Cases

Intraocular Worm

Intraocular Lymphoma

Malignant Hypertension and
the Retina

Chronic traumatic macular hole

Central Areolar Choroidal Dystrophy

Retinopathy of Prematurity

Toxoplasmosis

Subretinal Hemorrhage with Warfarin use

Choroidal Mass

Sympathetic Ophthalmia
 

Figure 1: Upon presentation VA: Count fingers 4 feet		Figure 2: Ultrasound of worm		Figure 3: Post-Op VA: 20/25

This case has to be my all time favorite. A 25 year old young man presented with 3 weeks of decreased vision. His visual acuity was count fingers, and he complained of a “worm coming across my vision”. In truth he was absolutely correct! As illustrated in figure 1, there was a large 7mm mobile cysticercosis cyst within the vitreous. An ultrasound (figure 2) shows the circular cyst wall with the body of the worm inside. I performed a vitrectomy surgery with a scleral buckle, and removed the cyst whole. As shown in the postoperative photo (figure 3), the worm left significant areas of scarring superior to the macula which resulted in some traction on the fovea. However, as his inflammation subsided, his vision steadily improved to 20/25. More fortunate still, he did not have any evidence of systemic disease from this parasitic infection, and therefore has made a full recovery.

02/14/2010

Figure 1		Figure 2		Figure 3

This is a fascinating case that presented to us with a previous diagnosis of wet AMD. The patient was a 59 year old female who had been receiving Avastin injections in her right eye for wet AMD. (See Figure 1, and the OCT of the lesion in Figure 2) However, on examination she was found to have marked vitreous cell in both eyes, as well as subtle areas of subretinal/choroidal infiltration in the mid-periphery. We were unhappy with the previous diagnosis and decided to perform a vitrectomy with vitreous biopsy based on the exam. The pathology of the vitreous aspirate (Figure 3) showed Large B Cell lymphoma. The patient was begun on intravitreal methotrexate injections, and with the help of systemic treatment from an oncologist, her vision improved from 20/200 to 20/25 in her left eye.

03/17/2010

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Figure 1		Figure 2		Figure 3

Like most interesting cases, this patient was referred to our office at 6PM on a Friday. The patient was a 37 year old male visiting Los Angeles on vacation, who had noticed decreasing vision, associated with changes in his color perception, in both eyes over the last week. His vision on examination was 20/200 OD and 20/150 OS. He denied any known systemic medical conditions and was not taking any medications. On dilated exam (Figures 1 and 3) he had optic nerve edema, hard exudates tracking along Henle’s layer, and flame hemorrhages. Cirrus OCT analysis revealed a serous retinal detachment in his right eye (Figure 2) As part of our routine workup of every new patient we check blood pressure, and in his case it was 208/155! This very quickly solved our diagnostic dilemma as this was clearly Stage IV Hypertensive Retinopathy. He was immediately referred to a local ED and from there admitted to the ICU where he stayed for two days in order to safely bring down his blood pressure.

05/31/2010

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Figure 1		Figure 2		Figure 3

This patient was a 65 year old female with a history of blunt trauma to her left eye over 40 years ago. Her vision on examination was 20/20 OD and 20/400 OS. She reported being punched in her left eye as a young woman with chronic decreased vision in that eye ever since. On dilated exam (Figure 1) she had a large macular hole with a cuff of subretinal fluid. Temporal to this macular hole was a chorioretinal scar running concentric to the optic nerve, and likely representing an old choroidal rupture. Cirrus OCT analysis (Figures 2 and 3) illustrated wonderfully the macular hole and surrounding detachment in this left eye. Fine crystals were visible in the area of detached retina further indicating the chronic nature of these changes. Unfortunately given the length of time from the original injury, and the chorioretinal scar underlying the fovea, this macular hole was not a good candidate for surgical repair.

