ENDOGENOUS ASPERGILLUS ENDOPHTHALMITIS IN AN IMMUNOCOMPETENT PATIENT WITH A REMOTE HISTORY OF PULMONARY TUBERCULOSIS.

PURPOSE: To report a case of Aspergillus fumigatus endogenous endophthalmitis in an immunocompetent patient initially diagnosed as acute retinal necrosis. METHODS: Case report. PATIENT: A 67-year-old woman with a remote history of treated pulmonary tuberculosis and no ocular history presented to an outside retina specialist with a sudden onset of floaters and blurred vision in one eye. Examination and fluorescein angiography at the time revealed findings suspicious for acute retinal necrosis, and the patient was started on oral valganciclovir and an intravitreal injection of ganciclovir. Despite treatment, the patient's vision and pain worsened. After evaluation at the University of Southern California Roski Eye Institute, she was diagnosed with a likely fungal endogenous endophthalmitis based on ultrasound findings and underwent emergent vitrectomy. A chest x-ray demonstrated partial collapse of the right upper lobe with hilar enlargement. RESULTS: Aspergillus fumigatus was cultured from vitreous, blood, and bronchoalveolar lavage samples, suggesting that the patient's infection had a pulmonary origin, most likely from the right upper lobe that had healed from previous tuberculosis infection. DISCUSSION: To the best of our knowledge, this is the first reported case of Aspergillus endogenous endophthalmitis in an immunocompetent patient secondary to pulmonary changes that occurred from previously treated tuberculosis.

Relationship of Epiretinal Membrane Formation and Macular Edema Development in a Large Cohort of Uveitic Eyes.

Purpose: To identify the temporal relationship and clinical characteristics of epiretinal membrane (ERM) and macular edema (ME) formation in uveitic eyes.Methods: A total of 269 subjects (444 uveitic eyes) met study inclusion criteria. Comprehensive ophthalmic examination, spectral domain-optical coherence tomography (SD-OCT), and clinical testing were carried out.Results: Of the 444 uveitic eyes, 229 eyes (51.6%) developed an ERM, whereas 87 eyes (19.1%) developed ME. The odds ratios (ORs) of systemic disease causing uveitis and resulting in ERM and ME were significantly higher in posterior uveitis (OR 6.56, 95% CI 2.98-14.46; p < .0001) and panuveitis (OR 10.09, 95% CI 4.05-25.15; p < .0001). Temporal analysis revealed that an ERM was noted concurrently or prior to ME development in 93.8% of eyes.Conclusions: ERM and ME are primarily observed in posterior uveitis and panuveitis associated with systemic diseases. The temporal relationship highlights the importance of characterization of ERM as it relates to the development of uveitic ME.

Two cases of uveitis associated with severe transaminitis during a Rickettsia typhi outbreak in Los Angeles County.

PURPOSE: To report the clinical presentation, multimodal imaging, and management of two patients with Rickettsia typhi infection who presented with transaminitis and bilateral uveitis. OBSERVATIONS: We report two cases of murine typhus-associated uveitis in the setting of a Rickettsia typhi outbreak in Los Angeles County. In case 1, a 29-year-old Hispanic female presented with scotoma of the right eye and bilateral floaters after 2 weeks of persistent fevers, maculopapular rash, and arthralgia. Clinical examination and optical coherence tomography (OCT) revealed vitreous cell and scattered white spots in both eyes at the level of the inner retina, and a cotton wool spot inferiorly in the left eye. Multiple hyperautofluorescent spots were seen on widefield fundus autofluorescence (FAF). Retinal vascular leakage and optic disc hyperfluorescence were visualized on widefield fluorescein angiography (FA). These findings were concerning for a white dot syndrome (WDS). The patient was started on oral prednisone 30 mg daily. Serologic testing during the convalescent phase returned positive for R. typhi infection and she was started on doxycycline. 3 weeks later, she reported complete resolution of scotoma and significant improvement of bilateral floaters.In the second case, a 42-year-old Hispanic male presented with sudden bilateral increased floaters and blurry vision after 12 days of persistent fever and headache. Clinical examination revealed trace flare with 1+ cell in the anterior chamber, 1+ vitreous cell, and multiple white dots in both eyes at the level of the inner retina. FAF showed scattered hyperautofluorescent spots in both eyes. FA demonstrated late retinal vascular leakage with bilateral hyperfluorescent optic discs. He was started on oral prednisone 40mg, prednisolone acetate 1% drops, and cyclopentolate 1% drops daily. 2 weeks later, serologic titers returned positive for murine typhus and he was started on doxycycline with gradual taper off of steroids. He subsequently had complete resolution of floaters, blurry vision, and the inner retinal white spots. CONCLUSIONS AND IMPORTANCE: Murine typhus-associated uveitis may present with scotoma and increased floaters in the setting of persistent fevers and transaminitis, with pre- or inner retinal white spots seen on fundus examination. Ophthalmologists may aid in prompt diagnosis and initiation of antibiotic therapy, which can shorten the course of the disease and in turn, reduce the risk of severe complications.

