Title: Central Serous Chorioretinopathy
Author: Sravanthi Vegunta, Eileen S. Hwang, Mary E. Hartnett
Photographer: Mel Chandler
Keywords/Main Subjects: Central serous chorioretinopathy; Scotoma; Metamorphopsia; Retinopathy; Corticosteroid use; Smoke stack
Diagnosis: Central serous chorioretinopathy
Brief Description: History of Present Illness: The patient was a 46 year old female with a history of hypothyroidism, depression and seasonal allergies who presented 5 weeks after the sudden onset of a floaters, a paracentral scotoma and distortion in her left eye. At first, her central vision was primarily affected, but shortly before her presentation in our clinic, the affected area spread to include an area to the right as (Figure 1). She denied visual problems with her right eye.
Her medical history was significant for the use of intranasal steroid spray for seasonal allergies. She had stopped this a few weeks prior to presentation after a visit to an outside ophthalmologist.
Initial examination: The patient’s visual acuity with correction was 20/20 in the right eye and 20/60 in the left eye. Intraocular pressure was 16 mmHg in the right eye and 16 mmHg in the left eye. She did not have a relative afferent pupillary defect. Confrontation visual fields and extraocular motility were intact. Anterior segment slit lamp examination was normal in both eyes. On fundus examination, both optic nerves were normal in appearance with a cup-to-disc ratio of 0.3 in the right eye and 0.2 in the left eye. The macula of her right eye was normal with a good foveal reflex. Her left macula had an area of pigment near the fovea (Figure 2) and the retina appeared elevated in the central macula, consistent with subretinal fluid (Figure 3). The retinal vasculature and periphery were normal in both eyes.
Clinical Course: Patient underwent optical coherence tomography (OCT; Figures 3 and 4) and fluorescein angiography (FA; Figure 6) of each eye.
The patient was diagnosed with central serous chorioretinopathy (CSCR) and informed that her visual changes were likely to improve without intervention. Information was provided to the patient regarding the limited quality data for treatment of CSCR with spironolactone, rifampin, epleronone, and low dose aspirin. Low dose aspirin was recommended to the patient since there was a controlled study supporting its use, and the risks were felt to be relatively benign.
CSCR is characterized by a build up of subretinal fluid in the macula caused by abnormalities of the choroidal circulation.1 Fluid leaks from the choroidal circulation and passes through hyperpermeable areas of the retinal pigment epithelium (RPE), accumulating in the subretinal space. 2
While the specific cause of CSCR is not well understood, some systemic risk factors have been associated with CSCR. In particular, corticosteroid administration and stressful events (presumably associated with high endogenous corticosteroid levels) are associated with the development of CSCR.2 The patient described in this case report was using intranasal steroids, which have specifically been associated with CSCR in addition to systemic, intraarticular, and topical steroids.
CSCR occurs most often in males between 20 and 50 years of age. It usually presents with acute onset of unilateral metamorphopsia, blurred vision and a relative scotoma. Patients can also experience micropsia, impaired dark adaptation, and color desaturation. Presenting visual acuity ranges from 20/15 to 20/200 with an average of 20/30. On fundus examination, there is often an elevation of the neurosensory retina in the central macula due to subretinal fluid. It is important to perform careful inspection of the fundus to exclude conditions such as optic nerve pit, age-related macular degeneration, polypoidal choroidal vasculopathy, uveitis, and intraocular tumor which can also cause subretinal fluid. OCT can be used to identify and follow the amount of subretinal fluid.1, 2 FA reveals leakage of dye from a focal choroidal/RPE defect which collects in the subretinal space. This usually occurs in an ink-blot pattern in which the spot of hyperfluorescence secondary to leakage spreads concentrically, but in 10-15% of cases, the dye can spread linearly in a smoke stack configuration, as demonstrated in the imaging from the patient described here.
