HPI: Patient is a 27 year old woman who was sent for asymptomatic abnormal retina appearance.
PMH:Past medical history was negative. Past ocular history was significant for mild myopia in both eyes. Specifically, she denied any history of trauma to the eyes or any condition needing eye drops.
Exam: Visual acuity was 20/20 in both eyes. IOP and pupils were normal in both eyes. The only abnormality is seen in the right macula as shown in the photographs below.
Hyperpigmentation due to past injury, inflammation or infection
Central Serous Chorioretinopathy
Torpedo maculopathy describes a congenital abnormal appearance in the macula of one eye. It is an void hypopigmented area in the temporal macula along the horizontal raphae. These lesions are non-progressive and asymptomatic. They are believed to be related to the fetal bulge of the eye presented in the 4th to 6th months of gestation. They are not associated with other developmental anomalies of the eye or the rest of the body.
The lesions associated with torpedo maculopathy can be associated with OCT changes and hyperautoflourescense but do not require an extensive workup for diagnosis. These patients do not need monitoring beyond that required by dictated by their age, systemic health and other eye conditions.
Golchet PR, Jampol LM, Mathura JR, et al Torpedo Maculopathy British Journal of Ophthalmology 2010;94:302-306
Shields CL, Guzman JM, Shapiro MJ, Fogel LE, Shields JA. Torpedo Maculopathy at the Site of the Fetal “Bulge”. Arch Ophthalmol. 2010;128(4):499-501.
IRVAN Syndrome – June 2017
A 65 year-old female was referred to Illinois Retina Associates for evaluation of decreased vision and macular changes in both eyes. She had a past ocular history of laser treatment in both eyes but had not seen a retinal specialist in a few years.
She was known for coronary artery disease, heart failure and atrial fibrillation and taking the following medication: hydralazine, coumadin, carvedilol, furosemide, pravastatin, aspirin. She was not diabetic.
Her uncorrected visual acuity was 20/400 in the right eye, and 20/60 in the left eye, not improved with pinhole. Anterior segment examination was unremarkable except for mild nuclear sclerosis in both eyes. On posterior segment examination of both eyes, there were scattered laser scars in both eyes, multiple sclerotic vessels, occasional retinal hemorrhages, and scattered fibrotic vascular dilations consistent with macroaneurysms (Figures 1 & 2).
Vision was 20/25 and 20/20, right and left eyes respectively. Intraocular pressures, pupils, nerves and anterior segments were unremarkable. Fundus examination of the right eye revealed subretinal fluid under fovea and inferior macula with no associated hemorrhage or exudates (Figure 1). The left eye was unremarkable (Figure 2).
Sickle Cell Retinopathy
Idiopathic multiple bilateral macroaneurysms
Macroaneurysms secondary to poorly controlled hypertension
Idiopathic retinal vasculitis, aneurysms and neuroretinitis (IRVAN) syndrome
On fluorescein angiography of both eyes, there were extensive areas of peripheral ischemia, focal retinal vasculitis, macroaneurysms, telangiectatic vessels, and evidence of prior laser (Figures 3 & 4).
Optical coherence tomography of the right eye showed subfoveal outer retinal atrophy and scarring (Figure 5), while the left eye had mild retinal striae (Figure 6).
The examination was consistent with “idiopathic retinal vasculitis-aneurysms-neuroretinitis” (IRVAN) syndrome.
The patient was followed closely for the development of vision-threatening complications. Given the absence of macular edema and neovascularisation, no treatment was needed at this time.
The patient was followed closely for the development of vision-threatening complications. Given the absence of macular edema and neovascularisation, no treatment was needed at this time.
A number of treatments have been described in the literature, including laser photocoagulation, cryotherapy, corticosteroids, anti-VEGF injection, immunosuppressive medication, and surgical intervention (vitrectomy), with mixed results. (3-5)
The visual outcomes are variable, depending on the aggressiveness of the clinical course and timely intervention. Close follow-up is essential to prevent visual loss. (1-5)
Samuel MA, Equi RA, Chang TS, et al. Idiopathic retinitis, vasculitis, aneurysms, and neuroretinitis (IRVAN): new observations and a proposed staging system. Ophthalmology. 2007;114:1526–1529Karagiannis D, Soumplis V, Georgalas I,
Pichi F, Ciardella AP. Imaging in the Diagnosis and Management of Idiopathic Retinal Vasculitis, Aneurysms, and Neuroretinitis (IRVAN). Int Ophthalmol Clin. 2012 Fall;52(4):275-82.
Kandarakis A. Ranibizumab for idiopathic retinal vasculitis, aneurysms, and neuroretinitis: favorable results. Eur J Ophthalmol 2010; 20(4): 792 – 794
Cheema RA, Al-Askar E, Cheema HR. Infliximab Therapy for Idiopathic Retinal Vasculitis, Aneurysm, and Neuroretinitis Syndrome. J Ocul Pharmacol Ther. 2011 Aug;27(4):407-10
Basha M, Brown GC, Palombaro G, Shields CL, Shields JA.Management of IRVAN syndrome with observation.Ophthalmic Surg Lasers Imaging Retina. 2014 May 1;45 Online:e18-22.
