Patient with diabetes presents with progressive blurred vision in right eye
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A middle-aged man with type 2 diabetes and no ocular history presented urgently to the New England Eye Center retina department with progressive blurred vision in his right eye.
The patient endorsed intermittent blurred vision in the past only when his blood sugar was elevated, but this would spontaneously resolve. Given the recent persistence of blurred vision in his right eye, he presented to the retina clinic for further evaluation. He denied eye pain, photosensitivity, photopsia, floaters or visual field defects. Medical history included a diagnosis of type 2 diabetes for the past 15 years, hypertension and hyperlipidemia for which the patient was prescribed metformin, insulin and simvastatin. He endorsed nonadherence with his medications, with his most recent HbA1c measuring 8%. His ocular history was positive only for mild myopia for which he wore glasses. He reported that his last routine dilated eye examination 1 year before presentation was unremarkable. He had no surgical history.
Examination
Best corrected visual acuity was 20/50 in the right eye and 20/20-2 in the left eye. IOPs were normal at 16 mm Hg in each eye. Pupils were equal, round and briskly reactive without a relative afferent pupillary defect. Extraocular movements and confrontation visual fields were normal. Color vision via HRR standard pseudoisochromatic test was four out of four in both eyes.
Anterior segment examination was notable only for 1+ nuclear sclerotic cataracts in both eyes. Dilated fundus examination of the right eye revealed edema of the optic nerve head with neovascularization of the optic disc and peripapillary flame-shaped intraretinal hemorrhages. Macular examination was notable for multiple lipid exudates, microaneurysms and dot-blot retinal hemorrhages. Peripheral examination noted diffuse dot-blot retinal hemorrhages and microaneurysms. In the left eye, dilated fundus examination demonstrated a cup-to-disc ratio of 0.2 with distinct disc margins. Macular examination also noted microaneurysms and dot-blot retinal hemorrhages. Peripheral examination noted diffuse dot-blot retinal hemorrhages, microaneurysms and neovascularization elsewhere along the vascular arcades (Figure 1).
Source: Allison Resnik, MD, and Caroline Baumal, MD
What is your diagnosis?
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Unilateral blurred vision
This patient presented with unilateral optic nerve swelling associated with peripapillary hemorrhages, neovascularization of the disc, peripheral superficial dot-blot hemorrhages, drusen and microaneurysms. The differential diagnosis for a patient with this presentation includes optic neuritis, diabetic papillopathy, hypertensive retinopathy, anterior ischemic optic neuropathy, optic disc drusen, compressive optic neuropathy, inflammatory etiology (demyelination, sarcoidosis) or infectious optic disc vasculitis (cat-scratch disease, syphilis). We were able to further narrow down our differential based on examination findings and the patient’s medical history. Given no history of autoimmune disease and no infectious symptoms, an autoimmune or infectious etiology was lower on the differential. The presence of unilateral optic nerve swelling with signs of diabetic retinopathy and a history of uncontrolled type 2 diabetes placed diabetic papillopathy highest on our differential.
Workup and management
OCT demonstrated intraretinal fluid within 500 µm of the center of the fovea, consistent with center-involving diabetic macular edema (Figure 2a). No subretinal or intraretinal fluid was noted in the left eye (Figure 2b). Fluorescein angiography was performed demonstrating fluorescein leakage at the optic disc and significant patchy areas of hypofluorescence in the periphery corresponding to peripheral retinal nonperfusion of the right eye (Figure 3a). The left eye demonstrated areas of fluorescein leakage along the superior and inferior vascular arcades, indicating retinal neovascularization with significant peripheral nonperfusion (Figure 3b).
Given the findings of diffuse retinal hemorrhages with macular edema and retinal neovascularization, a diagnosis of proliferative diabetic retinopathy in both eyes was made. These findings with unilateral optic nerve head swelling and a known history of type 2 diabetes made diabetic papillopathy the working diagnosis. The patient was referred to neuro-ophthalmology to rule out other etiologies for unilateral optic nerve head swelling. Humphrey visual fields 30-2 measured via automated perimetry demonstrated no visual field defects in either eye. Neuro-ophthalmology evaluation concluded that the patient’s unilateral optic disc swelling was secondary to diabetic papillopathy.
Discussion
Diabetic papillopathy, also known as diabetic papillitis, is a rare entity found in patients with either type 1 or type 2 diabetes with or without diabetic retinopathy. It was initially described in 1971 and thought to affect only young patients with type 1 diabetes. However, over the years, this disease process has been identified in patients of all ages with either type 1 or type 2 diabetes. This disease process is characterized by unilateral optic nerve edema with no signs of significant optic nerve dysfunction. Additional studies have shown no correlation between the presence of diabetic papillopathy and glycemic control or the presence of underlying diabetic retinopathy.