Visual disturbances often resolve spontaneously in a few months, although recurrence is common and chronic CSCR can occur. Some patients experience permanent visual changes. If the subretinal fluid fails to resolve with observation alone, photodynamic therapy (PDT) may be performed to hasten subretinal fluid resorption. There is no evidence that PDT improves final visual outcomes, but since photoreceptor atrophy begins as early as 4 months after onset, there may be a theoretical a long term benefit to treatment if the fluid persists for more than 3 months. However, PDT is not without significant risk of vision loss and secondary CNV. The use of half-dose verteporfin and a 10 minute time interval between infusion and laser application may reduce these risks while still providing efficacious treatment of the abnormal choroidal vasculature.
In additional to PDT, several other treatment options for CSCR have been studied. However, studies with large sample sizes and prospective, controlled design are lacking. Studies of the efficacy of bevacizumab and ranibizumab therapies have shown significantly improved retinal thickness and visual acuity in the first 3 months following treatment, but this improvement is not sustained beyond 6 months.3-7
Medications that inhibit mineralocorticoid production or antagonize the mineralocorticoid receptor have recently been studied as potential treatments for CSCR. Zhao et al.8 demonstrated efficacy of eplerenone in a CSCR animal model, and a small pilot study of 13 human patients showed promise.9. There are a handful of ongoing clinical trials for eplerenone, which is an FDA-approved medication used in the treatment of congestive heart failure.
Caccavale et al.10 studied the efficacy of aspirin for CSCR treatment with the rationale that aspirin may correct abnormalities of the choroidal vasculature and reduce serum levels of plasminogen activator inhibitor 1 (PAI-1), elevated levels of which have been found in patients with CSCR.11, 12 Caccavale et al. conducted a prospective trial in which patients treated with six months of 100 mg aspirin demonstrated improved visual acuity compared to historic controls. Significant improvements in visual acuity were present at one month and three months, and persisted to twelve months.
In the case of the patient described in this case report, she was eagerly seeking some kind of treatment. PDT was not recommended because at one month after onset, the risk of vision loss from PDT was not justified in a disease with a 90% spontaneous resolution rate. Eplerenone was considered, but excluded because of the need for monitoring serum potassium. The patient was started on low dose aspirin since there was some evidence supporting its efficacy, and the risks of this treatment were relatively low.
Series: Case Report 2015
- Kanski J, Bowling B. Clinical Ophthalmology, 7 ed. United Kingdom: Elsevier Health Sciences, 2011.
- Ryan S, Schachat A, Wilkinson C, Hinton D, Sadda S, Wiedemann P. Retina, 5 ed. United Kingdom: Elsevier Health Sciences, 2013.
- A randomized trial comparing intravitreal triamcinolone acetonide and focal/grid photocoagulation for diabetic macular edema. Ophthalmology 2008;2008 Sep;115:1447-9.
- Aydin E. The efficacy of intravitreal bevacizumab for acute central serous chorioretinopathy. J Ocul Pharmacol Ther 2013;29:10-3. doi: 10.1089/jop.2012.0072. Epub 2012 Aug 27.
- Lim JW, Ryu SJ, Shin MC. The effect of intravitreal bevacizumab in patients with acute central serous chorioretinopathy. Korean J Ophthalmol 2010;24:155-8. doi: 10.3341/kjo.2010.24.3.155. Epub 2010 Jun 5.
- Artunay O, Yuzbasioglu E, Rasier R, Sengul A, Bahcecioglu H. Intravitreal bevacizumab in treatment of idiopathic persistent central serous chorioretinopathy: a prospective, controlled clinical study. Curr Eye Res 2010;35:91-8. doi: 10.3109/02713680903428306.
- Park SU, Lee SJ, Kim M. Intravitreal anti-vascular endothelial growth factor versus observation in acute central serous chorioretinopathy: one-year results. Korean J Ophthalmol 2014;28:306-13. doi: 10.3341/kjo.2014.28.4.306. Epub 2014 Jul 22.
- Zhao M, Celerier I, Bousquet E, et al. Mineralocorticoid receptor is involved in rat and human ocular chorioretinopathy. J Clin Invest 2012;122:2672-9. doi: 10.1172/JCI61427. Epub 2012 Jun 11.
