A 21 year-old was referred to Illinois Retina Associates for abnormal vessels in her right eye. She had been experiencing flashing lights in her right eye for a few days and recently had a severe migraine episode which led to a visit to the emergency room, where she had a normal brain CT scan.
Her uncorrected visual acuity was 20/80 in the right eye, and 20/50 in the left eye, improved to 20/40 OU with pinhole. Anterior segment examination was unremarkable. On posterior segment examination, there were dilated and tortuous vessels in the superotemporal quadrant OD. The left eye posterior examination was unremarkable.
Retinal Capillary Heamngioblastoma (i.e. retinal angioma of von Hipple-Lindau)
Retinal Vasoproliferative Tumor (primary or secondary)
Fundus photography (Figures 1 & 2) and fluorescein angiogram (Figure 3) confirmed the presence of dilated and tortuous vessels in the superotemporal quadrant OD, with no evidence of leakage. Optical coherence tomography showed normal macula OD (Figure 4).
Given the typical appearance on clinical examination and imaging studies, a diagnosis of arterio-venous malformation on the Wyburn-Mason syndrome spectrum was made.
Given the possibility of an association of retinal arterio-venous malformations with other vascular anomalies of the brain and facial bones, MRI and MRA studies of the brain were recommended.
Wyburn-Mason is a congenital, non-hereditary condition that may go unnoticed for a long time if the malformations are minor and do not cause symptoms.
Patients with Wyburn-Mason syndrome can present a combination of systemic findings, the most common being: Arteriovenous malformations of the
Retina; typically unilateral but of varying degrees of severity
brain; depending on location and severity, this can lead to various neurological symptoms
facial bones (maxilla and mandible), which could result in severe bleeding during dental procedures
Nevi: subtle and uncommon
Visual complications are are but can occur if the malformation is leaking and resulting in macular edema.
Scuta GL, Cantor LB, Cioffi GA. Basic and Clinical Science Course. Singapore: American Academy of Ophthalmology, 2013:85-87
Ruggieri M., Konez O., Rocco Concenzo. Wyburn-Mason Syndrome. In: Ruggieri M. Pascual-Castroviejo I., Di Rocco C., ed. Neurocutaneous Disorders Phakomatoses and Hamartoneoplastic Syndromes New York, NY: Springer Vienna; 2008:345-352.
Bhojwani D, Vachhrajani M, Vasavada A. Wyburn Mason Syndrome: A Rare Phacomatosis Ophthalmology. 2016 Aug;123(8):1787.
Pattern Dystrophy – January 2017
A 53 year old female was referred to Illinois retina Associates for abnormal fundus apearance in both eyes. She does rely on glasses for bet vision, but is happy with vision with glasses or contacts.
The patient has a history of breast cancer treated with surgical removal. She has been in remission for over 5 years. She has seasonal allergies for which she occasionally takes antihistamines.
VA: 20/20, 20/80 PH 20/25
IOP: 20,19 mmHg
Pupils were equal and reactive without APD. The anterior segments were notable only for slight cataract in both eyes.
This patient has a pattern consistent with a bull’s eye maculopathy.
The differential for this consists of:
Chloroquine or hydroxycholoquine (plaquenil) toxicity
Age-related macular degeneration
Rod and cone dystrophy
Neronal ceroid lipofuscinosis
In this patient with excellent vision, no history of toxic exposures this patient was diagnosed as having a pattern distrophy. She has been stable without intervention for 2 years of follow-up.
Toxic bull’s eye retinopathy secondary to chloroquine toxicity causes the classic fundus appearance, and loss of the outer retina in the parafoveal area leading to a paracentral scotoma. Risk for chloroquine toxicity is associated with dose, with an increased risk at doses above 6.5mg/kg/day for hydroxychloroquine and 3mg/kg/day for chloroquine.The risk of toxicity is <1% after 5 years of therapy, 2% at 10 years and 20% at 20 years. Screening is recommended before starting therapy with full dilated exam and OCT. Full exam with OCT is then recommended at 5 years and each year thereafter. It is important that any toxicity be detected early because changes are not reversible.