Thomas A. Swift's Electric Rifle Injuries to the Eye and Ocular Adnexa: The Management of Complex Trauma.

PURPOSE: To report the ocular and adnexal injuries sustained by patients with Thomas A. Swift's electric rifles (TASER; TASER International, Scottsdale, AZ), review the literature, and discuss the management of this complex trauma. DESIGN: Multicenter, retrospective case series and literature review. PARTICIPANTS: Seventeen eyes of 16 patients (5 eyes of 5 patients treated at 3 institutions, and 12 eyes of 11 previously reported cases). METHODS: The clinical data of 17 eyes were pooled. Spearman's correlation coefficient was used to assess the association between the extent of TASER injury and patient outcomes. MAIN OUTCOME MEASURES: Extent of TASER injury (zone of injury, penetrating vs. perforating) and association with patient outcomes (visual acuity [VA] and retinal detachment [RD]). RESULTS: In our cohort, 4 patients were transported by law enforcement and 1 was transferred from a community hospital. Four patients were taken to the operating room for TASER removal and globe repair; 1 patient underwent removal in the emergency room. Of 17 pooled cases, 12 (71%) involved open-globe injury. Of these, there was a high rate of zone 3 injuries (100%; n = 12) and a high incidence of RD (73%; 8 of 11, eviscerated eye excluded). Among patients with closed-globe injury (n = 5), 1 patient demonstrated exudative RD and 1 patient demonstrated retinal dialysis with RD. Of 10 patients with RD, 1 (10%) achieved resolution with monitoring (exudative RD); 1 (10%) underwent cryopexy and pneumatic retinopexy; 3 (30%) underwent vitrectomy, and 5 (50%) with poor prognosis did not undergo vitreoretinal surgery. In the 3 patients who underwent vitrectomy, all 3 (100%) demonstrated redetachment resulting from proliferative vitreoretinopathy and required additional surgery. Visual acuity on presentation was significantly correlated with final VA (ρ = 0.783; P = 0.02). Men (94%) were more likely than women (6%) to sustain TASER trauma. Median age was 26 years. There was a 50% rate of loss to follow-up. CONCLUSIONS: Thomas A. Swift's electric rifle injuries to the eyes or ocular adnexa represent complex trauma. Zone 3 injuries are common. The visual prognosis is guarded, and eyes may require multiple surgeries to preserve vision. Patients are at high risk for loss to follow-up by way of incarceration.

Machine Learning Models for Diagnosing Glaucoma from Retinal Nerve Fiber Layer Thickness Maps.

PURPOSE: To assess the diagnostic accuracy of multiple machine learning models using full retinal nerve fiber layer (RNFL) thickness maps in detecting glaucoma. DESIGN: Case-control study. PARTICIPANTS: A total of 93 eyes from 69 patients with glaucoma and 128 eyes from 128 age- and sex-matched healthy controls from the Los Angeles Latino Eye Study (LALES), a large population-based, longitudinal cohort study consisting of Latino participants aged ≥40 years residing in El Puente, California. METHODS: The 6×6-mm RNFL thickness maps centered on the optic nerve head (Cirrus 4000; Zeiss, Dublin, CA) were supplied to 4 different machine learning algorithms. These models included 2 conventional machine learning algorithms, Support Vector Machine (SVM) and K-Nearest Neighbor (KNN), and 2 convolutional neural nets, ResNet-18 and GlaucomaNet, which was a custom-made deep learning network. All models were tested with 5-fold cross validation. MAIN OUTCOME MEASURES: Area under the curve (AUC) statistics to assess diagnostic accuracy of each model compared with conventional average circumpapillary RNFL thickness. RESULTS: All 4 models achieved similarly high diagnostic accuracies, with AUC values ranging from 0.91 to 0.92. These values were significantly higher than those for average circumpapillary RNFL thickness, which had an AUC of 0.76 in the same patient population. CONCLUSIONS: Superior diagnostic performance was achieved with both conventional machine learning and convolutional neural net models compared with circumpapillary RNFL thickness. This supports the importance of the spatial structure of RNFL thickness map data in diagnosing glaucoma and further efforts to optimize our use of this data.

