A Case of Hemorrhagic Retinal Arterial Macroaneurysm by Valsalva Maneuver
Published 2023 by Sally Chu
Program Number: R2023182
Article Type: Residents Day
Board: R-166
Purpose:
A patient with uncontrolled hypertension presents to clinic with a multi-layered hemorrhage extending from a retinal arterial macroneurysm (RAM) after sneezing. This is a rare case of hemorrhagic RAM induced by Valsalva maneuver.
Case History:
• 74-year-old Caucasian male • CC: black spot in vision OD noticed after sneezing • POH: 0.20 DD quiescent form retinal arterial aneurysm OD (noted on exam 4 days prior), diabetes without retinopathy OU, dry eyes OU, posterior vitreous detachment OD, cataracts OU, corneal scars OD • PMH: diabetes mellitus type 2, hypertension, hyperlipidemia, benign prostatic hyperplasia • Meds: lisinopril, metoprolol, hydrochlorothiazide, metformin, rivaroxaban, simvastatin, finasteride, tamsulosin, sildenafil
Pertinent Findings:
Clinical • VAsc: OD: 20/40 PH 20/25, OS: 20/40- PH 20/25 • Anterior segment: temporal subconjunctival hemorrhage OD • Posterior segment: 0.20 DD hemorrhagic RAM along IT arcade with large pre-retinal/retinal/subretinal/vitreous hemorrhage inferior temporal to macula extending inferiorly from RAM to far periphery OD, mild arteriole attenuation and tortuosity OU Physical • Recent HBA1C%: 6.3, Recent BP: 152/68 mmHg OCT Macula • OD: multilayer hemorrhage (vitreous, pre-retinal, retinal, subretinal) extending from inferior temporal RAM, vitreous cells associated with vitreous hemorrhage Referral: Retina
Differential Diagnosis:
proliferative diabetic retinopathy, Valsalva retinopathy, Terson syndrome, proliferative retinopathy associated with retinal vein occlusion, posterior vitreous detachment with retinal break
Diagnosis & Discussion:
RAMs are characterized by a focal dilation of a weakened arterial wall, typically at a bifurcation of the central retinal artery in the temporal retina. The vessel, weakened by atherosclerosis, is highly susceptible to arterial dilation from increased hydrostatic pressure, making hypertension a significant risk factor for RAMs. RAMs can be classified into three forms: quiescent, hemorrhagic, and exudative. The first tends to present asymptomatically while the latter two differ in their clinical presentation. When intraluminal pressure exceeds what the fragile aneurysm wall can withstand, an acute rupture results in multi-layer hemorrhages. In the event of a Valsalva maneuver, a forced expiration against a closed airway, a sudden rise in blood pressure, intrathoracic pressure, and intra-abdominal pressure results in a vast cardiovascular response. In patients with RAMs, a Valsalva maneuver can cause a transient fluctuation in blood pressure and trigger an acute RAM rupture.
Treatment, Management:
Due to the inferior location of the hemorrhagic RAM and unaffected acuities, the patient was initially observed within optometry and referred to his primary care physician to improve his blood pressure control and reduce risk factors. RAMs that do not affect the visual acuity tend to have a favorable prognosis as most spontaneously resolve over a period of months. In these cases, observation is the recommended management. In complicated cases where persistent hemorrhages threaten the macula, laser photocoagulation, anti-VEGF intravitreal injection, and pars plana vitrectomy are all viable options of treatment depending on the type of hemorrhage present. On this patient's follow-up, a retina specialist was consulted, and laser photocoagulation was elected to aid with hemorrhage resorption due to persistent subretinal and vitreous hemorrhages. Later follow-ups status post laser showed resolved hemorrhages and stable RAM on clinical examination as well as on OCT and OCT-Angiography.
Conclusion:
The pathophysiology of retinal arterial macroaneurysms makes them highly susceptible for rupture in cases of transient fluctuations in blood pressure. A diagnosis of ruptured hemorrhagic RAM by Valsalva maneuver should be considered in patients presenting with multi-level hemorrhages extending from a RAM given a case history of Valsalva maneuver prior to symptom onset.
Purpose:
A patient with uncontrolled hypertension presents to clinic with a multi-layered hemorrhage extending from a retinal arterial macroneurysm (RAM) after sneezing. This is a rare case of hemorrhagic RAM induced by Valsalva maneuver.
Case History:
• 74-year-old Caucasian male • CC: black spot in vision OD noticed after sneezing • POH: 0.20 DD quiescent form retinal arterial aneurysm OD (noted on exam 4 days prior), diabetes without retinopathy OU, dry eyes OU, posterior vitreous detachment OD, cataracts OU, corneal scars OD • PMH: diabetes mellitus type 2, hypertension, hyperlipidemia, benign prostatic hyperplasia • Meds: lisinopril, metoprolol, hydrochlorothiazide, metformin, rivaroxaban, simvastatin, finasteride, tamsulosin, sildenafil
Pertinent Findings:
Clinical • VAsc: OD: 20/40 PH 20/25, OS: 20/40- PH 20/25 • Anterior segment: temporal subconjunctival hemorrhage OD • Posterior segment: 0.20 DD hemorrhagic RAM along IT arcade with large pre-retinal/retinal/subretinal/vitreous hemorrhage inferior temporal to macula extending inferiorly from RAM to far periphery OD, mild arteriole attenuation and tortuosity OU Physical • Recent HBA1C%: 6.3, Recent BP: 152/68 mmHg OCT Macula • OD: multilayer hemorrhage (vitreous, pre-retinal, retinal, subretinal) extending from inferior temporal RAM, vitreous cells associated with vitreous hemorrhage Referral: Retina
Differential Diagnosis:
proliferative diabetic retinopathy, Valsalva retinopathy, Terson syndrome, proliferative retinopathy associated with retinal vein occlusion, posterior vitreous detachment with retinal break
Diagnosis & Discussion:
RAMs are characterized by a focal dilation of a weakened arterial wall, typically at a bifurcation of the central retinal artery in the temporal retina. The vessel, weakened by atherosclerosis, is highly susceptible to arterial dilation from increased hydrostatic pressure, making hypertension a significant risk factor for RAMs. RAMs can be classified into three forms: quiescent, hemorrhagic, and exudative. The first tends to present asymptomatically while the latter two differ in their clinical presentation. When intraluminal pressure exceeds what the fragile aneurysm wall can withstand, an acute rupture results in multi-layer hemorrhages. In the event of a Valsalva maneuver, a forced expiration against a closed airway, a sudden rise in blood pressure, intrathoracic pressure, and intra-abdominal pressure results in a vast cardiovascular response. In patients with RAMs, a Valsalva maneuver can cause a transient fluctuation in blood pressure and trigger an acute RAM rupture.
Treatment, Management:
Due to the inferior location of the hemorrhagic RAM and unaffected acuities, the patient was initially observed within optometry and referred to his primary care physician to improve his blood pressure control and reduce risk factors. RAMs that do not affect the visual acuity tend to have a favorable prognosis as most spontaneously resolve over a period of months. In these cases, observation is the recommended management. In complicated cases where persistent hemorrhages threaten the macula, laser photocoagulation, anti-VEGF intravitreal injection, and pars plana vitrectomy are all viable options of treatment depending on the type of hemorrhage present. On this patient's follow-up, a retina specialist was consulted, and laser photocoagulation was elected to aid with hemorrhage resorption due to persistent subretinal and vitreous hemorrhages. Later follow-ups status post laser showed resolved hemorrhages and stable RAM on clinical examination as well as on OCT and OCT-Angiography.