Stargardt disease is the most common retinal degeneration and has a highly variable phenotype. It is an autosomal recessive disease affecting the ATP-binding cassette associated with the ABCA4 gene. Symptoms include central vision loss, abnormal dark adaptation, abnormal color vision and non-specific visual complaints. findings include pigment mottling, atrophy fundus flecks or a bull's eye appearance. The central vision loss is often slowly progressive, but the disease course is extremely variable.
Age-related macular degeneration and pattern distrophy can lead to a bull’s eye pattern through changes in RPE mottling, atrophy and drusen placement.
Neuronal ceroid lipofucinosis is also known as Batten disease and is a pediatric disease characterized by progressive vision loss, seizures, motor problems, dementia and death.
Ophthalmologu 2016;123:1386-1394 by the Amaerican Academy of Ophthalmology
Westerfield C, Mukai S, Stargard’ts disease and the ABCR gene. Semin Opthalmol 2008:23(1):59-65.
Bozorg S, Ramirez-Montealegre D, Chung M, Pearce D. Juvenile neuronal ceroid lipofuscinosis (JCNL) and the eye. Sury Opthalmol 2009 Jul-Aug;54(4):463-71.
Retinal Vasculitis – December 2016
A 19 year old was referred to Illinois Retina Associates for light sensitivity, blurry vision, and floaters in her left eye.
She was known for Sjogren’s syndrome, anemia and migraines, and was taking Plaquenil for the past year.
She had a history of intermittent floaters OS since an episode of retinal vasculitis OS which occurred shortly after an episode of mononucleosis 2 years ago. The causative agent for the mononucleosis was believed to be the Epstein-Barr virus (EBV), confirmed through laboratory testing.
Her corrected visual acuity was 20/20 in the right eye, and 20/30 in the left eye, not improved with pinhole. Anterior segment examination was unremarkable. On posterior segment examination, there was a mild vitreous hemorrhage, scattered areas of vascular sheathing and neovascularization OS. The exam of the right eye was within normal limits.
Fundus photography (Figure 1) and fluorescein angiogram (Figures 2 & 3) confirmed the presence of vitreous hemorrhage, areas of retinal ischemia and neovascularization OS. Optical coherence tomography showed irregularity of retinal layers but no edema OS (Figure 4). All imaging studies were normal OD.
Viral (CMV, VZV, EBV)
Bacterial (Syphilis, Tuburculosis)
Systemic Lupus Erythematosis
Inflammatory Bowel Disease
Idiopathic Retinitis, vasculitis, aneurysms and neuroretinitis (IRVAN) syndrome
All laboratory investigations were normal and the diagnosis was established as EBV-related retinal vasculitis with secondary retinal ischemia, neovascularization and vitreous hemorrhage.
Given the presence of extensive retinal ischemia, multiple areas of neovascularization and vitreous hemorrhage, pan-retinal photocoagulation treatment was recommended to decrease risk of recurrent hemorrhage or other complications such as retinal traction or detachment. The vitreous hemorrhage spontaneously improved over time.
Many local as well as systemic diseases may lead to retinal vasculitis. It is crucial to identify the cause of the vasculitis and rule out systemic involvement to prevent complications. Some forms of retinal vasculitis may benefit from systemic immunomodulatory therapy.
If the vasculitis is only affecting the retina, fluorescein angiogram is essential in order to identify retinal ischemia and neovascularization. In such advanced cases, pan-retinal photocoagulation can be applied to prevent visual loss from vitreous hemorrhage or retinal detachment.
Macular edema can also be present in vasculitis. Macular OCTs are helpful in identifying and quantifying the edema. When present, macular edema may result in decreased vision and may respond to drops (Corticosteroids, non-steroidal anti-inflammatories), local steroid injections, or systemic immunosuppressants.
Keorochana N. A Case Report of Epstein-Barr Virus-associated Retinal Vasculitis: Successful treatment using only acyclovir therapy. Int. Med Case Rep J. 2016 Jul 25; 9:213-8.
Rosenbaum JT, Sibley CH, Lin P. Retinal Vasculitis. Curr Opin Rheumatol 2016 May; 28(3):228-35.
Talat L, Lightman S, Tomkins-Netzer O. Ischemic Retinal Vasculitis and its management. J. Ophthalmol 2014;2014:197675
Retinal Artery Occlusion – November 2016
A 37 year old previously followed at Illinois Retina Associates for lattice degeneration presented with abrupt loss of vision on the left eye. It was described as flashes and floaters followed by a veil over her entire visual field about one (1) hour prior to arrival.