07/04/2010

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Figure 1: OD Fundus Photo		Figure 2		Figure 3: OS Fundus Photo

Figure A: Early CACD		Figure B: Mid CACD		Figure C: Late CACD

This 50 year old white female has been losing vision in both eyes over the last 10 years and gone undiagnosed. She presented to our clinic with complaints of decreasing vision. Her visual acuity was hand motions bilaterally. During the history it was discovered that she had less than normal vision (20/60 range) since early childhood. She had strabismus surgery in the right eye in childhood. Throughout her early to mid adult years, the patient was told that she had idiopathic scar tissue on the retina. Color plate, amsler grid and confrontation visual fields were unable to be performed. She had sensory rotatory nystagmus and eccentric fixation. Upon fundus examination, it was noted that the patient had bilateral central macular atrophy (Figures 1 and 3). OCT scans revealed bilateral thinning of both the retina and choroid with non-existent retina where the fovea should be (Figure 2). Central Areolar Choroidal Dystrophy (CACD) is hard to diagnose in early stages since patients in this stage usually have near normal visual acuity and non-specific granular hyperpigmentation of the fovea, which has a myriad of differential diagnoses (Figure A, from Ryan’s Retina, 4th edition, 2006). As CACD slowly progresses throughout the fourth and fifth decade of life, visual acuity starts to decrease and the area of hyperpigmentation becomes larger (Figure B, from Ryan’s Retina, 4th edition, 2006). In the later stages of CACD, the lesions that the patient presented with are typical and at this stage, central visual acuity is typically 20/200 or below (Figure C, from Ryan’s Retina, 4th edition, 2006). Unfortunately, there is no known cure for CACD at this time. The patient will undergo genotyping for possible gene therapy and for genetic counseling.

09/06/2010

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Figure 1		Figure 2		Figure 3

This 26 week gestational aged infant was found to have Stage 3 Threshold ROP in Zone II of both eyes 12 weeks after birth. Dilated, tortuous arterioles and veins were imaged using a RetCamII (see arrow in figure 1) and indicated the presence of plus disease, which is one of the criterions for threshold ROP. Since the infant had a gestational period of 26 weeks and a birth weight of less than 500 grams the placed the infant in a high risk category for developing severe ROP and aggressive treatment was necessary to prevent the progression of ROP. The infant underwent laser therapy within 72 hours after the exam1 and a fluorescein angiography (figure 2) was performed several days after to determine the effectiveness of treatment. The FA and visual exam revealed continued tortuosity of vessels and leakage that indicated more treatment was necessary. An intravitreal injection of Avastin (0.625mg) was given in each eye and the procedure was well tolerated by the infant with no endophthalmitis noted over the following several days. A dilated exam showed a regression to Stage 2 (see top arrow in figure 3) with no plus disease (see bottom arrow in figure 3). The progress of the infant is still being monitored.
The severity of Retinopathy of Prematurity is described using several parameters; zone, stage, extent of stage and presence of plus disease. The zone refers to the location of the retina that has been affected by ROP, and the retina is split into three segments. Zone 1 refers to the area that is encompassed by a circle that is centered at the optic nerve and has a radius equivalent to 2 times the distance from the optic nerve to the fovea. Zone 2 begins at the border of zone 1and extends to the nasal horizontal ora serrata. Zone 3 begins at the border of zone 2 and encompasses the remaining retina. ROP is characterized by a junction of avascular retina with vascular retina and the 5 stages of ROP define the clinical appearance of the junction. In stage 1, only a line is visable. Stage 2 shows the formation of a ridge, and neovascularization may or may not be present. Stage 3 shows the presence of neovascular growth into the vitreous at the ridge. Stage 4 is split into two catagories; Stage 4A, showing a partial retinal detachment without macular involvement and stage 4B showing a partial retinal detachment with macular involvement. Stage 5 is defined as a complete retinal detachment. The extent of stage refers to the risk of unfavorable anatomical outcomes and is divided into no description, prethreshold and threshold. For example, an infant with Stage 2 ROP means that they do not have either prethreshold or threshold ROP. How to categorize prethreshold ROP and threshold ROP is beyond the scope of this flyer. Plus disease refers to the presence of dilated, tortuous retinal arterioles and veins and usually indicates that the ROP is progressing. If treatment is required, laser therapy is used first and has been shown in studies to be the most effective. Recent studies have reported that the use of Avastin in combination with laser on infants with Stage 3 ROP or above to be effective; however prospective studies are needed to show the efficacy of this combination.