Comparison of surgical outcomes of trabeculectomy, Ahmed shunt, and Baerveldt shunt in uveitic glaucoma.

BACKGROUND: Uveitis is defined as a collection of syndromes involving intraocular inflammation which can lead to pain, tissue damage, and vision loss. Ophthalmic surgery in uveitis patients can be challenging due to inflammation-induced fibrosis and scarring. Trabeculectomy and implantation of glaucoma drainage devices (aqueous shunts) have been used in surgical management of uveitic glaucoma, however there is a paucity of literature examining the comparative results of these entities in this unique setting. The purpose of this retrospective comparative study is to compare clinical outcomes of trabeculectomy with MMC, Ahmed shunt, and Baerveldt shunt surgery specifically in uveitic glaucoma. RESULTS: Median IOP, IOP reduction, glaucoma medication use, and visual acuity at 6- and 12-month follow-up were similar across groups. Postoperative hypotony rate was significantly different across trabeculectomy (53%), Baerveldt (24%), and Ahmed (18%) groups (p = 0.027); other complication rates were similar. Baerveldt eyes had a lower failure rate compared to trabeculectomy (p = 0.0054) and Ahmed (p = 0.0008) eyes. CONCLUSIONS: While there was no difference in IOP reduction between trabeculectomy, Ahmed, and Baerveldt, Baerveldt eyes had the lowest failure rate.

Is it melanoma-associated retinopathy or drug toxicity? Bilateral cystoid macular edema posing a diagnostic and therapeutic dilemma.

PURPOSE: To report the clinical presentation, multimodal imaging, and management of a patient with metastatic melanoma who presented with cystoid macular edema (CME). OBSERVATIONS: We report a case of a 71-year-old Caucasian male with metastatic melanoma who presented with bilateral cystoid macular edema after being on treatment with a programmed T cell death ligand 1 inhibitor, MPDL3280, for 1 year. Multimodal imaging techniques, including color fundus photographs, autofluorescence, spectral domain optical coherence tomography (OCT), fluorescein angiography (Spectralis, Heidelberg, Germany), and spectral-domain OCT angiography (Zeiss; California, USA) were performed to evaluate the etiology of his CME and to monitor his response to treatment. Clinical examination and multimodal imaging revealed 1 + chronic vitreous cells, an epiretinal membrane, and mild macular edema in both eyes. Fundus autofluorescence showed paravenous hypoautofluorescence in the right eye and scattered hypoautofluorescent spots in the left eye. Optical coherence tomography angiography (OCTA) revealed mild drop out of superficial vessels in the peri-foveal region bilaterally. These findings were concerning for melanoma-associated retinopathy, drug-related uveitis, or activation of a previous chronic autoimmune process. The patient was started on prednisone 30 mg oral daily and ketorolac tromethamine 0.5% 1 drop four times daily. He was then treated with bilateral sustained-release dexamethasone intravitreal implants (Ozurdex). He had complete resolution of CME, and was tapered off of oral steroids within 6 weeks. CONCLUSIONS AND IMPORTANCE: Melanoma-associated retinopathy can be accompanied by CME, which presents a diagnostic and therapeutic dilemma in cases where a new drug has been recently initiated. By treating the condition locally, the ophthalmologist may be able to taper systemic immunosuppression more quickly.

OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY OF ACUTE MACULAR NEURORETINOPATHY REVEALS DEEP CAPILLARY ISCHEMIA.