- Bousquet E, Beydoun T, Zhao M, Hassan L, Offret O, Behar-Cohen F. Mineralocorticoid receptor antagonism in the treatment of chronic central serous chorioretinopathy: a pilot study. Retina 2013;33:2096-102.
- Caccavale A, Romanazzi F, Imparato M, Negri A, Morano A, Ferentini F. Low-dose aspirin as treatment for central serous chorioretinopathy. Clin Ophthalmol 2010;4:899-903.
- Yamada R, Yamada S, Ishii A, Tane S. [Evaluation of tissue plasminogen activator and plasminogen activator inhibitor-1 in blood obtained from patients of idiopathic central serous chorioretinopathy]. Nihon Ganka Gakkai Zasshi 1993;97:955-60.
- Iijima H, Iida T, Murayama K, Imai M, Gohdo T. Plasminogen activator inhibitor 1 in central serous chorioretinopathy. Am J Ophthalmol 1999;127:477-8.
Title: The Not So Incidental Finding: A Cavernous Hemangioma
Authors: Christopher D. Conrady, MD, PhD, Jun Guan, MD, H. Christian Davidson, MD, and Bhupendra Patel, MD
Keywords/Main Subjects: Cavernous hemangioma; Orbit; Orbital mass; Proptosis
Secondary CORE Category: Orbit, Eyelids and lacrimal System / Orbital Neoplasms and Malformations / Vascular Tumors, Malformations, and Fistulas
Diagnosis/Differential Diagnosis: cavernous hemangioma
Description: Thirty-one-year-old female with past medical history significant for a traumatic brain injury, von Willebrand disease, and migraines, that presented to an outside ophthalmologist with recurrent headaches and “sparkles within her vision.”. She was noted to have a normal exam, including dilated fundus exam, with one exception, mild disk edema of the left optic nerve. Humphrey visual fields were normal. The patient was referred for imaging due to a change in headaches and associated visual symptoms not typical of prior migraines. MRI of the orbits identified a well-circumscribed mass within the left orbit abutting the globe (Figure 1).
The patient was referred to the oculoplastics service at the Moran for further evaluation and treatment due to concern of optic nerve compression with edema on exam. She was found to 20/15 vision, pupils were equal and reactive without relative afferent defect, extraocular motility full, and color vision was normal OU. However, there was noticeable periorbital asymmetry with increased fullness of the left lateral orbit compared to right and left-sided proptosis of 1.5 mm.
Due to concern of optic nerve changes, the patient underwent an anterior orbitotomy with complete resection of the orbital mass without complications. Histopathological specimens were consistent with an orbital cavernous hemangioma with a pseudocapsule and large vascular spaces lined with endothelium (Figure 2). The patient made a quick and full recovery with a resolution of symptoms.
Figure 1: Well-circumscribed left orbital mass on MRI of the orbits. (a) Axial MRI of the orbits with contrast with large, well-circumscribed, enhancing lesion of the left orbit. (b) Time course of enhancement showing fill-in of lesion. Radiographic findings are typical for cavernous hemangioma.
Figure 2: Cavernous hemangioma on histopathology. (a) Gross surgical specimen of the cavernous hemangioma. H&E staining was then performed for further evaluation with (b) 20x, (c) 40x, and (d) 100x images. Pseudocapsule and vascular spaces lined with endothelium are noted.
Summary of Case:
- Consider cavernous hemangiomas in both men and women with painless, unilateral proptosis in the 3rd to 5th decade of life.
- Use imaging to help aide in diagnosis as carvernous hemangiomas are well-circumscribed intraconal masses of which 90% can be diagnosed pre-operatively.
- Multiple emerging ways to access orbital tumors that decrease post-operative morbidity.
- Hormones (i.e. pregnancy and menopause) may influence size of lesion but no known factors predictive of growth.