The patient had a history of hypertension not currently requiring medication and seasonal allergies. Her only medication was an oral contraceptive.
Hand Motion OS
Pressures were 14 mmHg (OD) and 16 mmHg (OS)
Pupils were equal and reactive with an RAPD OS.
The anterior segments were unremarkable.
The right eye posterior segment exam showed stable lattice degeneration in the right eye. The left eye also had stable lattice degeneration and was initially unremarkable. However, on repeat exam about 30 minutes later subtle whitening of the macula with a cherry red spot was appreciated (Figure 1).
Angiography revealed extremely delayed filling in the left eye (Figures 2 & 3)
Differential diagnosis for central retinal artery occlusion includes Susac syndrome, giant cell arteritis, artherosclerotic disease, cardiac disease, coagulopathy, collagen vascular disease, arterial loops, migraine, hypotension and trauma.
The patient was sent to the emergency department for supplemental oxygen therapy and was found to have elevated blood pressure of 205/129.
She was diagnosed with a central retinal artery occlusion secondary to malignant hypertension.
Two weeks later vision was 20/25 in both eyes. The cherry red spot had resolved and only a few cotton wool spots remained.
Central retinal artery occlusions are most common in those in their 6th decade of life. Men are more often affected than women. Acutely, patients notice a severe decrease in vision. At presentation they may appear normal or have a cherry red spot in the middle of the fundus. A cilioretinal artery may perfuse the fovea in 15% of patients. Such patients have an 80% chance of returning to 20/50 vision or better. Those without cilioretinal perfusion of their fovea have a much more guarded visual prognosis.
Many treatment shave been purposed includeing: breathing an increased concentration of carbon dioxide, an increased concentration of oxygen (including trials of hyperbaric oxygen), lowering the intraocular pressure via paracentesis or pharmacologically to nudge the obstruction further down the arterial tree, and using intraarterial TPA to dissolve the clot. None of these has been established as a standard of care.
It is important to realize that there is a high rate of stroke after retinal artery occlusion with a 15 times increase in stroke rate in the first 30 days after onset. Thus, these patients need a full workup with their primary care physicians to minimize stroke risks. Some even advocate sending these patients to emergent stroke centers for acute management of risk.
Atebara NH, Brown GC, Cater J. Efficacy of anterior chamber paracentesis and Carbogen in treating nonarteritic central retinal arterial occlusion. AM J Ophthalmol 1995; 102:2029-2034.
Brown GC, Margargal LE. Central retinal obstruction and visual acuity. Ophthalmology 1982;89:14-19.
Recchia FM, Brown GC. Systemic disorders associated with retinal vascular occlusion. Curr Opin Ophthalmol 2000;11:462-467
Sang Jun Park, Nam-Kyong Choi, Bo Ram Yang, Kyu Hyung Park, Joongyub Lee, Sun-Young Jung, Se Joon Woo. Risk and Risk Periods for Stroke and Acute Myocardial Infarction in Patients with Central Retinal Artery Occlusion, Ophthalmology 2015;122:11
Proliferative Diabetic Retinopathy – October 2016
A 41 year-old female was referred to Illinois Retina Associates for blurry vision in both eyes of long duration. She was referred for bilateral retinal hemorrhages.
The patient is known for poorly controlled diabetes and hypertension. She had not had regular dilated eye examinations since the diabetes diagnosis.
Posterior segment exam
On anterior segment examination, visual acuity was 20/40 in both eyes. In the posterior pole, there were scattered retinal hemorrhages, vitreous and pre-retinal hemorrhages, venous beading, and diffuse pre-retinal neovascularization (Figures 1 & 2).
Fluorescein angiography of both eyes demonstrated peripheral nonperfusion, venous beading, and extensive leakage from the pre-retinal neovascularization (Figures 3 & 4).
OCT imaging revealed traction from a thickened posterior vitreous in the right eye and the lack of macular edema in both eyes (Figure 5 & 6).
Given the history of poorly controlled diabetes, the most likely cause of the extensive ischemia and neovascularization was proliferative diabetic retinopathy. Other potential causes to be considered include: bilateral central retinal vein occlusions, bilateral ocular ischemic syndrome, familial exudative retinopathy, sickle cell retinopathy.