11/25/2010

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Figure 1		Figure 2		Figure 3

This 18 year old female presented to our clinic with complaints of blurry vision and floaters in the right eye beginning 6 days prior to the visit. The right globe was slightly tender to the touch, however there was no complaints of pain in either eye. No floaters, distortions or black curtains were noted. Visual acuity in the right eye was 20/25 with no pinhole correction. Amsler grid test revealed no distortions in the right eye and her color vision was unaffected. A dilated exam of the right eye revealed several small chorioretinal scars located next to a large raised white lesion approximately 1 disc diameter infra-temporally from the macula and there was a moderate amount of vitritis overlying the lesion (figure 2). No lesions were found in the left eye. During the history, it was discovered that the patient was from Poland and had a history of steak tartare (raw beef) consumption. Optical Coherence Tomography (figure 1) and Fluorescein Angiography (figure 3)revealed a large area of active retinitis in the right eye measuring around 1.2 disc diameters surrounded by a ring of retinal edema, and large destructive lesion morphological variant of ocular toxoplasmosis was suspected. A work-up revealed the presence of toxoplasma IgG antibodies and the patient was started on clindamycin, pyrimethamine and prednisone.
Toxoplasma gondii is an obligate intracellular protozoan parasite which is widespread throughout the world, affecting both humans and animals. It is a zoonotic disease, and primary transmission occurs through the ingestion of raw or inadequately cooked meat, chicken or eggs that has been contaminated with oocytes. The main host of T. gondii are members of the Felidae family which acts as the primary vector for the disease. Oocytes are only produced within members of the Felidae family and are shed through feces, which can contaminate food sources for other intermediate hosts such as cows, pigs or chickens. When Oocytes are ingested, digestive enzymes break down the cyst wall and T. gondii organisms are released. These organisms enter the blood stream and move through-out the body, infecting any cell besides erythrocytes. During the acute stage of the disease, T. gondii multiplies within the host’s cell, forming tachyzoites, which are released when the cell ruptures. The tachyzoites infect other cells, and the process continues until the immune system responds to the active infection. When this occurs, the tachyzoites enter the host cell vacuoles and transform into slowly dividing bradyzoites. This marks the beginning of the latent stage of the disease. Over time, bradyzoites form tissue cysts 10 to 200µm in size, which are typically located in the brain and retina due to protection from the immune system that the blood-brain barrier and blood-retinal barrier confers. In many cases, the infection remains latent before reactivation of the disease during periods of depressed immunity.
Common ocular symptoms of the disease include blurry vision, floaters and in some cases distortions if retinal edema is present. The characteristic clinical finding of toxoplasmosis is either a white or grey outer or inner retinal lesion that has a “headlights in the fog” appearance due to overlying vitritis. If these lesions are seen, confirmation of the diagnosis should be done through serological IgG and IgM antibody detection. If a patient has a negative IgG and a positive IgM, this would signify a possible acute infection. Conversly if the IgG is positive and IgM is negative, this would signify a latent infection of over 1 year. If both IgG and IgM are positive, this would signify an infection within 12 months. If toxoplasmosis is confirmed, the usual first-line therapy is a combination of pyrimethamine, folinic acid, sulfadiazine, and prednisone. Clindamycin can be used if the patient is allergic to sulfa medications or as an adjunct to the first-line therapy medications.

12/25/2010

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Figure 1		Figure 2		Figure 3