- Presenting symptoms: Eyeball protrusion (90%), visual impairment (65%), double vision (20%), local Pain (18%), headache (12%), eyelid fullness/swelling (5%).
- For more details on the case, please see associated PowerPoint Presentation.
Format: PowerPoint presentation
- Di Tomasso et al., Progesterone receptor expression in orbital cavernous hemangioma. Virchows Arch. 2000.
- Fries and Char, Bilateral orbital cavernous hemangioma. Br. J. Ophtho. 1988.
- Goldberg et al., Orbital Tumors Excision without bony marginotomy under local and general anesthesia. J. Ophtho. 2014.
- Hsu and Hsu, Cavernous Hemangioma of the Orbit: 42 patients. J. Exp and Clinc Med, 2011.
- Jayaram et al., Potential correlation between menopausal status and the clinical course of orbital cavernous hemagniomas. Optho Plast Recon Surg. 2015.
- Paluzzi et al., “Round-the-Clock” Surgical Access to the Orbit. J. Neurol Surg, 2015
- Som and Curtin., Head and neck imaging. Mosby. ISBN: 0323009425
Faculty Approval by: Dr. Bhupendra Patel, MD
Christopher D. Conrady, ©2015. For further information regarding the rights to this collection, please visit: http://morancore.utah.edu/terms-of-use/
Disclosure: The authors have no financial conflicts of interest.
Title: Case Report of Susac Syndrome
Author(s): Russell Swan MD, Rachael Jacoby MD
Secondary CORE Category: Neuro-ophthalmology / Causes of Decreased Vision / Vascular Disorders
Keywords / Main Subjects: Susac syndrome; Branch retinal arterial occlusion
Diagnosis: Susac syndrome
Differential: Vasculitis, Churg Strauss, SLE, Sarcoid, Behcets, Eales, Lyme, Syphilis, TB, viral encephalitis, primary CNS lymphoma, MELAS, isolated BRAO
Brief Description: A twenty-seven year old female presents with one week of blurry vision in left eye. She has also had two weeks of dizziness and confusion; she presented to an outside hospital with inability to perform activities of daily living and not oriented to date. She was then transferred for further care at our facility. MRI showed multiple subcortical lesions in multiple vascular territories most noticeably in the corpus collosum.
Our patient was treated with Cellcept and high dose steroids; at her follow up visit she was on Prednisone 40 mg per day and 1000 mg of Cellcept. She will be followed by neurology with serial MRIs and ophthalmology with repeat wide field fluorescein angiogram to monitor for recurrent vascular occlusions.
Further systemic evaluation included:
- TEE: no vegetation, right to left PFO
- Doppler of legs: no DVT
- CTA: normal without stenosis
- Cerebral angiogram: no stenosis or vasculitis
Labs: normal CBC, CMP, negative cardiolipin antibody, normal CRP, normal A1C, normal lupus anticoagulant, negative Factor V, Protein C/s, antithrombin III, normal homocysteine, ACE, ANCA, SSA/SSB, ANA, RF, ESR;
Lumbar puncture: normal, no oligoclonal bands.
Summary of the Case: Susac syndrome is an autoimmune disease first described in 1979 by Dr. John Susac. Most often occurs in females between the ages of 20 to 40 with a 3:1 female to male ratio. Susac syndrome typically causes visual field changes, hearing loss and vertigo, headaches with associated vomiting, confusion and cognitive difficulties. The pathophysiology is unknown at this time. Treatment includes steroids, immune modulating treatments such as cyclophosphamide, mycophenaloate, azathioprine and newer biologics, treatment can also include IVIG. The disease process is usually self-limited, lasting between 2 -4 years. The prognosis is varied, as some patients do quite well with limited treatment while others might have a recurrent disease process. Some patients develop long term cognitive deficits, gait disturbances as well as hearing and vision loss.
