Interesting Clinical Cases

Intraocular Worm

This 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. A vitrectomy surgery with a scleral buckle was performed, and the cyst was removed 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.

Intraocular Lymphoma

This 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.

Malignant Hypertension and the Retina

This 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.

Chronic traumatic macular hole

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.

Central Areolar Choroidal Dystrophy (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.

Retinopathy of Prematurity

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.

Toxoplasmosis

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.

Subretinal Hemorrhage with Warfarin use

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).

Choroidal Mass

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.

Sympathetic Ophthalmia

This 37 year old 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.

Behcet's Disease

35 y.o. Lebanese male presented to the office complaining of decreased and blurry vision in both eyes for 3 months. He also noted floaters and photophobia in both eyes for a month. Patient has been living in the U.S. for 8 months, with a history of eating raw meat. Patient has no history of color deficiency or any blindness in the family.
Patient was seen at our office with VA at 20/60-2 in the right eye, no pinhole improvement, 20/60+2 to 20/50 with pinholes in the left eye. Anterior Examination of the AC revealed 3+cells in both eyes, with posterior synechiae of the iris to the lens in both eyes, and a 1+-2+ PSC cataract in both eyes (Figure 1). Anterior vitreous revealed 1+ non-pigmented cells in both eyes. Examination of the retinal vasculature showed arteriolar and venous sheathing throughout the entire retina in both eyes (Figure 2). High Definition Optical Coherence Tomography of the retinas revealed macular thickness of 261 microns in the right eye and 250 microns in the left eye, with macular cysts and decreased foveal contour in both eyes (Figure 3). Initial diagnosis was Panuveitis in both eyes.
Patient was told he needed further work up for FTA-ABS, testing for Sarcoidosis, PPD, CXR, CBC with differentials and ESR. He returned a week later for follow up. Work up was negative. An additional history of oral ulcer, and dysuria was discovered. Patient’s vision worsened in the right eye to count fingers at 3 feet. A diagnosis of Behcet’s Disease was made, based on his history and clinical findings. Patient was started on 140mg of prednisone for 2 weeks.
After the course of prednisone was finished, patient’s vision improved to 20/70+2 to pinhole 20/60-1 in the right eye and 20/50-2 to pinhole 20/30-2 in the left eye. Patient’s IOP increased to 38mmHg in the right eye and 40mmHg in the left eye, most likely due to response to steroids. Decrease of prednisone was advised. The dose of prednisone was decreased to100mg qd and was started on Combigan tid in both eyes to reduce IOP.

Patient presented to clinic one week later for IOP check with good IOP of 15mmHg in the right eye and 16mmHg in the left eye. Patient returned to clinic two weeks later for follow up. VA improved to 20/40 in the right eye and 20/50 in the left eye and there was decrease in inflammation in both eyes. Patient’s regimen of prednisone was reduced to 60mg qd. Patient was scheduled to return to clinic in 2 weeks for continued observation and treatment.
Behcet's Disease, also called Behcet's syndrome, is a rare disorder that causes chronic inflammation in blood vessels throughout the body. The exact cause of Behcet's is unknown, but it may be an autoimmune disorder. Both genetic and environmental factors may be responsible for Behcet's disease. The inflammation leads to numerous symptoms that may initially seem unrelated and vary from patient to patient. Symptoms may come and go on their own. Treatment aims to reduce the signs, symptoms and serious complications, such as blindness. The signs and symptoms that a patient may experience depend on which parts of the body are affected by the inflammation. Common symptoms caused by Behcet's disease include painful mouth sores, skin lesions, sores on the genitals, uveitis and inflammation of the blood vessels of the retina. Joint swelling and pain often affect the knees. Inflammation in veins and large arteries may cause redness, pain and swelling in the arms or legs. Some patients may even experience abdominal pain or diarrhea. Finally, the disease may cause inflammation in the brain and nervous system that leads to headache, fever, disorientation, poor balance or stroke. Treatment should be given by using steroids initially, such as prednisone to help reduce inflammation. Steroids have been shown to delay the onset of blindness by reducing the inflammation in the blood vessels of the retina. Steroid sparing biological agents are also used to treat inflammation and decrease the side effects of steroids in the long term.