Macular Telangiectasia – April 2017
A 54 year-old female was referred to Illinois Retina Associates for decreased vision and macular changes in both eyes. Her visual symptoms had been present for 5 years, but progressively worsening. She had no past ocular history.
She was known for back pain and elbow epicondylitis and taking the following medication: gabapentin, naproxen, alprazolam, fluoxetine.
Her uncorrected visual acuity was 20/50 in the right eye, and 20/100 in the left eye, not improved with pinhold. Anterior segment examination showed nuclear sclerosis in both eyes. On posterior segment examination, there was a gray appearance and pigment mottling in the temporal part of the macula in both eyes and the loss of foveal reflex (Figures 1 & 2).
Focal Laser Scarring
Toxic Maculopathy (Plaquenil)
On fundus autofluorescence imaging, there was increased autofluorescence in the foveal region of each macula and focal hypoautofluorescence corresponding to the pigment mottling (Figures 3 & 4). Fluroescein angiography showed hyperfluorescence due to leaking telangiectatic vessels around the fovea, more on the temporal side (Figures 5 & 6). Optical coherence tomographyt showed small foveal cystoid cavities, disorganized retinal layers on the temporal side, and pigment migration into the retina (Figures 7 & 8).
Macular telagiectasis, also called idiopathic juxtafoveal telangiectasis, is typically subdivided into 3 subtypes, depending on their presentation. Type 1 is unilateral and is associated with parafoveal capillary dilation, microaneurysms and lipid exudation. Type 2 is the most common, is bilateral, and does not present with lipid. Type 3 is very rare and is characterized by parafovea ischemia.
Macular telangiectasis type 2 most commonly presents in middle age, has no gender nor racial predilection and is not inherited. Commmon complaints include blurry vision, distortion and scotomas.
Imaging studies, including autofluorescence, fluorescein angiography and OCT can be helpful in making the diagnosis early, when findings may be subtle. Some early findings include graying of the retina, telangiectasias, crystalline deposits, right-angle venules and cystoid spaces.
Patients are at risk of developing subretinal neovascularization with a subsequent rapid decrease in vision. There is no proven treatment for the nonproliferative stage, but once neovascularization is present, it may respond well to anti-VEGF injections.
The disease tends to have a slowly progressive course in the majority of patients, with visual loss over time.
Gass JD, Blodi BA. Idiopathic juxtafoveolar retinal telangiectasis. Update of classification and follow-up study. Ophthalmology 1993;100(10):1536-46
Nowilaty SR, Al-Shamsi HN, Al-Khars W. Idiopathic juxtafoveolar retinal telangiectasis: a current review Middle East Afr J Ophthalmol 2010;17(3):224-41
Roller AB, Folk JC, Patel NM, Boldt HC, Russel SR, Abramoff MD, Mahajan VB Intravitreal bevacizumab for treatment of proliferative and nonproliferative type 2 idiopathic macular telangiectasia Retina 2011;31(9):1848-55
Watzke RC, Klein ML, Folk JC, Farmer SG, Munsen RS, Champfer RJ, Sletten KR. Long-term juxtafoveal retinal telangiectasia Retina 2005;25(6):727-35
Wong WT, Forooghian F, Majumdar Z, Bonner RF, Cunningham D, Chew EY Fundus autofluorescence in type 2 idiopathic macular telangiectasia: correlation with optical coherence tomography and microperimetry. Am J Ophthalmol 2009;148(4):573-83
Yannuzi LA, Bardal AM, Freund KB, Chen KJ, Eandi CM, Blodi B. Idiopathic macular telangiectasia. Arch Ophthalmol 2006;124(4)450-60
Giant Retinal Tear-March 2017
A 62 year old man presented to clinic with 4 days of a veil progressing over the inferior portion of his visual field. There was no associated pain or flashing lights.
Past Ocular History
History of uncomplicated cataract surgery in the left eye with posterior chamber lens in place. Cataract right eye.
VA was 20/20 right eye and count fingers at 4 feet left eye. IOP was 22 and 16mmHg, respectively. The anterior exam was unremarkable and showed mild nuclear sclerotic cataract in the right eye and a posterior chamber lens in the left eye.
The posterior exam was notable for the findings below in the left eye:
Giant Retinal Tear
Broken capsule in cataract surgery
Based on appearance and history of the diagnosis of giant retinal tear was made. The patient was taken to the OR for scleral buckle, vitrectomy, endolaser and gas which resulted in successful retinal reattachment.
Giant retinal tears are defined as a retinal tear affecting greater than 90 degrees of the retina. Although associated with connective tissue disorders such as Sticker syndrom, Ehlers-Danlos syndrome and Marfan Syndrome, and risk factors such as high myopia, over half of cases are idiopathic. Males are more often affected than females (70% vs 30%). Giant tears provide a surgical challenge, as the retina may be prone to folding and slipping. There is a substantial risk of proliferative vitreoretinopathy for these patients compared to a typical retinal detachment. There is also some debate among retinal specialists about the use of scleral buckles for giant retinal tear related detachments.
Retina, 5th edition, Ryan et al.2013, Chapter 109, 1844-1851
Wyburn Mason – February 2017
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
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
PDR – 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