Conclusion:
The pathophysiology of retinal arterial macroaneurysms makes them highly susceptible for rupture in cases of transient fluctuations in blood pressure. A diagnosis of ruptured hemorrhagic RAM by Valsalva maneuver should be considered in patients presenting with multi-level hemorrhages extending from a RAM given a case history of Valsalva maneuver prior to symptom onset.
A Case of Immune Checkpoint Inhibitor (Pembrolizumab) Induced Uveitis, Scleritis and Papillitis in a Patient with Breast Cancer
Published 2023 by Kristen Lamoreau
Co-Author(s): Kristen Lamoreau
Program Number: 235377
Article Type: Scientific Program
Board: 93
PURPOSE:
Pembrolizumab is a humanized monoclonal antibody that inhibits programmed cell death-1 (PD-1) pathway resulting in an enhanced immune response. Currently, pembrolizumab is an approved treatment for triple-negative breast cancer (TNBC). Very few patients (<1%) on pembrolizumab develop ocular complications. Ocular immunotherapy-related adverse events (IRAEs) are immune mediated and the most common adverse event is anterior uveitis, followed by panuveitis. In these cases, cessation of pembrolizumab with or without the addition of steroids generally improves symptoms.
CASE REPORT:
A 57-year-old woman with a history of triple-negative right breast cancer with ongoing pembrolizumab therapy presented with complaints of decreased vision in both eyes and eye pain. Visual acuity was 20/30 OD and 20/20-3 OS, pupils were reactive to light without an afferent pupillary defect, and color vision was intact. Slit lamp exam revealed granulomatous KP bilaterally. Fundus examination revealed Friesen grade IV OD and grade III OS disc edema. MRI demonstrated findings consistent with a T-sign (Scleritis) bilaterally. Blood work excluded infectious causes, and lumbar puncture was unrevealing with a normal opening pressure. An extensive workup of the central nervous system was also done without a diagnosis alternative to the conclusion that this was a complication of her ongoing immunotherapy. Pembrolizumab was then discontinued. She was started on a 60mg dose of prednisone with a gradual taper of 10mg per week. Close follow-up revealed progressive reduction of disc edema.
CONCLUSION:
This case serves as a reminder that when oncology patients experience ocular inflammatory disease, it is crucial for clinicians to be vigilant of the ocular and neurologic complications in patients receiving immune checkpoint inhibitors. While the occurrence of such complications is currently rare, it is anticipated that uveitis in oncology patients will become more common as immune checkpoint inhibitors are increasingly utilized in the treatment of various types of tumors.
PURPOSE:
Pembrolizumab is a humanized monoclonal antibody that inhibits programmed cell death-1 (PD-1) pathway resulting in an enhanced immune response. Currently, pembrolizumab is an approved treatment for triple-negative breast cancer (TNBC). Very few patients (<1%) on pembrolizumab develop ocular complications. Ocular immunotherapy-related adverse events (IRAEs) are immune mediated and the most common adverse event is anterior uveitis, followed by panuveitis. In these cases, cessation of pembrolizumab with or without the addition of steroids generally improves symptoms.
CASE REPORT:
A 57-year-old woman with a history of triple-negative right breast cancer with ongoing pembrolizumab therapy presented with complaints of decreased vision in both eyes and eye pain. Visual acuity was 20/30 OD and 20/20-3 OS, pupils were reactive to light without an afferent pupillary defect, and color vision was intact. Slit lamp exam revealed granulomatous KP bilaterally. Fundus examination revealed Friesen grade IV OD and grade III OS disc edema. MRI demonstrated findings consistent with a T-sign (Scleritis) bilaterally. Blood work excluded infectious causes, and lumbar puncture was unrevealing with a normal opening pressure. An extensive workup of the central nervous system was also done without a diagnosis alternative to the conclusion that this was a complication of her ongoing immunotherapy. Pembrolizumab was then discontinued. She was started on a 60mg dose of prednisone with a gradual taper of 10mg per week. Close follow-up revealed progressive reduction of disc edema.
CONCLUSION:
This case serves as a reminder that when oncology patients experience ocular inflammatory disease, it is crucial for clinicians to be vigilant of the ocular and neurologic complications in patients receiving immune checkpoint inhibitors. While the occurrence of such complications is currently rare, it is anticipated that uveitis in oncology patients will become more common as immune checkpoint inhibitors are increasingly utilized in the treatment of various types of tumors.
A Case of Ocular Ischemic Syndrome featuring Rare Bilateral Mid-peripheral Hemorrhages
Published 2023 by Justin Alexander
Program Number: R2023141
Article Type: Residents Day
Board: R-125
Purpose:
The purpose of this presentation is to highlight the clinical features, diagnostic approach, and management strategies for Ocular Ischemic Syndrome (OIS), a rare but potentially sight-threatening condition.
Case History:
69-year-old Caucasian male CC: Diabetic Eye Exam and OCT-RNFL for POAG. Patient has good vision with current glasses. No complaints reported at exam. POH: POAG Moderate stage OU, Nuclear sclerotic cataracts OU Type II Diabetes without retinopathy OU, A1c: 7.9%. PMH: Diabetes Type II, Hypertension, Hyperlipidemia, s/p right Endarterectomy Meds: Unknown due to Outside PCP provider. Ocular Meds: Latanoprost QHS OU, Brimonidine BID OU, COSOPT OU
Pertinent Findings:
a. BCVA: OD: 20/25 OS: 20/25 b. (-) NVI, (-) NVG, (-) NVD c. Lens: i. OD: 1+ NSC, 1+ CC, scattered vacuoles OS: 1+ NSC, 1+ CC, 1+ PSC, scattered vacuoles d. Treated IOP OD: 20, OS: 23 e. CD ratios OD: 0.10H/V OS: 0.30H/V f. Posterior Pole OU: Midperipheral Hemorrhages Physical BP: 195/80, 182/73 BP at home: 168/82 in office, H/o white coat syndrome Laboratory Testing done outside VA: s/p right Endarterectomy 2016 and, 30% blockage left internal carotid artery. Fundus Photos: Bilateral mid-peripheral hemes Macular OCT: WNL: Provided to rule out macular edema. Referred by Cardiologist
Differential Diagnosis:
Moderate Non-Proliferative Diabetic Retinopathy, Central Retinal Vein Occlusion
Diagnosis & Discussion:
The development of Ocular Ischemic Syndrome (OIS) is linked to atherosclerosis of the arteries, which leads to persistent inadequate blood supply to the eyes. OIS manifests unilaterally in 80% of cases, resulting in the involvement of just one eye. In situations like the current case, around 20% of instances involve stenosis of the Internal Carotid Artery (ICA), leading to insufficient blood perfusion affecting both eyes. When patients experience symptomatic carotid artery occlusion, approximately one-third of them will show related alterations in retinal blood vessels, even if they do not exhibit visual symptoms. In a usual case of OIS, individuals tend to experience unfavorable visual outcomes such as transient visual loss, and ischemic ocular pain. However, in this case, the patient exhibited positive vision despite the presence of intraretinal hemorrhages.