Treatment and follow-up
The main problem that had to be addressed was the extensive pre-retinal neovascularization in both eyes. The patient underwent panretinal photocoagulation laser therapy in both eyes.
Other treatment options to be considered could include vitrectomy surgery, which would allow removal of the vitreous hemorrhage and release of the traction on the macula (in the right eye), along with laser to the peripheral retina.
The patient will need to be followed closely for worsening vitreous hemorrhage, recurrence of neovascularization, macular edema, and tractional macular detachment.
Diabetic retinopathy afflicts a large number of patients with diabetes, and the prevalence increases with time since diabetes diagnosis and poor glycemic control. Non-proliferative diabetic retinopathy is characterized by retinal hemorrhages, intraretinal lipid, ischemia, venous beading. The more advanced stage of the disease, proliferative diabetic retinopathy, can present all of the above findings, in addition to pre-retinal neovascularization, vitreous hemorrhage, tractional retinal detachments, and/or anterior segment (iris and angle) neovascularization.
If not treated promptly, proliferative diabetic retinopathy can lead to severe loss of vision from vitreous hemorrhage and/or tractional retinal detachment. Urgent referral for treatment is essential to prevent loss of vision. The gold standard for treatment of proliferative diabetic retinopathy is peripheral laser therapy, which leads to regression of neovascularization. In cases with non-improving vitreous hemorrhage or tractional retinal detachments, vitrectomy may be necessary.
In addition to the peripheral retinal findings above, diabetic patients may present with decreased vision from macular edema at any stage of the disease. The treatment for diabetic macular edema is anti-VEGF or steroid intravitreal injections or laser.
In fact, it is recommended that patients with type 2 diabetes undergo regular dilated eye examinations to screen for the development of diabetic retinopathy starting at the time of diabetes diagnosis.
A 25 year old male was referred to Illinois Retina Associates for blurred vision in the right eye for the past week. There were also floaters but not flashing lights or pain. No problems were noted in the left eye. He had no known systemic illnesses. He is on no systemic or ocular medications. He has a history of trauma to the left eye and subsequently underwent cataract surgery in that eye without subsequent issue.
Vision on presentation was 20/150 in the right eye and 20/20 in the left. Pressures were normal at 13 mmHg and 16 mmHg in the right and left eyes, respectively. The anterior segment exam was unremarkable. Posterior segment exam showed 2+ vitreous cell and a superonasal chorioretinal scar with adjacent whitening in the right eye as shown below (Figure 1) and pigmented ‘quiet’ scar noted directly nasally (Figure 2).
Fluorescein Angiography showed no leakage either eye with some staining of the white fibrotic area in the right eye.
Systemic workup included toxoplasmosis IgG and IgM, Quantiferon Gold, rapid plasma reagin, complete blood count, erythrocyte sedimentation rate and chest X-ray. This was normal or negative with the exception of toxoplasmosis IgG.
The patient was initiated on therapy including triple therapy for toxoplasmosis with sulfasalazine, pyrimethamine and leucovorin and treatment of inflammation with oral and topical steroids. The patient’s vision improved slowly with therapy back to 20/25 as the vitreous debris slowly cleared.
The vision slowly began to improve as the vitreous debris cleared in response to treatment. Four months after presentation he was back to 20/25 with only minimal vitreous debris remaining. The whitening of the retina had regressed and he remained quiet as he was weaned off of steroid treatment and the antibiotics were stopped. Unfortunately, a month after cessation of therapy the disease reactivated at the posterior edge of the lesion (Figure 3). All medications were restarted.
Toxoplasmosis is a disease caused by the intracellular parasite Toxoplasma gondii. It is thought to be the most common cause of posterior uveitis worldwide. Most people are exposed to toxoplasmosis in their lifetime, only a minority of these will develop ocular disease. Many who do develop ocular disease can be safely watched, as long as the lesion is not near the macula.
For those with vision threatening disease or those who are immunocompromised, therapy with multiple antibiotics and steroid is recommended. The conventional combination is systemic pyrimethamine, sulfadiazine, and corticosteroids. Oral clindamycin may be considered as an additional agent. There has also been evidence to support use of intravitreal clindamycin and dexamethasone as an alternative to oral treatment.