• Egan, R., Gass, J. et al. Retinal arterial wall plaques in Susac Syndrome. American Journal Ophthalmology 2003, April 135 (4) 483-486
• Susac, J. Susac’s Syndrome. American Journal of Neuroradiology 2004, 25:352-352
Fundus photos of right eye are normal, left eye shows retinal whitening in the distribution of the superotemporal artery
Early phase of the fluorescein angiogram in the left eye shows delayed arterial filling in the superotemporal artery
Follow up wide field FA three months later shows continued delayed arterial filling in the superotemporal artery branch
Late phase of the follow up angiogram showing vessel wall staining in the inferotemporal vessels.
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Title: Case Report of Non-Arteritic Ischemic Optic Neuropathy (NAION)
Author (s): Eileen Hwang, Judith Warner
Photographer (s): Mel Chandler, Cyrie Fry
Keywords/Main Subjects: NAION, non-arteritic ischemic optic neuropathy, visual field defect, optic disc edema, optic nerve edema, optic neuropathy, afferent pupillary defect
Diagnosis/Differential Diagnosis: non-arteritic ischemic optic neuropathy
Chief complaint: visual field defect
History of present illness: The patient is a 73-year-old woman who presented two days after sudden onset of vision changes. She described a gray film in the inferior area of the vision in her right eye that had not changed since onset. The patient denied having fevers, chills, weight loss, jaw claudication, shoulder stiffness and weakness. Her medical history was significant for hypertension, hyperlipidemia, and rheumatoid arthritis, and her medications included enalapril 20 mg twice a day, hydrochlorothiazide 10 mg daily in the morning, and aspirin 81 mg daily. She was in the habit of measuring her blood pressure daily and reported that it was consistently in the 130s/50s-60s. She denied feeling lightheaded when standing up. She denied snoring.
Initial examination: The patient’s visual acuity was 20/20 in each eye with pin hole and she did not have a relative afferent pupillary defect. She had mild elevation without edema of the superior optic disc in the right eye and a retinal nerve fiber layer hemorrhage nasal to the disc (Figure 1). Her cup-to-disc ratio in the right eye was 0.2. The cup-to-disc ratio in her left eye was 0.6. The patient was referred to glaucoma, and subsequently to neuro-ophthalmology.
Follow up examination in the neuro-ophthalmology clinic: Two weeks later, the patient’s visual acuity with pinhole was 20/20 in the right eye and 20/20 in the left eye. She had a 0.6 log unit right relative afferent pupillary defect. Her color vision was decreased on the right with 80% red desaturation and 11/13 Ishihara plates compared to 13/13 on the left. Her critical flicker fusion frequency was 39 Hz on the right and 41 Hz on the left. On slit lamp examination, there was 350 degrees of disc edema in the right eye with obscuration of the nasal vessels. There were also exudates on the nasal disc and a retinal nerve fiber layer hemorrhage nasal to the disc (Figure 2). On Humphrey visual field testing, there was an inferior arcuate defect in the right eye. The left eye visual field showed an inferior rim artifact (Figure 3).
Clinical course: The patient was diagnosed presumptively with non-arteritic ischemic optic neuropathy although other possibilities that were considered were a compressive mass lesion, central retinal vein occlusion, diabetic papillitis, hematological abnormalities (thrombocytosis, anemia), vasculitis, and hypertensive papillitis. Lab tests were performed to rule out giant cell arteritis, and causes of hypercoagulability such as thrombocytosis, polycythemia, and multiple myeloma. The results of these laboratory studies (erythrocyte sedimentation rate, c-reactive protein, complete blood count, and serum protein electrophoresis) were normal.
The patient decided to start brimonidine twice daily for neuro-protection after an informed discussion about the limited evidence supporting its use. In conjunction with her primary care doctor, she decreased her enalapril to once a day in the morning. Her diastolic blood pressure remained in the 50s-60s.