Treatment, Management:
The primary approach for addressing ischemic events like OIS involves preventive care. The patient was directed to our care by their cardiologist to undergo a comprehensive eye examination, aiming to identify any pathological signs resulting from their extensive history of diabetes, hypertension, and hyperlipidemia. Prior to the eye examination, a carotid doppler was carried out, followed by a right Endarterectomy. To maintain stable IOP and prevent Neovascular glaucoma, the patient's treatment plan incorporated Latanoprost, Brimonidine, and COSOPT. After the follow-up, improvements were noted in both blood pressure and A1c levels resulting in reduced intra-retinal hemorrhages. In the absence of NVI, ischemic alterations are not of a chronic concern. As a result, surgical intervention may not yield substantial visual benefits for the patient, although symptomatic relief could be improved. The patient was put on aspirin therapy as a preventive measure against strokes.
Conclusion:
The case of OIS characterized by the presence of rare bilateral mid-peripheral hemorrhages shows the relationship between vascular health and ocular manifestations. Through careful, comprehensive medical management, and eye examinations, there exists a pathway to alleviate symptomatic distress and preserve vision. The unique presentation of bilateral mid-peripheral hemorrhages serves as a reminder of the nuanced nature of ocular diseases, urging for continued research and clinical awareness.
Purpose:
The purpose of this presentation is to highlight the clinical features, diagnostic approach, and management strategies for Ocular Ischemic Syndrome (OIS), a rare but potentially sight-threatening condition.
Case History:
69-year-old Caucasian male CC: Diabetic Eye Exam and OCT-RNFL for POAG. Patient has good vision with current glasses. No complaints reported at exam. POH: POAG Moderate stage OU, Nuclear sclerotic cataracts OU Type II Diabetes without retinopathy OU, A1c: 7.9%. PMH: Diabetes Type II, Hypertension, Hyperlipidemia, s/p right Endarterectomy Meds: Unknown due to Outside PCP provider. Ocular Meds: Latanoprost QHS OU, Brimonidine BID OU, COSOPT OU
Pertinent Findings:
a. BCVA: OD: 20/25 OS: 20/25 b. (-) NVI, (-) NVG, (-) NVD c. Lens: i. OD: 1+ NSC, 1+ CC, scattered vacuoles OS: 1+ NSC, 1+ CC, 1+ PSC, scattered vacuoles d. Treated IOP OD: 20, OS: 23 e. CD ratios OD: 0.10H/V OS: 0.30H/V f. Posterior Pole OU: Midperipheral Hemorrhages Physical BP: 195/80, 182/73 BP at home: 168/82 in office, H/o white coat syndrome Laboratory Testing done outside VA: s/p right Endarterectomy 2016 and, 30% blockage left internal carotid artery. Fundus Photos: Bilateral mid-peripheral hemes Macular OCT: WNL: Provided to rule out macular edema. Referred by Cardiologist
Differential Diagnosis:
Moderate Non-Proliferative Diabetic Retinopathy, Central Retinal Vein Occlusion
Diagnosis & Discussion:
The development of Ocular Ischemic Syndrome (OIS) is linked to atherosclerosis of the arteries, which leads to persistent inadequate blood supply to the eyes. OIS manifests unilaterally in 80% of cases, resulting in the involvement of just one eye. In situations like the current case, around 20% of instances involve stenosis of the Internal Carotid Artery (ICA), leading to insufficient blood perfusion affecting both eyes. When patients experience symptomatic carotid artery occlusion, approximately one-third of them will show related alterations in retinal blood vessels, even if they do not exhibit visual symptoms. In a usual case of OIS, individuals tend to experience unfavorable visual outcomes such as transient visual loss, and ischemic ocular pain. However, in this case, the patient exhibited positive vision despite the presence of intraretinal hemorrhages.
Treatment, Management:
The primary approach for addressing ischemic events like OIS involves preventive care. The patient was directed to our care by their cardiologist to undergo a comprehensive eye examination, aiming to identify any pathological signs resulting from their extensive history of diabetes, hypertension, and hyperlipidemia. Prior to the eye examination, a carotid doppler was carried out, followed by a right Endarterectomy. To maintain stable IOP and prevent Neovascular glaucoma, the patient's treatment plan incorporated Latanoprost, Brimonidine, and COSOPT. After the follow-up, improvements were noted in both blood pressure and A1c levels resulting in reduced intra-retinal hemorrhages. In the absence of NVI, ischemic alterations are not of a chronic concern. As a result, surgical intervention may not yield substantial visual benefits for the patient, although symptomatic relief could be improved. The patient was put on aspirin therapy as a preventive measure against strokes.
Conclusion:
The case of OIS characterized by the presence of rare bilateral mid-peripheral hemorrhages shows the relationship between vascular health and ocular manifestations. Through careful, comprehensive medical management, and eye examinations, there exists a pathway to alleviate symptomatic distress and preserve vision. The unique presentation of bilateral mid-peripheral hemorrhages serves as a reminder of the nuanced nature of ocular diseases, urging for continued research and clinical awareness.
A Case of Optic Neuropathy Secondary to Compression from Dolichoectatic Internal Carotid Artery (DICA)
Published 2023 by Jessica Chung
Program Number: R2023107
Article Type: Residents Day
Board: R-91
Purpose:
Patient presents with clinical signs correlating to NAION. However, with progressive visual field loss and left optic nerve pallor, MRI scans ordered reveals compressive optic neuropathy 2' to mass effect from a dilated left ICA.
Case History:
81-year-old Caucasian male -CC: Shadow in the inferior half of the visual field progressing superiorly OS -POH: PVD OU, ERM OU, pseudophakia OU, history of traumatic RD with s/p Photocoagulation OU, Decompensated left hyperphoria -PMH: Chronic kidney disease, hypertension, hyperlipidemia, Type 2 diabetes mellitus, prostate cancer, sleep apnea, TIA, hypertensive heart disease without CHF. -MEDS: atorvastatin, Entresto, metoprolol, empagliflozin, famotidine, furosemide, aspirin, spironolactone
Pertinent Findings:
Clinical -BCVA OD: 20/25, OS: 20/25 -DFE: small C/D ratio OU, initial presentation of edematous superior rim with a small drance heme OS progressing to mild pallor, PVD OU, peripheral photocoagulation scars @ 6:00 & 12:00, few DBH OU Physical -Last A1c: 7.4% -Last BP(mmHg): 142/76 Laboratory Testing: Normal CBC, sed rate, CRP levels OCT macula: OD: VMT; OS: GCL with progressive inferior>superior thinning OCT RNFL: OD: Borderline thinning IT; OS: Initial elevation ST/SN with progressive thinning at following exams HVF 24-2: OD: scattered inferior defects; OS: progressive increase in dense inferior cluster defects MRI/A: -Left ICA compression on left optic nerve -Anterior clinoid process meningioma Optos: Superior optic nerve edema progressing to optic nerve pallor Referral: Neurosurgery
Differential Diagnosis:
Current differentials include, Non-arteritic ischemic optic neuropathy (NAION), Arteritic anterior ischemic optic neuropathy (AAION), CRAO, compressive neuropathy, toxic neuropathy, optic neuritis
Diagnosis & Discussion:
There are multiple possible etiologies/risk factors of optic neuropathy, such as optic nerve compression, inflammation, vascular disease, and trauma. While NAION is a common cause, it is important to pursue a thorough workup when atypical features are present. DICA causing optic nerve compression has been reported in a few case reports but little is known about its etiology. Hypertension and other vascular conditions have been noted in patients with DICA(1,2). Because DICA is uncommon and can be asymptomatic, most cases are found incidental through neuroimaging(2,3,4,5). In one report, a significant percentage of normal tension glaucoma (NTG) patients had optic nerve compression by ICA(6). This suggests consideration of an alternative cause of optic neuropathy in patients with atypical NTG. This case report further supports the importance of thorough clinical work up with cases of unexplained optic neuropathy and progressive visual field loss to prevent delay in diagnosis.