This 96 year old patient presented to our clinic for a follow-up visit with complaints of a severe, painless, decrease in vision in both eyes. Vision in the left eye was 20/400 using the periphery only and count fingers at 4’ in the right eye. Distortions with a large central scotoma were present in both eyes. A slit lamp exam revealed large subretinal hemorrhages in both eyes, and fundus photos (Figure 1-right eye) and OCT scans (Figure 2-comparison of OCT image from 1 month prior, right eye) were taken. Indocyanine Green Angiography showed a large, diffuse area of leakage near and under the hemorrhage (figure 3-right eye). An Intravitreal injection of Lucentis and dexamethasone was given to help decrease the size of the hemorrhage, and a PDT was performed 4 days later in an effort to mitigate the leakage from the neovascular membrane. Monthly Intravitreal injections of Lucentis and dexamethasone were given following the PDT and at three month follow-up, vision had increased to 20/100 in the left eye with no SRH noted. Prior to this episode, the patient had been followed in our clinic over the past 4 years for wet age-related macular degeneration. The patient’s vision had been stable at 20/40 in the left eye and 20/400 in the right eye. The patient had a history of atrial fibrillation and was taking the blood thinner warfarin.
The incidence of subretinal hemorrhages (SRH) in AMD patients is around 140 per 1,000 and occurs exclusively in patients with wet AMD. A subretinal hemorrhage forms as a result of a ruptured vessel within an extrachoroidal neovascular membrane, causing blood to leak under the retina, and in some cases into the vitreous. Several studies have shown that patients with wet AMD who take antithrombotic medications (i.e. warfarin, plavix, etc.) have a higher incidence of subretinal hemorrhages and have poorer outcomes. The use of aspirin, however, was not significantly associated with an increased risk of SRH. A retrospective, cross-sectional study recently published in Retina by Kiernan et al, stated that 49.2% of patients in the study with subretinal hemorrhages were taking an antithrombotic medication. Further analysis of the data revealed that the cumulative incidence of SRH was 63.5% among patients taking an antithrombotic medication versus 29.2% among patients not taking them (annual incidence of 0.10% versus 0.04%; p<.0001). Furthermore, cumulative incidence increased to 77% in patients on two or more antithrombotic medications. In a study by Kuhli-Hattenbach et al, though only 40% of the patients presenting with a subretinal hemorrhage were on a antithrombotic medication, this group had a statistically significant difference in the size of the hemorrhage (9.71 disc areas versus 2.99 disc areas; p <.0001) Once a subretinal hemorrhage occurs, few treatment options are available and a poor visual prognosis is the norm. Typically these patients end up with count finger vision, or in some cases, bare light perception; significantly affecting the quality of life for their remaining years. Risk of a subretinal hemorrhage increases with advancing age and the presence of arterial hypertension further increases the risk of a vision-threatening sub-retinal hemorrhage (53.5% of all patients with a SRH had arterial hypertension, and patients with arterial hypertension who were taking an antithrombotic medications had the worst outcomes).
Antithrombotic medications are prescribed for people who are at an increased risk of thrombosis, or as a secondary prophylaxis in individuals with a history of thrombus formation, such as deep vein thrombosis. Prescribing an anticoagulant (especially warfarin) to patients with Atrial Fibrillation (AF) is common practice, however a recent study in the American Journal of Cardiology concluded that anticoagulants may be used inappropriately. The study found that a large proportion (around 73%) of patients who had little to no risk of stroke were on an anticoagulant, whereas a relatively low proportion (around 60%) of high stroke risk patients were taking an anticoagulant. Guidelines on the management of AF by The American College of Cardiologist and American Heart Association state that a vitamin K antagonist is typically recommended in patients with more than 1 moderate risk factor (over 75, hypertension, heart failure, impaired LV systolic function, and diabetes Mellitus) or if they have a history of a thromboembolic event. For lower risk patients, aspirin (81mg-325 mg daily) can be used. In the event cardioversion (a procedure where either an electrical current or a medication is used to convert an abnormal heart rhythm into a normal heart rhythm) is performed, use of antithrombotic is recommended prior to the procedure and up to 4 weeks after the procedure. After 4 weeks, continued use of the antithrombotic should be determined normally. A newer technique for treatment of AF is cardio-ablation. In this procedure, the pulmonary vein antrum is isolated and destroyed with either radio waves or cryotherapy. During the procedure, the use of an antithrombotic (warfarin or plavix) is important; however, continued use after the surgery is still debatable. Recently, a study from a multicenter trial showed that patients with no recurrences of AF, were off antiarrhythmic medications, and had no contraindications (severe pulmonary stenosis or severe LA dysfunction) could safely discontinue anticoagulant usage. Because of the increased risk of sub-retinal hemorrhage seen in wet AMD patients on these medications, it would be beneficial to explore ways of discontinuing their use (in consultation with their cardiologist).