PURPOSE: To present the multimodal retinal imaging findings, including optical coherence tomography (OCT) angiography, of an atypical case of acute macular neuroretinopathy (AMN). METHODS: The multimodal retinal imaging findings, including structural OCT and OCT angiography, of an atypical case of AMN are presented. RESULTS: A 23-year old woman, with a history of an unrelenting sinusitis treated with prednisone therapy and decongestants, presented with an acute scotoma of the right eye. Structural OCT illustrated an area of hyperreflectivity in the outer plexiform and outer nuclear layers nasal to the fovea, with associated disruption of the inner segment ellipsoid band consistent with AMN. At the 2-week follow-up visit, intraretinal fluid was noted with repeat OCT, with spontaneous resolution of the fluid at the 2-month follow-up visit. Optical coherence tomography angiography illustrated a flow void in the deep retinal capillary plexus associated with the AMN lesion. CONCLUSION: Atypical findings of AMN are presented in this case report including the presence and spontaneous resolution of intraretinal fluid. Although OCT angiography illustrated a flow deficit within the deep retinal capillary plexus, the cause of AMN remains elusive.

VISUAL AND ANATOMICAL OUTCOMES AFTER DIABETIC TRACTION AND TRACTION-RHEGMATOGENOUS RETINAL DETACHMENT REPAIR.

PURPOSE: To evaluate visual and anatomical outcomes of diabetic tractional retinal detachment repaired with pars plana vitrectomy. METHODS: Operative records were used to retrospectively identify all patients with tractional retinal detachments secondary to proliferative diabetic retinopathy surgically repaired with pars plana vitrectomy between November 1, 2009, and January 1, 2015 at the LAC + USC (Los Angeles County + University of Southern California) Medical Center. RESULTS: A total of 403 eyes with diabetic tractional retinal detachment in 359 patients were included. Successful reattachment of the retina was achieved in 87.6% of eyes after one surgery and 92.6% of eyes at the final follow-up. Best-corrected visual acuity at the final follow-up improved two or more lines in 56.3% of eyes, was stable in 23.8% of eyes, and decreased two or more lines in 19.9% of eyes. Eyes repaired with 23-gauge and 25-gauge vitrectomy systems had similar success rates as eyes treated with 20-gauge instrumentation (P = 0.73). Eyes receiving silicone oil tamponade had lower single-surgery reattachment rates (77.6% vs. 87.6%; P = 0.013), lower reattachment rates at the final follow-up (85.7% vs. 92.6%; P = 0.048), and higher rates of vision loss (34.7% vs. 19.9%; P < 0.0001) but were more likely to have concurrent rhegmatogenous detachment (47.0% vs. 21.3%; P < 0.0001) and macula involving detachment (74.5% vs. 60.0%; P < 0.0001). CONCLUSION: In this large, single-center retrospective study of patients with advanced diabetic tractional retinal detachment, vitrectomy achieved excellent anatomical outcome and improved or stabilized vision in 80.1% of eyes. Smaller gauge vitrectomy systems were found to have similar outcomes to 20-gauge instrumentation.

INTRAVENOUS IMMUNOGLOBULIN IN THE TREATMENT OF JUVENILE RETINITIS PIGMENTOSA-ASSOCIATED CYSTOID MACULAR EDEMA AND UVEITIS.

PURPOSE: To evaluate the efficacy of intravenous immunoglobulin in the treatment of cystoid macular edema and uveitis caused by juvenile retinitis pigmentosa in a woman interested in planning a pregnancy. METHODS: Descriptive case report. A 25-year-old woman with retinitis pigmentosa presented with decreased visual acuity, interested in a second opinion. RESULTS: Intravenous immunoglobulin therapy was begun at 2-week intervals. Visual acuity improved from 20/100 to 20/70 by 6 weeks, where it stabilized for the remaining year. Serial optical coherence tomography imaging revealed marked improvement in cystoid macular edema after 2 treatments of intravenous immunoglobulin at 2 weeks of follow-up, with complete resolution noted at 4 months, a finding that persisted at 1 year of follow-up. CONCLUSION: Our findings support the use of intravenous immunoglobulin as a therapeutic option for the resolution of cystoid macular edema and management of uveitis secondary to juvenile retinitis pigmentosa.

Sickle cell disease and the eye.

PURPOSE OF REVIEW: To review recent literature pertaining to sickle cell retinopathy (SCR) and, in particular, sickle cell maculopathy. RECENT FINDINGS: Several recent studies suggest that macular perfusion abnormalities seen in patients with sickle cell disease of various genotypes may affect both the superficial and deep capillary plexi, with a predilection for the deep capillary plexus. Further, these changes may be associated with areas of macular thinning, as well as with peripheral retinal ischemia, even in individuals without visual symptoms, contrary to what has previously been described in both diabetic retinopathy and retinal vein occlusion. Several cases also suggest that paracentral acute middle maculopathy may be the pathophysiologic mechanism by which microvascular occlusion leads to macular thinning. SUMMARY: Sickle cell disease can manifest in a number of ways within the orbit as well as intraocularly because of its nonspecific vasoocclusive episodes. However, SCR is the most common ophthalmic manifestation of this disease. Historically, SCR has been considered a peripheral retinopathy, but the development and use of spectral-domain optical coherence tomography and optical coherence tomography angiography suggest that significant macular vascular changes occur early in this disease, even in asymptomatic individuals.