Arantes, T. E. F., Silveira, C., Holland, G. N., Muccioli, C., Yu, F., Jones, J. L., . . . Belfort Jr., R. (2015). Ocular involvement following postnatally acquired toxoplasma gondii infection in Southern brazil: A 28-year experience.American Journal of Ophthalmology, 159(6), 1002-1012.e2. doi:http://dx.doi.org.ezproxy.rush.edu/10.1016/j.ajo.2015.02.015
Grigg, M. E., Dubey, J. P., & Nussenblatt, R. B. (0727). Ocular toxoplasmosis: Lessons from brazil
Soheilian, M., Ramezani, A., Azimzadeh, A., Sadoughi, M. M., Dehghan, M. H., Shahghadami, R., . . . Peyman, G. A. (2011). Randomized trial of intravitreal clindamycin and dexamethasone versus pyrimethamine, sulfadiazine, and prednisolone in treatment of ocular toxoplasmosis.Ophthalmology, 118(1), 134-141. doi:http://dx.doi.org.ezproxy.rush.edu/10.1016/j.ophtha.2010.04.020
Hypertension – August 2016
A 45 year-old was referred to Illinois Retina Associates for a diabetic exam. She had noticed a gradual decrease in vision over the previous two weeks in both eyes.
She was known for diabetes, hypertension, asthma and sickle cell trait. She was on multiple blood pressure medications and insulin.
Her best corrected visual acuity was 20/40 in the right eye and 20/100 in the left eye. Anterior segment examination was unremarkable. On posterior segment examination, there was bilateral severe optic nerve edema and star-shaped exudates, scattered posterior pole flame-shaped hemorrhages and cotton-wool spots.
Increased intracranial pressure
Infectious optic neuropathy
Inflammatory optic neuropathy
Sickle cell retinopathy
Fundus photography showed the optic nerve edema and macular exudation in both eyes (Figures 1 & 2). Suspected macular edema was confirmed with OCT (Figures 3 & 4).
The patient’s blood pressure was checked and found to be 210/150 mmHg. She admitted to not being compliant with her blood pressure medication. The patient was referred to the emergency room for prompt blood pressure control. The patient was diagnosed with hypertensive retinopathy
Six weeks after achieving blood pressure control, her visual acuity, her visual acuity improved to 20/30 in both eyes, and the macular edema was diminishing without further intervention (Figures 5 & 6).
Malignant hypertension is defined as blood pressure high enough to cause end-organ damage, typically over 180/120 mmHg. Common fundus findings in patients with malignant hypertension include bilateral optic nerve edema, macular edema, star-shaped macular exudates, cotton-wool spots and retinal hemorrhages. the diagnosis is made by identifying these fundus changes and checking the blood pressure.
Although these findings are commonly found in uncontrolled hypertension, they are non-specific. Therefore, a number of different differential diagnoses need to be considered if the blood pressure is not acutely elevated, including infectious and inflammatory causes, as well as increased intracranial pressure.
Once a diagnosis of malignant hypertension is made, urgent referral for bringing the blood pressure under control is necessary because of the potential life-threatening complications, such as stroke and myocardial infarctions.
Hayreh SS, Servais GE, Virdi PS. Fundus lesions in malignant hypertension, V: hypertensive optic neuropathy Ophthalmology.1986;93:74-87.
Luo BP, Brown GC. Update on the ocular manifestations of systemic arterial hypertension. Curr Opin Ophthalmol2004;15:203-210.
Suzuki M, Minamoto A, Yamane K, et al. Malignant hypertensive retinopathy studied with optical coherence tomography.Retina.2005;25:383-384.
Photic Maculopathy – July 2016
A 17 year-old was referred to Illinois Retina Associates for sudden decreased vision in his right eye.
He was otherwise healthy, with no known past medical history and not taking any medication. He presented with sudden appearance of a “blank spot in vision” in the right eye the day before.
His visual acuity was 20/70 uncorrected, 20/50 with pinhole in the right eye, and 20/20 in the left eye. Anterior segment examination was unremarkable. On posterior segment examination, a small round yellowish lesion was noted close to the foveal center in the right eye. The left eye’s funduscopy was normal.
Fundus photography showed the macular lesion (Figure 1), while the left eye appeared normal. Spectral-domain OCT showed disruption of the outer retinal layers (Figure 2).