The exact pathophysiology of diabetic papillopathy is unknown and has been a topic of controversy in the literature over many years. Many argue that diabetic papillopathy is a mild form of nonarteritic anterior ischemic optic neuropathy (NAION) as it shares similar findings of optic nerve ischemia, specifically optic disc swelling and hyperemia. Others believe they are two separate entities based on specific characteristic findings, although almost all agree that diabetic papillopathy is an ischemic neuropathy that causes mild, if any, optic nerve dysfunction in patients with diabetes.
Diabetic papillopathy is a diagnosis of exclusion as one must rule out other etiologies for unilateral or bilateral optic nerve head swelling. This differential includes, but is not limited to, NAION, arteritic anterior ischemic optic neuropathy, mass infiltration, optic neuritis, optic disc drusen and papilledema. Examination findings and diagnostic imaging can help differentiate diabetic papillopathy from these other entities. Consultation with a neuro-ophthalmologist is almost always warranted to determine the need for neurologic imaging and additional workup.
On presentation, patients may be asymptomatic or complain of mild blurry vision. Visual acuity is only mildly reduced, and pupillary examination rarely demonstrates a relative afferent pupillary defect. Our patient presented similarly with only a slight decline in visual acuity and no abnormal findings on pupillary examination. Retinal examination demonstrates optic nerve head hyperemia and edema with telangiectasias of the optic disc. Interestingly, diabetic retinopathy is not present in all cases, and only 50% demonstrate dilation of the optic nerve head vasculature.
Functional assessments, such as visual acuity and visual field testing, can help differentiate between diabetic papillopathy and NAION. In diabetic papillopathy, visual function is largely preserved whereas patients with NAION have acute vision loss. Visual field testing most typically demonstrates an enlarged blind spot and rarely demonstrates profound visual field defects in diabetic papillopathy. In NAION, visual field defects are pronounced, indicating significant optic nerve dysfunction. Our patient had objective visual field testing via perimetry that demonstrated no defects, indicating no significant optic nerve dysfunction.
Additionally, further imaging studies such as fluorescein angiography can demonstrate characteristic findings in diabetic papillopathy that help differentiate it from other neuropathies. Most commonly, patients with diabetic papillopathy demonstrate early hyperfluorescence of the optic disc with associated early leakage on fluorescein angiography.
The most widely agreed upon criteria to accurately diagnose diabetic papillopathy include a confirmed diagnosis of type 1 or type 2 diabetes, optic disc edema (either unilateral or bilateral), absence of significant optic nerve dysfunction, and absence of nerve inflammation, infection or infiltration. These criteria were developed based on multiple case reports published in the literature that described the clinical characteristics differentiating diabetic papillopathy from other neuropathies. Regarding our case, the patient met the following criteria: a known history of diabetes, unilateral optic disc edema with no signs of optic nerve dysfunction and no signs or exam findings concerning for inflammation, infection or infiltration of the optic disc.
Given the rarity and self-limitation of this disease, there is a lack of substantial evidence regarding treatment options for patients with diabetic papillopathy. In most cases, disc edema resolves spontaneously within 4 to 8 weeks. Therefore, it is difficult to discern whether resolution of the optic disc edema is secondary to treatment or simply the natural progression of the disease itself. Few studies have reported the successful use of anti-VEGF therapy and corticosteroids as potential treatment options. One case report demonstrated resolution of optic nerve edema in a 50-year-old patient with type 2 diabetes after an intravitreal injection of 4 mg/0.1 mL triamcinolone acetonide. Another study found improvement in optic nerve edema 3 weeks after use of the anti-VEGF bevacizumab. A few case reports have also successfully used both steroids and anti-VEGF therapy together for the management of diabetic papillopathy. Ultimately, the treatment regimen must be individualized for each patient depending on disease severity at presentation and any additional findings of underlying diabetic retinopathy. It is also of utmost importance for the patient to have good glycemic control.
Diabetic papillopathy is an infrequent but important ocular manifestation in patients with diabetes. It presents with either unilateral or bilateral optic nerve edema with hyperemia and minimal vision loss or optic nerve dysfunction. However, as diabetic papillopathy is a diagnosis of exclusion, when a patient with diabetes presents with unilateral or bilateral optic nerve swelling, it is crucial to rule out other etiologies before making the diagnosis of diabetic papillopathy.
Case resolution
Our patient had findings consistent with center-involving macular edema in addition to diabetic papillopathy, which was likely contributing to his decline in visual acuity. Therefore, the decision was made to administer intravitreal anti-VEGF therapy to treat both the macular edema and optic nerve edema. Multiple rounds of anti-VEGF therapy were required for successful resolution of both his optic nerve edema and macular edema. He subsequently underwent panretinal photocoagulation laser treatment of both eyes for his high-risk features. One year after treatment, the patient’s visual acuity improved to 20/20 in both eyes. He is followed closely every 4 to 6 weeks to assess for any evidence of recurring macular edema. Thus far, he has had treat-and-extend therapy, most recently needing an anti-VEGF injection at a 12-week interval. Additionally, he has achieved glucose control with a most recent HbA1c of 6.2%.
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- For more information:
- Allison Resnik, MD, and Caroline Baumal, 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.