Parylene scaffold for cartilage lesion.

Evaluate parylene scaffold feasibility in cartilage lesion treatment, introducing a novel paradigm combining a reparative and superficial reconstructive procedure. Fifteen rabbits were used. All animals had both knees operated and the same osteochondral lesion model was created bilaterally. The parylene scaffold was implanted in the right knee, and the left knee of the same animal was used as control. The animals were euthanized at different time points after surgery: four animals at three weeks, three animals at six weeks, four animals at nine weeks, and four animals at 12 weeks. Specimens were analyzed by International Cartilage Repair Society (ICRS) macroscopic evaluation, modified Pineda histologic evaluation of cartilage repair, and collagen II immunostaining. Parylene knees were compared to its matched contra-lateral control knees of the same animal using the Wilcoxon matched-pairs signed rank. ICRS mean ± SD values for parylene versus control, three, six, nine and twelve weeks, respectively: 7.83 ± 1.85 versus 4.42 ± 1.08, p = 0.0005; 10.17 ± 1.17 versus 6.83 ± 1.17, p = 0.03; 10.89 ± 0.60 versus 7.33 ± 2.18, p = 0.007; 10.67 ± 0.78 versus 7.83 ± 3.40, p = 0.03. Modified Pineda mean ± SD values for parylene versus control, six, nine and twelve weeks, respectively: 3.37 ± 0.87 versus 6.94 ± 1.7, p < 0.0001; 5.73 ± 2.05 versus 6.41 ± 1.7, p = 0.007; 3.06 ± 1.61 versus 6.52 ± 1.51, p < 0.0001. No inflammation was seen. Parylene implanted knees demonstrated higher collagen II expression via immunostaining in comparison to the control knees. Parylene scaffolds are a feasible option for cartilage lesion treatment and the combination of a reparative to a superficial reconstructive procedure using parylene scaffolds led to better results than the reparative procedure alone.

Choroidal neovascularization secondary to tuberculosis: Presentation and management.

PURPOSE: While there are many known etiologies of choroidal neovascularization (CNV), tuberculosis is not a well-known causative agent. In this case series, we highlight CNV occurring secondary to tuberculous chorioretinitis, its presentation, and its management. OBSERVATIONS: We retrospectively reviewed the charts and imaging of four patients who presented with presumed tuberculous chorioretinitis and CNV. Three of these patients had signs of intraocular inflammation and were also found to have active macular CNV. The one remaining patient had chorioretinal scars from prior posterior uveitis and previously treated macular CNV membranes. The three patients with active disease were started on anti-tuberculosis medications and oral corticosteroids, and they also received intravitreal anti-vascular endothelial growth factor (VEGF) injections as needed for the CNV. There was a significant improvement in the clinical course of all three patients with active disease-the intraocular inflammation subsided, and CNV recurrences were mitigated. Upon completion of systemic treatment, all patients have remained quiescent. CONCLUSIONS AND IMPORTANCE: Our findings demonstrate that CNV may occur in the course of tuberculous chorioretinitis with marked loss of vision, and management with anti-tuberculosis medications, oral corticosteroids, and intravitreal anti-VEGF injections results in notable improvement in their clinical course.

SEVERE BILATERAL RETINAL VASCULAR OCCLUSION AS FIRST PRESENTATION OF SYSTEMIC LUPUS ERYTHEMATOSUS AND ANTIPHOSPHOLIPID SYNDROME.