Treatment, Management:
This patient was referred for a neurosurgery consultation to evaluate surgical treatment for DICA. Ultimately, surgery was not an option for this patient and his acuity remains 20/25 though his visual field defect has deepened. Many cases show stability in vision loss, though some cases do demonstrate progression (5,8). There is limited evidence for surgical options in progressive cases – one case report noted visual improvement after optic nerve decompression(9). DICA has been associated with vascular conditions such as hypertension and sleep apnea(1,2). Therefore, control of vascular conditions, especially our patient's uncontrolled sleep apnea along with hypertension should be beneficial but more research needs to be done on vascular risks of DICA.
Conclusion:
Optic nerve compression by ICA should be considered in cases of optic nerve pallor and progressive visual field loss. It is possible ICA compression is a more common diagnosis and should be considered in cases of atypical NAIONs and NTG. Treatment should be considered on a case-by-case basis.
Purpose:
Patient presents with clinical signs correlating to NAION. However, with progressive visual field loss and left optic nerve pallor, MRI scans ordered reveals compressive optic neuropathy 2' to mass effect from a dilated left ICA.
Case History:
81-year-old Caucasian male -CC: Shadow in the inferior half of the visual field progressing superiorly OS -POH: PVD OU, ERM OU, pseudophakia OU, history of traumatic RD with s/p Photocoagulation OU, Decompensated left hyperphoria -PMH: Chronic kidney disease, hypertension, hyperlipidemia, Type 2 diabetes mellitus, prostate cancer, sleep apnea, TIA, hypertensive heart disease without CHF. -MEDS: atorvastatin, Entresto, metoprolol, empagliflozin, famotidine, furosemide, aspirin, spironolactone
Pertinent Findings:
Clinical -BCVA OD: 20/25, OS: 20/25 -DFE: small C/D ratio OU, initial presentation of edematous superior rim with a small drance heme OS progressing to mild pallor, PVD OU, peripheral photocoagulation scars @ 6:00 & 12:00, few DBH OU Physical -Last A1c: 7.4% -Last BP(mmHg): 142/76 Laboratory Testing: Normal CBC, sed rate, CRP levels OCT macula: OD: VMT; OS: GCL with progressive inferior>superior thinning OCT RNFL: OD: Borderline thinning IT; OS: Initial elevation ST/SN with progressive thinning at following exams HVF 24-2: OD: scattered inferior defects; OS: progressive increase in dense inferior cluster defects MRI/A: -Left ICA compression on left optic nerve -Anterior clinoid process meningioma Optos: Superior optic nerve edema progressing to optic nerve pallor Referral: Neurosurgery
Differential Diagnosis:
Current differentials include, Non-arteritic ischemic optic neuropathy (NAION), Arteritic anterior ischemic optic neuropathy (AAION), CRAO, compressive neuropathy, toxic neuropathy, optic neuritis
Diagnosis & Discussion:
There are multiple possible etiologies/risk factors of optic neuropathy, such as optic nerve compression, inflammation, vascular disease, and trauma. While NAION is a common cause, it is important to pursue a thorough workup when atypical features are present. DICA causing optic nerve compression has been reported in a few case reports but little is known about its etiology. Hypertension and other vascular conditions have been noted in patients with DICA(1,2). Because DICA is uncommon and can be asymptomatic, most cases are found incidental through neuroimaging(2,3,4,5). In one report, a significant percentage of normal tension glaucoma (NTG) patients had optic nerve compression by ICA(6). This suggests consideration of an alternative cause of optic neuropathy in patients with atypical NTG. This case report further supports the importance of thorough clinical work up with cases of unexplained optic neuropathy and progressive visual field loss to prevent delay in diagnosis.
Treatment, Management:
This patient was referred for a neurosurgery consultation to evaluate surgical treatment for DICA. Ultimately, surgery was not an option for this patient and his acuity remains 20/25 though his visual field defect has deepened. Many cases show stability in vision loss, though some cases do demonstrate progression (5,8). There is limited evidence for surgical options in progressive cases – one case report noted visual improvement after optic nerve decompression(9). DICA has been associated with vascular conditions such as hypertension and sleep apnea(1,2). Therefore, control of vascular conditions, especially our patient's uncontrolled sleep apnea along with hypertension should be beneficial but more research needs to be done on vascular risks of DICA.
Conclusion:
Optic nerve compression by ICA should be considered in cases of optic nerve pallor and progressive visual field loss. It is possible ICA compression is a more common diagnosis and should be considered in cases of atypical NAIONs and NTG. Treatment should be considered on a case-by-case basis.
A Case of Schnyder Corneal Dystrophy
Published 2023 by Leah Margolis
Program Number: R2023039
Article Type: Residents Day
Board: R-23
Purpose:
A patient with Schnyder Corneal Dystrophy (SCD) presents with blurred vision, central corneal crystals, and limbal arcus. This case report discusses typical findings and treatment for SCD, and important points for patient education and expectations.
Case History:
20 year old Hispanic female presents to cornea clinic for a 1 year follow-up CC: Concern for “blue/white ring” around her eyes, worsening Secondary complaints: Blurred vision, dryness and irritation OU POH: Schnyder corneal dystrophy (dx age 6); low hyperopia/astigmatism OU PMH: Borderline elevated cholesterol No FHx of SCD or other corneal conditions Meds: ATs PRN
Pertinent Findings:
Clinical BCVA 20/25 OD, 20/30 OS Pachymetry 582 OD, 571 OS Corneal findings: arcus 360 OU, central crystalline deposits in anterior stroma (2.8mm V x 3.5mm H OD, 2.2mm V x 3.6mm OS) with no overlying epithelial defect, mild surrounding haze OU Physical Cholesterol labs (date unknown; likely >2 years): LDL 132, ref normal 100 Ancillary Testing Slit lamp photos: arcus 360 deg, central crystalline deposits; symmetric appearance between the eyes Anterior segment OCT: central circumscribed area of refractile deposits in stroma, more concentrated anterior stroma Pentacam: irregular astigmatism OU
Differential Diagnosis:
Schnyder corneal dystrophy, infectious crystalline keratopathy, cystinosis, Bietti crystalline corneoretinal dystrophy, lymphoproliferative disorders (I.e. multiple myeloma, monoclonal gammopathy)
Diagnosis & Discussion:
Schnyder corneal dystrophy is a rare corneal stromal dystrophy with autosomal dominant inheritance. Corneal findings include central corneal haze and/or crystals, arcus lipoides, and mid-peripheral haze later in the disease. The characteristic corneal crystals are composed of cholesterol and lipid deposits in the anterior stroma. Notably, corneal crystals are not required for the diagnosis of SCD. The disease has been localized to the UBIAD1 gene on chromosome 1p36.3, which is involved in cholesterol metabolism. The pathophysiology of SCD is thought to involve a localized defect of lipid metabolism. Systemic control of cholesterol has been shown not to alter the progression of corneal disease. SCD is a slowly progressive disease, with onset as early as the first decade of life. Main symptoms include glare and reduced vision worse in photopic conditions.