3/01/2011

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Figure 1		Figure 2		Figure 3

This 58 year old patient from Dubai presented to our clinic with decreased vision in the left eye for three weeks. She was originally examined in Dubai, where a large choroidal mass lying adjacent to an exudative retinal detachment was found in the left eye, and was advised to seek a doctor in the United States or Europe for further diagnosis and treatment. Upon examination in our clinic, visual acuity was 20/70 in the left eye, intraocular pressure was normal, and no changes in color vision were noted. Metamorphopsia was noted on amsler grid examination extending centrally to infranasally. No visual field defects were noted and no APD was found. OCT scan confirmed the presence of subretinal fluid with an exudative retinal detachment encroaching the macular area (figure 2, left image). A Fluorescein Angiography was performed, which showed areas of profuse leakage around the mass, and hyper-fluorescence within the mass (figure 1). Indocyanine-green Angiography, which was performed concurrently, showed hypofluoresence within the mass, without the pathoneumonic hyperfluorescent bulls-eye pattern on late-frames found in a choroidal hemangioma. The patient was referred to a primary care doctor for work-up. She was found to have a breast mass with a fixed auxillary node. She also had a pleural effusion on chest x-ray. A biopsy of the mass confirmed that they were malignant, but estrogen-sensitive, breast carcinoma. The patient was started on systemic chemotherapy by an oncologist, and was told to return to our office for treatment of the tumor inside the eye. We decided that the patient would undergo an intravitreal injection of Avastin (which will help shrink the tumor) followed by photodynamic therapy (which will dry out the lesion and help resolve the exudative RD). The use of both these treatments has been published in several prestigious, peer-reviewed journals over the past several years, and has reported positive results. At a 4 month follow-up visit (the patients most recent), vision was 20/25. OCT images confirmed the resolution of the exudative retinal detachment and a reduced macular thickness (figure 2, right image). A repeat Fluorescein Angiography showed diminished leakage around the mass and hypofluoresence within the mass throughout the procedure (figure 3), indicating reduced activity of the tumor.
Choroidal metastasis is the most common solid, intraocular malignant tumor, and arises from the hematogenous spread of cancer cells from a primary site. According a report by the Wills Eye Hospital, 67% of cases occurred in females, and primary cancer site was identified in 66% of all cases. In females the relative frequency of primary carcinoma site was: 68% breast, 12% lung, 2% gastrointestinal, 1% skin, and the rest in other various locations. In males, primary carcinoma sites were: 40% lung, 9% gastrointestinal, 6% prostate, 6% renal, skin 4%, and the rest in various other locations. Metastatic tumors of the eye were at one time believed to be relatively rare; however a relatively recent study indicated that more than 10% of all patients who died of cancer had intraocular metastatic foci upon pathological examination. The most common presenting symptoms include blurry vision, decreased vision, pain, photopsia, floaters, and visual field defects. Choroidal metastasis lesions can occur unifocally or multifocally, and bilaterally in 20%-40% of cases. Differential diagnosis should include choroidal melanoma , choroidal osteoma, choroidal hemangioma, choroidal neovascularization with disciform scars, and posterior scleritis. Typically choroidal metastases have a creamy yellow appearance as opposed to the reddish orange color typical of choroidal hemangiomas, and are usually flatter then choroidal melanomas and osteomas. Confirmation of the diagnosis is made through taking a comprehensive general medical history, Fluorescein/ Indocyanine Green Angiography, and A/B scan ultrasonography. Fine needle aspiration biopsy is rarely performed, and should only be considered when a histopathological verification or when a primary tumor site cannot be found in patients characteristic choroidal metastasis lesions. Typically treatment includes a combination of chemotherapy and ocular radiation therapy. Due to the adverse side effects of using radiation therapy (cataract formation, epithelial defects, radiation retinopathy, and optic neuropath), other treatment modalities are being studied, including use of Avastin and Photodynamic Therapy.