PURPOSE: To describe a patient with undiagnosed systemic lupus erythematosus and secondary antiphospholipid syndrome presenting with bilateral severe retinal vaso-occlusion on first presentation. METHODS: Retrospective case report. A 42-year-old Hispanic female with no medical history presented with 2 weeks of bilateral decreased vision. RESULTS: At initial presentation, the patient's best-corrected visual acuity was 2 ft/200 and 20/400 in the right and left eyes, respectively. Dilated fundus examination revealed diffuse, confluent cotton-wool spots, and severe vascular occlusions in both eyes. Fluorescein angiography confirmed that the patient had both arterial and venous vascular occlusions and retinal vasculitis. A thorough work-up revealed that the patient had systemic lupus erythematosus and APS with positive antibodies to anticardiolipin, beta-2-glycoprotein, and lupus anticoagulant. The patient was treated with steroids, immunosuppression agents, anticoagulation, and extensive panretinal photocoagulation. One month later, best-corrected visual acuity had improved to 20/200 and 20/40, the vasculitis had substantially improved, and no neovascularization was present. CONCLUSION: Severe retinal vaso-occlusion may be the first presenting signs for patients with systemic lupus erythematosus and APS.

THE SECOND BLIND SPOT: SMALL RETINAL VESSEL VASCULOPATHY AFTER VACCINATION AGAINST NEISSERIA MENINGITIDIS AND YELLOW FEVER.

PURPOSE: To describe a case of small retinal vessel vasculopathy postvaccination. METHODS: We report the case of a 41-year-old white man who presented with a "second blind spot," describing a nasal scotoma in the right eye that started 4 days after vaccinations against Neisseria meningitidis and the yellow fever virus, and after a 2-month period of high stress and decreased sleep. Clinical examination, Humphrey visual field testing, and multimodal imaging with fundus photographs, autofluorescence, fluorescein angiography, and spectral domain optical coherence tomography and angiography were performed. RESULTS: Clinical examination revealed a well-circumscribed, triangular area of retinal graying of about 1-disk diameter in size, located at the border of the temporal macula. This corresponded to a deep scotoma similar in size to the physiologic blind spot on Humphrey visual field 24-2 testing. There was mild hypoautofluoresence of this lesion on autofluorescence, hypofluorescence on fluorescein angiography, and focal attenuation of a small artery just distal to the bifurcation of an artery supplying the involved area. Spectral domain optical coherence tomography through the lesion conveyed hyperreflectivity most prominent in the inner and outer plexiform layers, with extension of the hyperreflectivity into the ganglion cell and inner nuclear layers. Spectral domain optical coherence tomography angiography demonstrated arteriolar and capillary dropout, more pronounced in the superficial retinal layer compared to the deeper retinal layer. At 1-month follow-up, his scotoma improved with monitoring, with reduction from -32 dB to -7 dB on Humphrey visual field testing. There was clinical resolution of the area of graying and decreased hyperreflectivity on spectral domain optical coherence tomography, with atrophy of the inner retina. Spectral domain optical coherence tomography angiography showed progression of arteriolar and capillary dropout, more so in the superficial than in the deep capillary plexus. CONCLUSION: We describe a case of small artery occlusion in a previously healthy patient, 4 days after vaccination against N. meningitidis and yellow fever. Fluorescein angiography yielded greater diagnostic value than OCTA for evaluating the occlusion, whereas spectral domain optical coherence tomography angiography enabled better visualization of capillary dropout and layer-specific assessment. Further research is required to determine whether vaccination in general, or directed specifically at N. meningitidis or yellow fever, is associated with small vessel vasculopathy and the underlying pathogenesis.

Ultra-Wide-Field Fluorescein Angiography in Microscopic Polyangiitis.

A 25-year-old Hispanic female presented with 5 months of dry eyes and 2 months of bilateral photophobia and decreased vision. On examination, she had bilateral anterior uveitis and mild disc edema of the left eye. A complete infectious and inflammatory work-up was positive for elevated antinuclear antibodies and p-ANCA, leading to a diagnosis of microscopic polyangiitis. One year after initial treatment and steroid taper, an ultra-wide-field fluorescein angiography revealed peripheral vasculitis, outside of the standard traditional field of view, leading to an increase in immunomodulatory therapy and illustrating the utility of wide-field angiography for managing patients with uveitis.

Quantifying Retinal Microvascular Changes in Uveitis Using Spectral-Domain Optical Coherence Tomography Angiography.