Pseudotumor Cerebri

25 y.o. white obese female with type 1 diabetes for the past 10 years presented to the clinic with a history of migraine headaches. She was referred by an optometrist to rule out optic disc edema. Upon examination, patient had no complaints of any changes to visual acuity, distortions, flashes or floaters. Her visual acuity was measured at 20/20 in both eyes with correction. The patient's eye pressures were normal with the right eye being good IOP of 10mmHg and the left eye at 9mmHg.
Exam with slit lamp revealed a cup to disc ratio of 0.1 OU. Additional optic nerve observation revealed optic disc swelling in both eyes, greater in the left than in the right. The macula of both eyes was normal for the patient's age. A HD-OCT (see Figure 2) was performed and revealed good foveal contour of both eyes. Macula thickness was normal. Optic nerve OCT also revealed edema of the optic nerve, greater in the right than in the left.
A diagnosis of Pseudotumor Cerebri and Disc Edema was made. Patient was told she needs a work up to rule out other causes of papilledema with a CT scan of the head and lumbar puncture with opening pressure. She was told to RTC in 2 weeks and was advised to change her diet, exercise and lose about 15lbs in order to manage diagnosis. Patient was also educated about risks of not managing her condition and possible alternatives such as medication. She was referred to her neurologist for follow up.
Patient returned to clinic with complaints of dizziness and headaches. Lab results were obtained and MRI was normal, showing no lesions. Spinal tap opening pressure was abnormal at 36 cm of water. Upon examination, her visual acuity was 20/30 -2 in the right eye and 20/20 in the left eye with correction. Patient was prescribed 250mg of Acetazolamide po BID and was advised of the negative effects of excess weight on her condition. Patient is to RTC in one month for follow up.
Patient returned to clinic with less symptoms and visual acuity of 20/20 with correction in both eyes. She has been responding well to the acetazolamide with less disc edema in both eyes. She is to continue with acetazolamide 250mg po QID, was encouraged to lose more weight and is to RTC in 3-4 weeks.
Idiopathic intracranial hypertension, also called pseudotumor cerebri is a neurological disorder caused by increased intracranial pressure. Symptoms can include headache, nausea, vomiting, tinnitus, double vision and other visual symptoms. If the condition is left untreated, it can lead to swelling of the optic disc which can progress to vision loss. The increased pressure can lead to papilledema and this occurs practically in all cases of pseudotumor cerebri but not all patients experience symptoms from this. This condition occurs more often in women than in men, especially if the women are overweight or obese. Medications such as cyclosporine, minocycline, nitrofurantoin, phenytoin and tetracycline can increase the risk for this condition. It is important to have a MRI or CT scan if there is suspicion of brain mass. Typically, treatment is directed to the cause of the pseudotumor. Usual treatment plans include weight loss, salt or fluid restriction, medications such as acetazolamide and shunting procedures to relieve pressure from spinal fluid buildup. At times, the condition can disappear on its own within 6 months. Follow up with an ophthalmologist is crucial to help when visual symptoms occur. Some persons can have their symptoms return, about 10-20% of the time.

Neuroretinitis due to Cat Scratch Disease

24 y.o. white male presented to the office for evaluation of papilledema. Upon examination, patient complained of decreasing vision in both eyes x 6 days. He also complained of distortion in his vision. Patient's visual acuity was measured at count fingers at 1 foot in the right eye and 20/400 in the left eye. The patient's eye pressures were normal with the right eye being at 13mmHg and the left eye at 15mmHg.
Exam with slit lamp revealed a cup to disc ratio of 0.3 in both eyes. Additional optic nerve exam revealed bilateral disc edema. Examination of the macula revealed development of macular star in both eyes. A HD-OCT was performed and revealed right eye macula thickness at 479 and left eye macula thickness at 735 microns. Optic nerve scan revealed abnormal thickness with peri-papillary subretinal fluid in both eyes with thickness of 241 microns in the right eye and 249 microns in the left eye.
Patient was referred to neurologist for CT scan of head and orbits. He returned to clinic for follow up exam. His CT scan came back negative for any lesions in the head. Patient's vision was count fingers at 2 feet in the right eye and 20/70 in the left eye. Patient was prescribed Acetazolamide 250mg 2tab po q 12 hrs for 2 weeks. He was admitted to the hospital for treatment and was found to be HIV+. He has several cats at home. He was tested for and diagnosed with cat-scratch disease due to elevated IgM antibodies to cat scratch and was given a regimen of Doxycycline and Rifampin. He returned to clinic in 10 days with improved vision of count fingers in the right eye at 5 feet and 20/50 in the left eye. He continued course of treatment and returned to clinic in 2 weeks. His vision improved to 20/50 in the right eye and 20/40 in the left eye. He also retested for HIV, because the first test was an ELISA test, and the Western Blot was negative, his first results being a false positive.
Patient returned to clinic in 6 weeks with improved vision of 20/30 to 20/25 with pinholes in the right eye and 20/25 in the left eye. He discontinued course of Doxycycline and Rifampin. There was less edema with the right eye macular thickness being at 216 microns and left eye macula thickness at 207 microns. Optic nerve scan revealed less subretinal fluid with the right eye at 193 microns and left eye at 161 microns. Patient was told to RTC in 3 months for follow up. Neuroretinitis can be classified as infectious or idiopathic. Most infectious neuroretinitis cases are due to cat-scratch disease caused by Bartonella bacteria. Patients visual acuity can initially be 20/200 or worse in about 50% of cases, final visual acuity can be 20/40 or better in about 90% of cases. When neuroretinitis is suspected, it is important to document social history such as recent travel, contact with sick individuals or animals, and sexual activity. Some patients can have a central visual field defect and may manifest a relative afferent pupillary defect (RAPD). A slit lamp examination will reveal an inflamed optic disc and/or macular star pattern. Typically, only optic disc inflammation may be present in an early presentation of neuroretinitis as the macular star may take 1-2 weeks to manifest. A study on infectious neuroretinitis suggested that visual symptoms in neuroretinitis are due to macular pathology rather than optic nerve dysfunction. Directing treatment at the underlying etiology is the most effective course. If infectious etiology is suspected, a consultation with an infectious disease specialist may be recommended. Treatment for patients found to have cat-scratch disease can be approached with antibiotics and steroids can be added later to help resolve inflammation. Patients can have a significant response to treatment if diagnosed early, and see as well as 20/25, as in this case.

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