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I.

Case History

Patient demographics: 49-year-old white male veteran


Chief complaint: blurry spot in vision OS x 1 week
Medical history:
o Diabetes type 2 for 14 years, last blood glucose: 183 mg/dl, last
HbA1c: 10.4%
o Obesity, hyperlipidemia, hypertension
Ocular history:
o Diabetes type 2 with history of mild non-proliferative diabetic
retinopathy (NPDR) OU without clinically significant macular edema
(CSME)
o Cataracts OU (NS)
o Dry eye syndrome OU
Medications
o amoxicillin
o atorvastatin calcium
o cholecalciferol
o glipizide
o metformin

II. Pertinent findings

Uncorrected visual acuity: OD: 20/40-, Pinhole OD: 20/25; OS: 20/20-2
Posterior segment findings:
o Optic nerve findings
OD cup-to-disc ratio: 0.20 round; disc edema superiorly and
nasally, drance hemorrhages superiorly and nasally
OS cup-to-disc ratio: 0.10 round; disc edema superiorly and
nasally, drance hemorrhages superonasally
o Maculae flat and avascular with no CSME OU
o Posterior Pole findings:
OD: cluster of dot hemorrhages between macula and optic
nerve head (ONH), dot hemorrhages nasal to ONH, no
neovascularization elsewhere (NVE)
OS: single dot hemorrhage at superior arcade, blot
hemorrhages at inferior arcade (-)NVE
Heidelberg optical coherence tomography (OCT) of optic nerve
o Superior and nasal disc edema OU
Heidelberg optical coherence tomography (OCT) of macula
o OD: small cystic spaces with exudates
o OS: normal
Humphrey Visual Field (HVF) 30-2 Sita Fast
o OD: reliable; full with no defects
o OS: reliable; inferior defect crossing vertical midline

III. Differential diagnosis


Primary: presumed bilateral diabetic papillopathy (DP)
diagnosis remains presumed awaiting neurology consult/lumbar
puncture scheduled for September 15, 2015
Other differentials: papilledema, idiopathic intracranial hypertension,
infectious or de-myelinating optic neuritis, non-arteritic and arteritic
ischemic optic neuropathy, optic nerve head drusen, metastatic infiltration,
stage IV hypertensive retinopathy

IV. Diagnosis and discussion


Diagnosis: presumed bilateral diabetic papillopathy
o requires other known causes of disc edema to be ruled out
Pathogenesis:
o exact pathogenesis of the condition is unclear
Clinical features:
o disc edema in a diabetic patient
o patients frequently have small cup-to-disc ratios
Differentiating non-arteritic ischemic optic neuropathy (NAION) vs. DP
o discussion of fluorescein angiography findings
Course of disease:
o Most cases show self-resolution within three months to one year of
onset with minimal long-term effects on optic nerve/visual field.
New treatment approaches: inhibitors of vascular endothelial growth factor
(VEGF)

V. Treatment, management
The following are the tests ordered at initial visit along with their results:
Magnetic resonance imaging (MRI) of the brain with and without contrast:
normal, no evidence of intracranial lesions, normal ventricles for age
Vitamin B12, erythrocyte sedimentation rate, C-reactive protein, angiotensin
converting enzyme all normal, anti-nuclear antibody negative
Complete blood count showed leukocytosis
o Hematology/oncology referral ruled out leukemia
Neurology consult for lumbar puncture to rule out increased intracranial
pressure
Patient referred to retina specialist due to cystic spaces and recent literature
regarding possible anti-VEGF treatment retina specialist concurs with
current plan to observe patient, initiate work-up, no treatment recommended

Follow-up #2: Fundus Fluorescein Angiography (FFA)


-one month after initial exam
Patient reports no change in symptoms
Uncorrected visual acuity: OD: 20/25-2 OS: 20/20-2
Posterior segment findings stable to initial exam with mild NPDR
FFA reveals areas of peripapillary staining/hyperfluorescense OS>OD with no
diffuse leakage

Follow-up #3: Dilated fundus examination, Heidelberg OCT, HVF 30-2 Sita Fast
-two months after initial exam
Patient reports no change in symptoms
Uncorrected visual acuity: OD: 20/30-2, Pinhole OD:20/25 OS: 20/20
Posterior segment findings:
o Cup-to-disc ratios:
OD: 0.20 round, disc edema greatest inferonasally with slight
edema superiorly
OS: 0.10 round, disc edema greatest inferiorly with slight
edema superonasally
o Maculae flat and avascular with no CSME OU
o Posterior pole findings:
OD: scattered dot hemorrhages with 2-3 blot hemorrhages
between optic nerve and macula, no NVE
OS: dot and blot hemorrhages radiating from inferotemporal
disc margin to macula, no NVE
o Peripheral fundus unremarkable OU with no holes, tears, or
detachments
Heidelberg OCT Optic Nerve:
o OD: increased edema superiorly compared to initial exam
o OS: edema noted superiorly at onset has decreased in thickness, new
area of significant edema inferiorly
Heidelberg OCT Macula: OD only
o small cystic spaces noted previously show subtle increase in size since
initial exam
Humphrey Visual Field 30-2 Sita Fast
o OD: reliable; scattered non-specific defects
o OS: reliable; inferior defect crossing vertical midline stable
Plan: return in 2 months for dilated fundus examination and repeat
Heidelberg OCT Optic Nerve

VI. Conclusion

Clinical Pearls
A full workup including optical coherence tomography, visual field,
fluorescein angiography, magnetic resonance imaging, lumbar puncture,
complete blood count, vitamin B12/folate, erythrocyte sedimentation rate, C-
reactive protein, anti-nucleotide antibody, and angiotensin converting
enzyme is indicated to rule out other etiologies.
An evaluation with a retina specialist is indicated if findings are accompanied
by macular edema. Some studies have shown rapid resolution of disc edema
status post intravitreal injection with inhibitors of vascular endothelial
growth factor.
Once diagnosed, patients with diabetic papillopathy should be followed more
closely than their retinopathy alone suggests, possibly on 3 month intervals
until full resolution as it has been suggested that diabetic papillopathy is a
precursor to worsening non-proliferative diabetic retinopathy or progression
to proliferative diabetic retinopathy (PDR).
Alerting primary care physicians of diabetic patients who are at greater risk
of developing DP (such as those with smaller cup-to-disc ratios), could
caution them to implement a slower reduction in blood glucose in those
patients.

*Note to reviewer:
Available images for presentation include the following:
Fundus photos
Heidelberg Optic Nerve (serial OCTs)
Heidelberg OCT Macula (serial OCTs)
Fluorescein angiography
Visual fields