isolated disc hemorrhage should trigger a glaucoma workup as the presence of a Drance hemorrhage indicates a higher risk for glaucomatous progression and RNFL loss, independent of whether the IOP is elevated or normal. As in this case, a diagnosis of glaucoma may not be clear when an initial disc hemorrhage is identified because glaucoma testing may be normal initially. It is recommended that these patients undergo serial exams with OCT RNFL and HVF to ensure that any glaucomatous changes will be identified
In patients with NTG, visual field deficits tend to be closer to fixation and more likely to affect the patient’s functional status earlier in the disease, further highlighting the need to follow these patients closely. While the Collaborative Normal Tension Glaucoma Study demonstrated that only 35% of normal-tension eyes will progress, risk factors for progression include optic nerve hemorrhage, migraine and female gender; therefore, it was imperative to start IOP-lowering therapy to hopefully slow the progression of disease. NTG is theorized to involve decreased perfusion of the optic nerve head, and so it is also important to screen for associated systemic conditions such as sleep apnea, anemia, vasospastic disorders and nocturnal hypotension as part of the treatment algorithm so that these conditions can be addressed and treated in an effort to slow down glaucomatous change.
- References:
- Collaborative Normal-Tension Glaucoma Study Group. Am J Ophthalmol. 1998;doi:10.1016/s0002-9394(98)00223-2.
- Ganeshrao SB, et al. Invest Ophthalmol Vis Sci. 2019;doi:10.1167/iovs.18-25421.
- Gracitelli CP, et al. PLoS One. 2014;doi:10.1371/journal.pone.0105611.
- Lee J, et al. Ophthalmology. 2019;doi:10.1016/j.ophtha.2018.12.031.
- Song BJ, et al. Indian J Ophthalmol. 2014;doi:10.4103/0301-4738.133481.
- Trivli A, et al. Exp Ther Med. 2019;doi:10.3892/etm.2018.7011.
- For more information:
- Chelsea Gottschalk, MD, and Astrid C. Werner, MD, can be reached at New England Eye Center, Tufts University School of Medicine, 800 Washington St., Box 450, Boston, MA 02111; website: www.neec.com.
- Edited by Allison V. Coombs, DO, MS, and Nisha S. Dhawlikar, MD, MPH. They can be reached at New England Eye Center, Tufts University School of Medicine, 800 Washington St., Box 450, Boston, MA 02111; website: www.neec.com.