Treatment, Management:
Mydriatics may allow patients to see around the central corneal crystals, as SCD disproportionately affects photopic vision. This patient was treated with 0.5% tropicamide and low power reading glasses at her last visit, with no subjective improvement. Other options for treatment are surgical and include PTK to remove corneal crystals, and PKP or DALK for patients with significant glare or reduced vision. However, corneal findings have been shown to recur after surgery. It is important for patients to understand that surgery will not eliminate arcus, as the peripheral cornea is not replaced in a transplant. A larger button increases the risk of graft rejection and irregular astigmatism, potentially requiring wear of GP or scleral lenses for optimal vision after surgery. Due to this patient's young age and good BCVA, she will continue to be monitored, rather than pursue surgical intervention at this time. Punctal plugs were inserted at her visit to address her complaint of dryness.
Conclusion:
Schnyder Corneal Dystrophy occurs as a localized defect of lipid metabolism, not necessarily systemic disease. Unfortunately, there is no known treatment to stop progression of this condition. The visual symptoms of SCD can be managed with PTK or corneal transplantation, although dystrophy can recur after surgery.
Purpose:
A patient with Schnyder Corneal Dystrophy (SCD) presents with blurred vision, central corneal crystals, and limbal arcus. This case report discusses typical findings and treatment for SCD, and important points for patient education and expectations.
Case History:
20 year old Hispanic female presents to cornea clinic for a 1 year follow-up CC: Concern for “blue/white ring” around her eyes, worsening Secondary complaints: Blurred vision, dryness and irritation OU POH: Schnyder corneal dystrophy (dx age 6); low hyperopia/astigmatism OU PMH: Borderline elevated cholesterol No FHx of SCD or other corneal conditions Meds: ATs PRN
Pertinent Findings:
Clinical BCVA 20/25 OD, 20/30 OS Pachymetry 582 OD, 571 OS Corneal findings: arcus 360 OU, central crystalline deposits in anterior stroma (2.8mm V x 3.5mm H OD, 2.2mm V x 3.6mm OS) with no overlying epithelial defect, mild surrounding haze OU Physical Cholesterol labs (date unknown; likely >2 years): LDL 132, ref normal 100 Ancillary Testing Slit lamp photos: arcus 360 deg, central crystalline deposits; symmetric appearance between the eyes Anterior segment OCT: central circumscribed area of refractile deposits in stroma, more concentrated anterior stroma Pentacam: irregular astigmatism OU
Differential Diagnosis:
Schnyder corneal dystrophy, infectious crystalline keratopathy, cystinosis, Bietti crystalline corneoretinal dystrophy, lymphoproliferative disorders (I.e. multiple myeloma, monoclonal gammopathy)
Diagnosis & Discussion:
Schnyder corneal dystrophy is a rare corneal stromal dystrophy with autosomal dominant inheritance. Corneal findings include central corneal haze and/or crystals, arcus lipoides, and mid-peripheral haze later in the disease. The characteristic corneal crystals are composed of cholesterol and lipid deposits in the anterior stroma. Notably, corneal crystals are not required for the diagnosis of SCD. The disease has been localized to the UBIAD1 gene on chromosome 1p36.3, which is involved in cholesterol metabolism. The pathophysiology of SCD is thought to involve a localized defect of lipid metabolism. Systemic control of cholesterol has been shown not to alter the progression of corneal disease. SCD is a slowly progressive disease, with onset as early as the first decade of life. Main symptoms include glare and reduced vision worse in photopic conditions.
Treatment, Management:
Mydriatics may allow patients to see around the central corneal crystals, as SCD disproportionately affects photopic vision. This patient was treated with 0.5% tropicamide and low power reading glasses at her last visit, with no subjective improvement. Other options for treatment are surgical and include PTK to remove corneal crystals, and PKP or DALK for patients with significant glare or reduced vision. However, corneal findings have been shown to recur after surgery. It is important for patients to understand that surgery will not eliminate arcus, as the peripheral cornea is not replaced in a transplant. A larger button increases the risk of graft rejection and irregular astigmatism, potentially requiring wear of GP or scleral lenses for optimal vision after surgery. Due to this patient's young age and good BCVA, she will continue to be monitored, rather than pursue surgical intervention at this time. Punctal plugs were inserted at her visit to address her complaint of dryness.
Conclusion:
Schnyder Corneal Dystrophy occurs as a localized defect of lipid metabolism, not necessarily systemic disease. Unfortunately, there is no known treatment to stop progression of this condition. The visual symptoms of SCD can be managed with PTK or corneal transplantation, although dystrophy can recur after surgery.
A Case of Spontaneous Carotid Cavernous Fistula with Ocular Complications
Published 2023 by Lilian Thoi
Co-Author(s): William Thibodeaux
Program Number: 235350
Article Type: Scientific Program
Board: 66
PURPOSE:
A carotid cavernous fistula (CCF) is a condition characterized by an abnormal communication between the cavernous sinus and internal carotid artery and can lead to various ocular complications. The purpose of this poster is to detail the clinical presentation as well as the neuroimaging studies needed to diagnose this condition. This case is unique in that the diagnosis was confirmed with catheter angiography, which was ordered after magnetic resonance angiography (MRA) and magnetic resonance imaging (MRI) studies were deemed unremarkable.
CASE REPORT:
A 79-year-old female presented with an abducens nerve palsy, conjunctival chemosis, dilated corkscrew conjunctival vessels, and elevated intraocular pressure of the left eye in the absence of trauma. Though the patient's presentation was highly suggestive of a left CCF, MRA and MRI were unremarkable, and the diagnosis was only made with catheter angiography. The patient subsequently underwent embolization of the carotid cavernous fistula with a direct approach via the superior ophthalmic vein. All associated ocular conditions resolved within three months following the embolization surgery.
CONCLUSION:
CCFs are rare and can cause severe ocular complications. When patients present with ocular signs and symptoms that are highly suggestive of this condition and more accessible neuroimaging studies such as computed tomography (CT), computer tomography angiography (CTA), MRI, and MRA yield normal findings, further investigation is warranted. In this particular case, traditional catheter angiography was needed in order to diagnose this condition and initiate treatment, which led to resolution of the patient's ocular complications.
PURPOSE:
A carotid cavernous fistula (CCF) is a condition characterized by an abnormal communication between the cavernous sinus and internal carotid artery and can lead to various ocular complications. The purpose of this poster is to detail the clinical presentation as well as the neuroimaging studies needed to diagnose this condition. This case is unique in that the diagnosis was confirmed with catheter angiography, which was ordered after magnetic resonance angiography (MRA) and magnetic resonance imaging (MRI) studies were deemed unremarkable.
CASE REPORT:
A 79-year-old female presented with an abducens nerve palsy, conjunctival chemosis, dilated corkscrew conjunctival vessels, and elevated intraocular pressure of the left eye in the absence of trauma. Though the patient's presentation was highly suggestive of a left CCF, MRA and MRI were unremarkable, and the diagnosis was only made with catheter angiography. The patient subsequently underwent embolization of the carotid cavernous fistula with a direct approach via the superior ophthalmic vein. All associated ocular conditions resolved within three months following the embolization surgery.
CONCLUSION:
CCFs are rare and can cause severe ocular complications. When patients present with ocular signs and symptoms that are highly suggestive of this condition and more accessible neuroimaging studies such as computed tomography (CT), computer tomography angiography (CTA), MRI, and MRA yield normal findings, further investigation is warranted. In this particular case, traditional catheter angiography was needed in order to diagnose this condition and initiate treatment, which led to resolution of the patient's ocular complications.