Upon further questioning, the patient recalled being at a dance party with bright moving lights and lasers the night before the decrease in vision. The most probable diagnosis was photic maculopathy.
Over the next 7 months, the patient’s visual acuity stabilized at 20/50 in the right eye. The appearance of the macular lesion changed slightly on fundus photography, diminishing in size and appearing more pigmented (Figure 3). OCT imaging showed some restoration of the damaged outer retinal layers (Figure 4).
Photic maculopathy occurs after exposure to strong direct lights, which produce an electrochemical reaction leading to retinal damage. Multiple cases have been reported from different exposure sources, including operating microscopes, laser beams, arc welding, lighting. Solar retinopathy has a similar pathogenesis, except it tends to be binocular and is due to sun gazing.
Typically, the patients present with decreased vision and a yellow foveal spot which fades somewhat over time and may lead to localized hyper or hypopigmentation. OCT imaging usually shows disruption of the outer retinal layers or the RPE, which may either progress to atrophy or improve over time.
There is no treatment at this time, and close observation for the development of any other complication is recommended.
de Almeida FPP et al. Photic Myopathy Following Exchange of Crystalens: Clinical and Spectral-domain OCT Features. Retinal Physician. 2009 Oct.
Kweon EY et al. Operating microscope light-induced phototoxic maculopathy after transscleral sutured posterior chamber intraocular lens implantation. Retina. 2009 Nov-Dec;29(10):1491-5.
Posteal EA et al. Long-term follow-up of iatrogenic phototoxicity. Arch Ophthalmol. 1998 Jun;116(6):753-7.
Roe RH et al. Complications of Vitreoretinal Surgery. Review of Ophthalmology. 2008 Jan.
Epiretinal Membrane – June 2016
A 68 year old man complained of gradual blurry vision with metamorphosia in his right eye over the past 1-2 years. He reports the distortion centrally to be getting worse with time. He has had cataract surgery in both eyes.
Visual acuity with myopic correction was 20/60 in the right eye and 20/30 in the left. The anterior segment was within normal limits with well-centered posterior capsular intraocular lenses. The posterior segment exam revealed diffuse epiretinal membrane (ERM) in the right eye.
Fundus photographs revealed the presence an ERM and optical coherence tomography confirmed the finding and elucidated its morphology. (Figure 1).
The patient elected to pursue pars plana vitrectomy with internal limiting membrane (ILM) and ERM peel. The membrane was removed without complication.
The following series of pictures reveal the process of removing a macular pucker.
Figure 2: Staining the ILM (and negatively stain the ERM) with a green dye.
Figure 3: Initiate membrane peel with looped instrument.
Figure 4: Engagement of ERM with forceps.
Figure 5: Majority of ERM grasped with forceps.
Figure 6: Underlying ILM grasped with forceps.
Figure 7: Post peel with typical intraretinal heme (which resolve without consequence).
The patient’s visual acuity has been improving slowly after surgery as might be expected. Six months after the surgery he has 20/40 visual acuity and reports much improved central distortion in his right eye (Figure 8).
When glial cells from the neurosensory retina proliferate and migrate to the surgace of the ILM they settle to form a semi-translucent fibrocellular sheath. This new glial scar tissue is commonly referred to as Cellophane Maculopathy, Macular Pucker, or Epiretinal Membrane (ERM).
Most ERM’s are idiopathic, and occur as a result of aging. This event occurs within the vitreoretinal junction where the posterior hyaloid membrane and the ILM meet. It is widely accepted that idiopathic ERM’s occur after these two entities separate, or detach from one another. The posterior vitreous detachment (PVD) may leave breaks within the ILM which allows for the migration and settlement of neurosensory support cells. This glial fibrocellular sheath is contractile in nature, and may cause distortion of all layers of the neurosensory retina. Macular contraction is also responsible for irregularities on the surface of the inner retina. As a result of this event patients will notice decreased visual acuity (VA), and metamorphosia (distortion).
Empiretinal membrane is a very common cause of distorted or blurry vision in patients above 50 years old. In this particular case, the patient had an uncomplicated cataract extraction and his residual blurry vision was thought secondary to epiretinal membrane. In this situation, one must decide between observation and surgery. If operating, what is the optimal visual acuity when surgical correction makes the most sense?