7/04/2011

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Figure 1		Figure 2		Figure 3

This 37 y.o. Hispanic male presented with 1 week of vision loss in his good right eye. He sees floating dots in his vision, and has a slightly injected conjunctiva for 1 week. He is an accountant and says that he can’t see to work as of today. He says that this has been treated in the past by intravitreal steroid injections by another retina specialist approximately 1-2x/year for last 5 years. He developed glaucoma and needed to have filtering surgery last year because drops did not control his intraocular pressure. He is 20/80+1 with correction in his right improving to 20/60+2 on pinhole. He is light perception in his left eye. He had blunt trauma to his left eye at age 16 when a baseball struck his eye and dropped his vision to LP. He developed a cataract in his right eye at age 27 and underwent CE/IOL. He has no other medical or surgical hx.
On exam his eye pressure is 12 in the right and 21 in the left. He is on Combigan bid and Predforte qid in the right eye. He has 2+ conj injection, 2+AC cell, 2+ anterior vitreous pigmented cell coating the back of the IOL densely, thickening of the macula, and multiple, raised, cream colored chorio-retinal lesions in the mid-periphery in the right eye (figure 1). There is dense band keratopathy on the cornea in the left eye, and no view to the back.
OCT (figure 2) shows a thickened central macula of 378 microns with loss of foveal pit, and FA shows multiple areas of early leakage, areas of RPE window defects in areas of old inflammation and cystoid macular edema in the late frames in the right eye (figure 3).
His diagnosis is based on his history and clinical findings: he has panuveitis in his good eye, developed early onset cataract at age 27 due to inflammation, got inflammatory/steroid induced glaucoma, and has CME with multiple Dalen-Fuchs nodules in the retina after a history of severe blunt trauma to his left eye. He had a blood work up for granulomous infection/inflammation and chest X-ray which was negative. He has Sympathetic Ophthalmia. He was started on systemic steroids (80 mg prednisone qd for 1 month) to stop the immune response in the bone marrow to his good eye, increased his topical steroid to 6x/day, and referred to an oculoplastics specialist for enucleation of his almost blind left eye(to blunt immune response by the inciting eye). His vision improved to 20/30-2 at one month after initiating tx, with significant decrease of CME on OCT. There was trace cell in the AC and anterior vitreous, and his IOP is 14. He will undergo enucleation in the left eye in the next 2 wks.
Sympathetic Ophthalmia is an extremely rare, yet potentially vision-threatening bilateral granulomatous panuveitis following a penetrating eye injury to one eye, and occurs in around 0.2% to 0.5% of non-surgical eye wounds. In the olden days of the Napoleonic Wars, there was a well known practice of putting cow manure on a blind traumatized eye on the battlefield to prevent blinding of the other good eye in the following months. Apparently, the injured eye got a severe bacterial infection and the body reacted to these antigens rather than the eye antigens. Though the etiology is not fully understood, it is believed that the underlying pathophysiology results from an autoimmune reaction against exposed ocular antigens (which are sequestered, thus not seen as self, from the immune system during development due to blood-eye-brain barrier), specifically the melanocytes within the outer segments of the retina. The disease usually expresses itself as Dalen-Fuchs nodules and recent immunochemical research has shown differential expressions of various cytokines and chemokines between these histopathologies. The symptoms of Sympathetic Ophthalmia typically begin anywhere from several days to several years after the injury (one case of 88 yrs after initial penetrating injury to the eye) and include blurry vision, floaters, loss of accommodation, photophobia and in some cases pain. Common clinical findings range from anterior uveitis, mutton-fat keratic precipitates, moderate vitritis, and multiple yellowish-white choroidal lesions. When Sympathetic Ophthalmia is suspected, it is important to rule out other granulomatous causing diseases such as Vogt-Koyanagi-Harada syndrome, sarcoidosis, tuberculosis, or syphilis. The diagnosis of Sympathetic Ophthalmia is made through a combination of eliciting a positive history of penetrating or severe ocular trauma, choroidal thickening, multiple sites of early leakage with late coalescence on fluorescein angiography, and hypofluorescent spots corresponding with the lesions on ICG angiography. Sympathetic Ophthalmia is initially treated aggressively with systemic corticosteroids and pred-forte; however in most cases enucleation of the inciting eye is necessary.

11/01/2011

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