A Case of an Enigmatic Amelanotic Lesion
Published 2023 by Christina Tian
Program Number: R2023198
Article Type: Residents Day
Board: R-182
Purpose:
Focal scleral nodule is a benign lesion that may be easily confused for inflammatory conditions or intraocular masses. This case presents the clinical features, imaging, and lab testing that leads to the diagnosis of FSN.
Case History:
`- 53-year-old Hispanic male - CC: Constant blurry vision at distance and near OU, worsening for the past 4-5 years. The patient did not wear glasses but had worn contact lens in the past. His last eye exam was over 10 years ago - POH: refractive error OU, h/o orbital fracture OS after being hit by soccer ball in 1994 - PMH: diabetes type 2, obstructive sleep apnea - Medications: Metformin, Empagliflozin, CPAP
Pertinent Findings:
`- Entering DVAs OD 20/25-2, OS 20/30 - BCVA OD/OS 20/20 - Pupils, EOMs, CVF normal in both eyes - Normative IOPs OU - Anterior segment unremarkable, (-) cells/flare - Posterior segment evaluation/DFE OD: lattice degeneration superior far periphery OS: 0.5 DD round yellowish-white lesion 3DD inferior to the optic nerve head with no signs of vitritis, lipofuscin or fluid; chorioretinal scar inferior temporal periphery - EDI-OCT over the lesion OS Elevated, dome-shaped lesion arising from the sclera with thinning of overlying choroid, (-) subretinal fluid - Fundus Autofluorescence OS: hyperfluorescent round lesion inferior to ONH - Lab Testing CBC: normal, Quantiferon-TB: negative, RPR/ FTA-ABS: negative, ACE: normal
Differential Diagnosis:
Focal scleral nodule, amelanotic choroidal nevus, choroidal metastasis, choroidal melanoma, sclerochoroidal calcification, choroidal osteoma, choroidal granuloma
Diagnosis & Discussion:
Focal Scleral Nodule (FSN) is a rare idiopathic condition that presents with a unilateral, round, yellow-white, elevated, benign lesion arising from the sclera and is often found posterior to the equator. Previously termed unifocal helioid choroiditis and solitary idiopathic choroiditis, these lesions are now called focal scleral nodules due to its scleral origin and absence of choroiditis. Diagnosis of FSN is based on a detailed case history, clinical features, multimodal imaging, and laboratory testing. OCT imaging reveals a dome, nodular, or volcanic shaped lesion that is confined to the sclera and an absent or thin overlying choroid. Fundus autofluorescence shows hyperautofluorescence of the lesion. Laboratory testing such as Quantiferon-TB Gold, ACE, VDRL, RPR, FTA-ABS, and a chest x-ray should be ordered to rule out inflammatory etiologies. The majority of FSN lesions are inactive, and therefore patients are typically asymptomatic.
Treatment, Management:
Patients with asymptomatic, inactive FSN lesions can be monitored regularly with a routine eye exam. However, active lesions or those that are close to the macula or optic nerve should be observed more closely and may be treated with systemic corticosteroids.
Conclusion:
Focal Scleral Nodule is a diagnosis of exclusion. A thorough ocular exam, multimodal imaging, and lab testing should be performed to rule out potentially life-threatening inflammatory conditions or intraocular tumors.
Purpose:
Focal scleral nodule is a benign lesion that may be easily confused for inflammatory conditions or intraocular masses. This case presents the clinical features, imaging, and lab testing that leads to the diagnosis of FSN.
Case History:
`- 53-year-old Hispanic male - CC: Constant blurry vision at distance and near OU, worsening for the past 4-5 years. The patient did not wear glasses but had worn contact lens in the past. His last eye exam was over 10 years ago - POH: refractive error OU, h/o orbital fracture OS after being hit by soccer ball in 1994 - PMH: diabetes type 2, obstructive sleep apnea - Medications: Metformin, Empagliflozin, CPAP
Pertinent Findings:
`- Entering DVAs OD 20/25-2, OS 20/30 - BCVA OD/OS 20/20 - Pupils, EOMs, CVF normal in both eyes - Normative IOPs OU - Anterior segment unremarkable, (-) cells/flare - Posterior segment evaluation/DFE OD: lattice degeneration superior far periphery OS: 0.5 DD round yellowish-white lesion 3DD inferior to the optic nerve head with no signs of vitritis, lipofuscin or fluid; chorioretinal scar inferior temporal periphery - EDI-OCT over the lesion OS Elevated, dome-shaped lesion arising from the sclera with thinning of overlying choroid, (-) subretinal fluid - Fundus Autofluorescence OS: hyperfluorescent round lesion inferior to ONH - Lab Testing CBC: normal, Quantiferon-TB: negative, RPR/ FTA-ABS: negative, ACE: normal
Differential Diagnosis:
Focal scleral nodule, amelanotic choroidal nevus, choroidal metastasis, choroidal melanoma, sclerochoroidal calcification, choroidal osteoma, choroidal granuloma
Diagnosis & Discussion:
Focal Scleral Nodule (FSN) is a rare idiopathic condition that presents with a unilateral, round, yellow-white, elevated, benign lesion arising from the sclera and is often found posterior to the equator. Previously termed unifocal helioid choroiditis and solitary idiopathic choroiditis, these lesions are now called focal scleral nodules due to its scleral origin and absence of choroiditis. Diagnosis of FSN is based on a detailed case history, clinical features, multimodal imaging, and laboratory testing. OCT imaging reveals a dome, nodular, or volcanic shaped lesion that is confined to the sclera and an absent or thin overlying choroid. Fundus autofluorescence shows hyperautofluorescence of the lesion. Laboratory testing such as Quantiferon-TB Gold, ACE, VDRL, RPR, FTA-ABS, and a chest x-ray should be ordered to rule out inflammatory etiologies. The majority of FSN lesions are inactive, and therefore patients are typically asymptomatic.
Treatment, Management:
Patients with asymptomatic, inactive FSN lesions can be monitored regularly with a routine eye exam. However, active lesions or those that are close to the macula or optic nerve should be observed more closely and may be treated with systemic corticosteroids.
Conclusion:
Focal Scleral Nodule is a diagnosis of exclusion. A thorough ocular exam, multimodal imaging, and lab testing should be performed to rule out potentially life-threatening inflammatory conditions or intraocular tumors.
A Classic Case of Vogt-Koyanagi-Harada Syndrome in a Young Asian Female Presenting with Bilateral Exudative Retinal Detachments
Published 2023 by Graham Chung
Program Number: R2023158
Article Type: Residents Day
Board: R-142
Purpose:
A young Asian female presents with bilateral exudative retinal detachments and dizziness associated with onset of blurred vision, which improved after systemic steroid treatment. Vogt-Koyanagi-Harada should be considered even in the absence of cutaneous signs.