Four weeks after initial symptom onset, the patient reported that the visual field defect had suddenly extended to include part of her central vision. Her visual acuity was stable, but the magnitude of her right relative afferent pupillary defect increased to 0.9 log units and her color vision worsened to 5/13 Ishihara plates. On slit lamp examination, her optic nerve edema had almost completely resolved except for mild blurring of the margin inferiorly. Humphrey visual field testing demonstrated a new superior paracentral scotoma with improvement of the inferior altitudinal defect. Due to the unusual nature of her late progression and the lack of a “disc at risk” (i.e. small cup to disc ratio) in the contralateral eye, the decision was made to order cerebral imaging to rule out a compressive or infiltrative lesion.
Anterior ischemic optic neuropathy (AION) is characterized by rapid onset of unilateral vision loss associated with optic nerve edema. There are two types of anterior ischemic optic neuropathy – arteritic anterior ischemic optic neuropathy (AAION), and non-arteritic anterior ischemic optic neuropathy (NAION). To make a diagnosis of NAION, AAION should be sufficiently ruled out, which can be by a history negative for symptoms of temporal arteritis (headache, scalp tenderness, jaw claudication, fever, weight loss, and proximal joint pain) and young age (less than 50 years old), but also by laboratory testing or temporal artery biopsy, depending on the level of clinical suspicion. In the evaluation of a patient with acute onset of unilateral vision changes and optic nerve edema, optic neuritis must also be considered, and characteristics such as a younger patient age and pain with eye movements are suggestive of optic neuritis rather than NAION.
After acute onset, the vision loss usually remains static, although it can also progress in either a gradual or stepwise fashion for a few weeks before stabilizing. On examination, there is optic nerve edema that can be segmental and usually is not chalky white. Peripapillary hemorrhages are common. The optic nerve in the contralateral, unaffected eye usually has a small cup-to-disk ratio, and this is thought to contribute to risk of NAION because the crowding supports propagation of ischemia and swelling. Visual field defects due to NAION are most commonly altitudinal, although many other types of defects can be present as well.
Fluorescein angiography in patients with acute NAION demonstrates delayed filling of the prelaminar optic disc, supporting the role of anterior ischemia in the pathogenesis of NAION. Possible risk factors for NAION that may exacerbate optic nerve ischemia include nocturnal hypotension and sleep apnea. Recently, it has been demonstrated that erectile dysfunction medications increase the risk of NAION, although this is a very rare event (Campbell, 2015). After NAION has been diagnosed, interventions should focus on minimizing risk factors, since there are no acute interventions that have been shown to improve outcomes. There is poor evidence that brimonidine eye drops or oral low-dose aspirin can prevent future events in the contralateral eye (Kupersmith, 1997), but at our institution, brimonidine is usually started in both eyes for its neuroprotective effect. It is usually stopped in the acutely affected eye after the optic nerve edema has resolved since that disc is no longer at risk for NAION secondary to atrophy but continued in the unaffected eye. Low dose aspirin is usually recommended as well.
Visual acuity and visual field defects can improve somewhat in the months following the initial onset, but usually do not return to baseline. There is rarely ever progression beyond 2 months, and therefore any evidence of progression should be an indication to look for other causes of unilateral optic nerve edema, such as a mass lesion or intraocular inflammation.
Miller NR, Newman NJ, Biousse V, Kerrison JB, Editors. Walsh and Hoyt’s Clinical Neuro-ophthalmology. Lippincott Williams & Wilkins, Philadelphia, 2005.
Campbell UB, Walker AM, Gaffney M, Petronis KR, Creanga D, Quinn S, Klein BE, Laties AM, Lewis M, Sharlip ID, Kolitsopoulos F, Klee BJ, Mo J, Reynolds RF. Acute nonarteritic anterior ischemic optic neuropathy and exposure to phosphodiesterase type 5 inhibitors. J Sex Med. 2015 Jan;12(1):139-51.
Kupersmith MJ, Frohman L, Sanderson M, Jacobs J, Hirschfeld J, Ku C, Warren FA. Aspirin reduces the incidence of second eye NAION: a retrospective study. J Neuroophthalmol. 1997 Dec;17(4):250-3.
Copyright statement: Copyright ©2015. For further information regarding the rights to this collection, please visit: http://morancore.utah.edu/terms-of-use/