As with any ocular surgery, there are multiple factors including the patient’s age, ocular and systemic comorbidities, as well as his or her subjective visual symptoms that must be taken into account when deciding if or when to operate.
Optical Coherence tomography allows for direct visualization of the epiretinal membrane. One can ascertain qualities such as the area of the retina affected, thickness of the membrane, as well as the level of distortion of the underlying retina.
The procedure has progressed over the years, and now with small guage and new instrumentation, is often being considered sooner in the course of disease.
The majority of patients who undergo pars plana vitrectomy with ILM peel for epiretinal membrane have excellent visual outcomes.
Macular Pucker: To peel or not to peel the internal limiting membrane? A Microperimetric Response Ripandelli G, Carinci, F, Piaggi P, Guidi G, Pileri M, Cupo G, Sartini MS, Parisi V, Baldanzellu S, Giusti C, Nardi M, Stirpe M, Lazzeri S. Retinal. 2015 Mar;35(3)498-507
Preoperative and intraoperative prognostic factors of epiretinal membranes using chromovitrectomy and internal limiting membrane peeling Machado LM, Furlani BA, Navarro RM, Farah ME, Maia A, Magalhaes O Jr, Rodrigues EB, Moraes N, Maia M. Opthalmic Surg Lasers Imaging Retina. 2015 Apr;46(4):457-62
Foveal contour changes following surgery for idiopathic epiretinal membrane. Matthews NR, Tarima S, Kim DG, Kim JE. Invest Ophthalmol Vis Sci.2014 Nov 13;55(12):7754-60
Outer Retinal Morphological Changes and Visual Function after Removal of Epiretinal Membrane Rii T, Itoh Y, Inoue M, Hirota K, Hirakata A. Can J Ophthalmol. 2014 Oct;49(5):436-42
Arnold Chiari – May 2016
A 24 year-old woman was referred to Illinois Retina Associates for blurry vision in her right eye.
She was otherwise healthy, known for occasional episodes of tachycardia not needing treatment, and taking only oral contraceptives. She complained of gradual vision loss in the right eye starting the prior day and recently worsening headaches.
Although her visual acuity was 20/20 in both eyes, she had a subjective complaint of decreased vision in her right eye. There was no afferent pupillary defect, and confrontational visual fields were normal. On posterior segment examination of the right eye, there were a few scattered peripapillary, flame-shaped and blot hemorrhages, mild disc edema, and mild venous dilation and tortuosity. There was mild elevation of the left optic nerve.
Venous stasis retinopathy/impending central retinal vein occlusion
Intracranial hypertension, idiopathic or secondary to other causes
Fundus photography revealed the blot and flamed-shaped hemorrhages in the right eye (Figure 1), while the left eye appeared normal. A fluorescein angiogram showed normal circulation patterns in both eyes (Figure 2).
Additional testing was performed by a hematologist, including a work-up for hypercoagulability disorders, but all the investigations were negative.
A brain MRI revealed an Arnold Chiari malformation and syringomyelia.
Optic nerve edema and venous stasis retinopathy secondary to Arnold-Chiari malformation
The patient’s visual symptoms subsided within a few months of presentation, and teh fundus hemorrhages resolved completely (Figure 3). Her headaches have significantly improved as well. She is currently followed by a neurosurgeon.
Arnold-Chiari is a structural defect of the cerebellum, resulting in its downwards placement within the skull, and can sometimes result in cerebrospinal fluid accumulation and increased intracranial pressure. Symptoms of high intracranial pressure include, among others, headaches and optic nerve swelling.
Syringomyelia is a disorder characterized by cerebrospnal fluid accumulation in a cyst form within the spinal cord, and is often associated with Arnold-Chiari.
Haaland Stone WJ, Ittner EA, Teitelbaum BA, Messner LV. Progressive, asymptomatic papilledema as the presenting sign of a Chiari I Malformation. Optometry. 2012 Mar 30;83(3):114-9
McVige JW, Leonardo J. Neuroimaging and the clinical manifestations of Chiari Malformation Type I (CMI) Curr Pain Headache REp. Jun;19(6):18
Zhang JC, Bakir B, Lee A, Yalamanchili SS. Papilloedema due to Chiari I Malformation. BMJ Case Rep.1002 Oct 16;2011