PURPOSE: To quantify retinal capillary density and morphology in uveitis using spectral-domain optical coherence tomography angiography (SD-OCTA). DESIGN: Cross-sectional, observational study. METHODS: Healthy and uveitic subjects were recruited from 2 tertiary care eye centers. Prototype SD-OCTA devices (Cirrus; Carl Zeiss Meditec, Inc, Dublin, California, USA) were used to generate 3 × 3-mm2 OCTA images centered on the fovea. Subjects were placed into 3 groups based on the type of optical microangiography (OMAG) algorithm used for image processing (intensity and/or phase) and type of retinal segmentation (automatic or manual). A semi-automated method was used to calculate skeleton density (SD), vessel density (VD), fractal dimension (FD), and vessel diameter index (VDI). Retinal vasculature was assessed in the superficial retinal layer (SRL), deep retinal layer (DRL), and nonsegmented retinal layer (NS-RL). A generalized estimating equations model was used to analyze associations between the OCTA measures and disease status within each retinal layer. A P value < .05 was accepted as significant. Reproducibility and repeatability were assessed using the intraclass correlation coefficient (ICC). RESULTS: The SD, VD, and FD of the parafoveal capillaries were lower in uveitic eyes compared with healthy eyes in all retinal segments. In addition, SD and VD were significantly lower in the DRL of subjects with uveitic macular edema. There was no correlation in any capillary parameters and anatomic classification of uveitis. CONCLUSIONS: Quantitative analysis of parafoveal capillary density and morphology in uveitis demonstrates significantly lower capillary density and complexity. SD-OCTA algorithms are robust enough to detect these changes and can provide a novel diagnostic index of disease for uveitis subjects.

Retinopathy in lupus transitioned to Kikuchi-Fujimoto disease.

PURPOSE: We present a patient with systemic lupus erythematosus with significant vaso-occlusive retinal findings mimicking antiphospholipid antibody syndrome, who developed Kikuchi-Fujimoto disease. OBSERVATIONS: Our patient was initially diagnosed with systemic lupus erythematosus with antiphospholipid antibody syndrome given consistent serologic markers and profound retinal vascular ischemia. However, on subsequent follow up, she presented with fever and lymphadenopathy and underwent lymph node biopsy, which declared histologic findings of Kikuchi-Fujimoto disease. Repeat markers for antiphospholipid antibody syndrome were negative and she was taken off lifelong anticoagulation. CONCLUSIONS AND IMPORTANCE: Systemic lupus erythematosus and Kikuchi-Fujimoto disease may have many similar features and even biomarkers, and given the potential overlap of presentation, clinicians must carefully distinguish between these diseases to prevent unnecessary treatment.

Clinical and laboratory characteristics of ocular syphilis: a new face in the era of HIV co-infection.

BACKGROUND: Ocular syphilis is reemerging as an important cause of uveitis in the new era of common co-infection with HIV. This study will reveal the clinical and laboratory characteristics in the group of individuals co-infected with ocular syphilis and HIV compared with HIV-negative individuals. In this retrospective observational case series, medical records of patients diagnosed with ocular syphilis with serologic support from 2008 to 2014 were reviewed. Ocular and systemic manifestation and laboratory profiles were reviewed. RESULTS: Twenty-nine eyes of 16 consecutive patients (10 HIV-positive and 6 HIV-negative) were included. All patients were males, and mean age of onset for ocular syphilis was 43 (mean 42.65 ± 13.13). In both HIV-positive and HIV-negative groups, ocular manifestations of syphilis were variable including anterior uveitis (4 eyes), posterior uveitis (8 eyes), panuveitis (13 eyes), and isolated papillitis (4 eyes). In HIV-positive patients, panuveitis was the most common feature (12/18 eyes, 67 %) and serum rapid plasma reagin (RPR) titers were significantly higher (range 1:64-1:16,348; mean 1:768; p = 0.018) than in HIV-negative patients. Upon the diagnosis of ocular syphilis in HIV-positive patients, HIV-1 viral load was high (median 206,887 copies/ml) and CD4 cell count ranged from 127 to 535 cells/ml (mean 237 ± 142; median 137). Regardless of HIV status, cerebrospinal fluid (CSF) exam was frequently abnormal: positive CSF fluorescent treponemal antibody absorption (FTA-ABS) or Venereal Disease Research Laboratory (VDRL) test results in seven patients or either elevated CSF WBC count or elevated CSF protein in six patients. CONCLUSIONS: Our results reveal that the patients with ocular syphilis with high serum RPR titers may have concomitant HIV infection requiring further testing for HIV status and ocular syphilis is likely associated with the central nervous system involvement and therefore needs to be managed according to the treatment recommendations for neurosyphilis.