Case History:
30-year-old Asian female CC: Referred for retinal evaluation by outside optometrist for macular edema. Patient reports significant intermittent blurry vision in both eyes and feeling dizzy for about one week, not associated with any activity or time of day. POH/PMH/Meds: None
Pertinent Findings:
Clinical Uncorrected Visual Acuity: 20/100 OD, 20/80 OS; no improvement with refraction Unremarkable anterior segment Intraocular Pressure: 15/12 with iCare Dilated Fundus Examination: choroidal folds through the macula with subretinal edema along the superior and inferior arcades and pigmented cells in posterior vitreous OU Ancillary Testing Macula OCT: subretinal fluid in macular region and along arcades OU OCT-Angiography: no blood flow through subretinal spaces
Differential Diagnosis:
Vogt-Koyanagi-Harada syndrome Lymphoma Lyme Disease Sarcoidosis Lupus choroidopathy Posterior scleritis Cat scratch disease Leukemia Metastatic Carcinoma Central serous chorioretinopathy
Diagnosis & Discussion:
Vogt–Koyanagi–Harada syndrome (VKHS) is a rare multisystemic autoimmune disease that affects tissues containing melanin, including the eye, inner ear, meninges, and skin. The disease is primarily characterized by bilateral uveitis associated with varying combinations of auditory, neurological and cutaneous manifestations. The demographic that is most affected are Asians, Native Americans, and Hispanics. It affects females more than males and generally affects patients between the ages of 20 to 50-years-old. The classic clinical course is characterized by bilateral panuveitis, hypoacusis, and meningitis, in addition to cutaneous involvement with poliosis, vitiligo, and alopecia. Although the exact cause of VKH disease remains unknown, it is thought to be a T-cell-mediated autoimmune process directed against melanocytes1. With the exception of cutaneous complications, this patient had the classic presentation of bilateral uveitis with exudative retinal detachments and vertigo.
Treatment, Management:
This patient was seen and treated by a retinal specialist who started her on 16 mg of methylprednisolone twice a day and followed up in four days. Since signs and symptoms improved at the second visit, methylprednisolone treatment was replaced with 40 mg of prednisolone daily for one week and then tapered to 20 mg daily the second week. Prednisolone dosage continued to be decreased by half every two weeks as improvement was seen at each follow-up. After three months of treatment, all corticosteroids were discontinued. Early and aggressive treatment of oral or IV corticosteroids are the initial therapy for patients with VKH, as studies have shown better prognosis2. A slow steroid taper of up to six months is recommended to reduce chances of recurrence and other complications3. Current studies include non-steroidal immunomodulatory therapy as a valid second line of treatment, since recurrence (while on steroids) and steroid associated complications have been known to occur2.
Conclusion:
Although we do not know the exact pathophysiology of VKH, it should remain on our list of differentials whenever a patient presents with bilateral posterior uveitis or bilateral serous detachments. Treatment with oral corticosteroids should be initiated early and aggressively to reduce risk of recurrence and complications.
Purpose:
A young Asian female presents with bilateral exudative retinal detachments and dizziness associated with onset of blurred vision, which improved after systemic steroid treatment. Vogt-Koyanagi-Harada should be considered even in the absence of cutaneous signs.
Case History:
30-year-old Asian female CC: Referred for retinal evaluation by outside optometrist for macular edema. Patient reports significant intermittent blurry vision in both eyes and feeling dizzy for about one week, not associated with any activity or time of day. POH/PMH/Meds: None
Pertinent Findings:
Clinical Uncorrected Visual Acuity: 20/100 OD, 20/80 OS; no improvement with refraction Unremarkable anterior segment Intraocular Pressure: 15/12 with iCare Dilated Fundus Examination: choroidal folds through the macula with subretinal edema along the superior and inferior arcades and pigmented cells in posterior vitreous OU Ancillary Testing Macula OCT: subretinal fluid in macular region and along arcades OU OCT-Angiography: no blood flow through subretinal spaces
Differential Diagnosis:
Vogt-Koyanagi-Harada syndrome Lymphoma Lyme Disease Sarcoidosis Lupus choroidopathy Posterior scleritis Cat scratch disease Leukemia Metastatic Carcinoma Central serous chorioretinopathy
Diagnosis & Discussion:
Vogt–Koyanagi–Harada syndrome (VKHS) is a rare multisystemic autoimmune disease that affects tissues containing melanin, including the eye, inner ear, meninges, and skin. The disease is primarily characterized by bilateral uveitis associated with varying combinations of auditory, neurological and cutaneous manifestations. The demographic that is most affected are Asians, Native Americans, and Hispanics. It affects females more than males and generally affects patients between the ages of 20 to 50-years-old. The classic clinical course is characterized by bilateral panuveitis, hypoacusis, and meningitis, in addition to cutaneous involvement with poliosis, vitiligo, and alopecia. Although the exact cause of VKH disease remains unknown, it is thought to be a T-cell-mediated autoimmune process directed against melanocytes1. With the exception of cutaneous complications, this patient had the classic presentation of bilateral uveitis with exudative retinal detachments and vertigo.
Treatment, Management:
This patient was seen and treated by a retinal specialist who started her on 16 mg of methylprednisolone twice a day and followed up in four days. Since signs and symptoms improved at the second visit, methylprednisolone treatment was replaced with 40 mg of prednisolone daily for one week and then tapered to 20 mg daily the second week. Prednisolone dosage continued to be decreased by half every two weeks as improvement was seen at each follow-up. After three months of treatment, all corticosteroids were discontinued. Early and aggressive treatment of oral or IV corticosteroids are the initial therapy for patients with VKH, as studies have shown better prognosis2. A slow steroid taper of up to six months is recommended to reduce chances of recurrence and other complications3. Current studies include non-steroidal immunomodulatory therapy as a valid second line of treatment, since recurrence (while on steroids) and steroid associated complications have been known to occur2.
Conclusion:
Although we do not know the exact pathophysiology of VKH, it should remain on our list of differentials whenever a patient presents with bilateral posterior uveitis or bilateral serous detachments. Treatment with oral corticosteroids should be initiated early and aggressively to reduce risk of recurrence and complications.
A Combined Case of IIH (Idiopathic Intracranial Hypertension) and MOG (Myelin Oligodendrocyte Glycoprotein) Optic Neuritis
Published 2023 by Amina Cheema
Co-Author(s): Jaymeni Patel, Leonard Messner
Program Number: 235370
Article Type: Scientific Program
Board: 86
PURPOSE:
Myelin Oligodendrocyte Glycoprotein (MOG) Optic Neuritis is an antibody-mediated demyelination of the central nervous system. Clinical presentations include acute, unilateral optic neuritis along with reduced color vision, a relative afferent pupillary defect, pain on eye movement, and reduced visual acuity. Idiopathic Intracranial Hypertension (IIH) is a condition which can lead to bilateral papilledema and optic atrophy due to increased intracranial pressure. IIH is a condition of exclusion that presents in obese females of childbearing age. The purpose of this case presentation is to review signs, symptoms, and management for both conditions as one can confound the other in clinical presentation.
CASE REPORT:
A 27-year-old female presented to the Urgent Care with eyelid soreness, pain of eye movement, and occasional headaches. Clinical findings were pertinent for mildly indistinct margins consistent with IIH. At neuro-ophthalmic consultation five days later, the patient had reduced vision OS and reduced color vision OS. The dilated fundus exam and OCT showed a marked increase in disc edema OS>OD. The patient's MRI showed flattening of the posterior orbit, mild asymmetric T2 hyperintensity enchantment OS, and mild narrowing of the deeper Dural sinuses at the junction of the transverse and sigmoid sinus. The bloodwork was positive for MOG antibodies and the opening pressure on the lumbar puncture was 27 cmH20. The patient was diagnosed with MOG optic neuritis and papilledema owing to IIH. The patient was treated with intravenous (IV) methylprednisolone followed by an oral taper.
CONCLUSION:
Treatment for IIH varies from the treatment of MOG optic neuritis. A variety of treatment options can be used for IIH, such as lifestyle changes, oral medications, or surgical intervention based on the condition's severity. Lifestyle changes are focused on weight management while oral medications may include acetazolamide or topiramate. Neurosurgical intervention is considered for cases recalcitrant to medical management. Initial treatment for MOG optic neuritis includes IV steroids such as methylprednisolone. With recurrent cases of optic neuritis, other treatments may be considered such as immune-modulating agents. This patient presented with IIH OU with an acute ON OS. The patient has been monitored closely for the last year and has not had recurrences. Vision remains at 20/20 in each eye, ganglion cell thickness remains unchanged, and the visual field is full in each eye. Color vision, dilated fundus examination, OCT, and HVF testing should be monitored closely at follow-up visits.
PURPOSE:
Myelin Oligodendrocyte Glycoprotein (MOG) Optic Neuritis is an antibody-mediated demyelination of the central nervous system. Clinical presentations include acute, unilateral optic neuritis along with reduced color vision, a relative afferent pupillary defect, pain on eye movement, and reduced visual acuity. Idiopathic Intracranial Hypertension (IIH) is a condition which can lead to bilateral papilledema and optic atrophy due to increased intracranial pressure. IIH is a condition of exclusion that presents in obese females of childbearing age. The purpose of this case presentation is to review signs, symptoms, and management for both conditions as one can confound the other in clinical presentation.
CASE REPORT:
A 27-year-old female presented to the Urgent Care with eyelid soreness, pain of eye movement, and occasional headaches. Clinical findings were pertinent for mildly indistinct margins consistent with IIH. At neuro-ophthalmic consultation five days later, the patient had reduced vision OS and reduced color vision OS. The dilated fundus exam and OCT showed a marked increase in disc edema OS>OD. The patient's MRI showed flattening of the posterior orbit, mild asymmetric T2 hyperintensity enchantment OS, and mild narrowing of the deeper Dural sinuses at the junction of the transverse and sigmoid sinus. The bloodwork was positive for MOG antibodies and the opening pressure on the lumbar puncture was 27 cmH20. The patient was diagnosed with MOG optic neuritis and papilledema owing to IIH. The patient was treated with intravenous (IV) methylprednisolone followed by an oral taper.
CONCLUSION:
Treatment for IIH varies from the treatment of MOG optic neuritis. A variety of treatment options can be used for IIH, such as lifestyle changes, oral medications, or surgical intervention based on the condition's severity. Lifestyle changes are focused on weight management while oral medications may include acetazolamide or topiramate. Neurosurgical intervention is considered for cases recalcitrant to medical management. Initial treatment for MOG optic neuritis includes IV steroids such as methylprednisolone. With recurrent cases of optic neuritis, other treatments may be considered such as immune-modulating agents. This patient presented with IIH OU with an acute ON OS. The patient has been monitored closely for the last year and has not had recurrences. Vision remains at 20/20 in each eye, ganglion cell thickness remains unchanged, and the visual field is full in each eye. Color vision, dilated fundus examination, OCT, and HVF testing should be monitored closely at follow-up visits.
A Comparison of Foveal Avascular Zone Dimensions in Glaucomatous and Non-Glaucomatous Eyes
Published 2023 by Blake Henson
Co-Author(s): Blake Henson
Program Number: 235261
Article Type: Scientific Program
Board: 247
PURPOSE:
The foveal avascular zone (FAZ) is a region within the macula that is devoid of retinal vasculature allowing for optimal optical quality. Optical Coherence Tomography Angiography (OCT-A) is a non-invasive imaging technology that allows for the visualization of microvasculature of the retina and choroid that surrounds the FAZ. In this study, the dimensions and area of the FAZ were measured in subjects with normal tension glaucoma (NTG) and primary open angle glaucoma (POAG) as well as normal subjects.
METHOD:
The Zeiss Cirrus OCT-A was used to perform a 3x3 mm scan of the FAZ of glaucomatous and non-glaucomatous eyes. Horizontal, vertical, and diagonal dimensions of the FAZ were obtained using the polygonal selection tool on the Cirrus OCT. The area of the FAZ was analyzed using Image J software. Twenty-one subjects were recruited for the study. Eight eyes were classified as having NTG, 15 eyes were classified as having POAG, and 11 eyes were normal.
RESULTS:
The mean horizontal diameter was 530.182 µm for normal eyes, 599.231 µm for POAG, and 656.500 µm for NTG (p=0.177). The mean vertical diameter was 498.727 µm for normal eyes, 542.231 µm for POAG, and 610.125 µm for NTG (p=0.114) while the mean diagonal diameter was 559.455 µm for normal eyes, 624.154 µm for POAG, and 679.625 µm for NTG (p=0.136). The average area of the FAZ was 0.171 mm2 in normal eyes, 0.246 mm2 in those with POAG, and 0.321 mm2 in those with NTG (p=0.018). There was no statistically significant difference between FAZ area in normal vs. POAG (p=0.30) and POAG vs. NTG (p=0.38). However, there was a statistically significant difference in FAZ area between normal subjects and those with NTG (p=0.016).
CONCLUSION:
In this study, no statistically significant difference was found in FAZ dimensions between normal subjects and those diagnosed with POAG or NTG. It was determined that FAZ area was statistically significantly larger in subjects diagnosed with NTG when compared to normal subjects (p=0.016). The use of OCT-A to quantify FAZ area may have future implications for diagnosing NTG.
PURPOSE:
The foveal avascular zone (FAZ) is a region within the macula that is devoid of retinal vasculature allowing for optimal optical quality. Optical Coherence Tomography Angiography (OCT-A) is a non-invasive imaging technology that allows for the visualization of microvasculature of the retina and choroid that surrounds the FAZ. In this study, the dimensions and area of the FAZ were measured in subjects with normal tension glaucoma (NTG) and primary open angle glaucoma (POAG) as well as normal subjects.
METHOD:
The Zeiss Cirrus OCT-A was used to perform a 3x3 mm scan of the FAZ of glaucomatous and non-glaucomatous eyes. Horizontal, vertical, and diagonal dimensions of the FAZ were obtained using the polygonal selection tool on the Cirrus OCT. The area of the FAZ was analyzed using Image J software. Twenty-one subjects were recruited for the study. Eight eyes were classified as having NTG, 15 eyes were classified as having POAG, and 11 eyes were normal.
RESULTS:
The mean horizontal diameter was 530.182 µm for normal eyes, 599.231 µm for POAG, and 656.500 µm for NTG (p=0.177). The mean vertical diameter was 498.727 µm for normal eyes, 542.231 µm for POAG, and 610.125 µm for NTG (p=0.114) while the mean diagonal diameter was 559.455 µm for normal eyes, 624.154 µm for POAG, and 679.625 µm for NTG (p=0.136). The average area of the FAZ was 0.171 mm2 in normal eyes, 0.246 mm2 in those with POAG, and 0.321 mm2 in those with NTG (p=0.018). There was no statistically significant difference between FAZ area in normal vs. POAG (p=0.30) and POAG vs. NTG (p=0.38). However, there was a statistically significant difference in FAZ area between normal subjects and those with NTG (p=0.016).
CONCLUSION:
In this study, no statistically significant difference was found in FAZ dimensions between normal subjects and those diagnosed with POAG or NTG. It was determined that FAZ area was statistically significantly larger in subjects diagnosed with NTG when compared to normal subjects (p=0.016). The use of OCT-A to quantify FAZ area may have future implications for diagnosing NTG.
