Whipple disease is a rare systemic infection of Tropheryma whipplei with nonspecific clinical features. Ocular involvement is uncommon but has been reported in both anterior and posterior segments of the eye. We present the case of a 70-year-old man with a history of cutaneous T-cell lymphoma, who developed unilateral ocular inflammation with hypopyon, vitritis, and migrating nummular retinal lesions. Despite intravitreal antimicrobial therapy, inflammation persisted. Four months later, he developed systemic symptoms and findings on duodenal biopsy consistent with Whipple disease. This case highlights an atypical ocular presentation suspected to be associated with Whipple disease and the diagnostic challenges in immunocompromised patients lacking classical gastrointestinal symptoms.

BACKGROUND:To present two separate cases of occlusive retinal vascular disease with secondary cystoid macular edema in patients with a past medical history significant for hereditary spherocytosis and splenectomy. CASE PRESENTATION:We present two patients with atypical ocular changes in the setting of a distant history of splenectomy with underlying hereditary spherocytosis. The first patient is a 52-year-old Caucasian male with findings consistent with adult-onset Coats disease, predominantly presenting as type 1 macular telangiectasia. The second patient is a 79-year-old Caucasian female with acute branch retinal vein occlusion with a remote history of bilateral nonarteritic anterior ischemic optic neuropathy. CONCLUSION:There is limited literature exploring the association between retinal vascular disease, hereditary spherocytosis, and splenectomy. These cases highlight that the link between vascular events and a remote history of splenectomy remains inconclusive, especially in the absence of evidence to differentiate it from other potential diagnoses. Both patients in our cases had secondary macular edema, which responded to treatment with intravitreal antivascular endothelial growth factor medications.

Purpose To report the findings supported by multimodal imaging in a case of secondary vitreoretinal lymphoma presenting with inner retina and optic nerve head infiltration. Observations A 64-year-old man with systemic diffuse large B-cell lymphoma presented with reduced visual acuity. Moderate anterior chamber and vitreous cell were present. Fundus exam showed bilateral disc edema and diffuse opaque macular infiltrates with a pseudo cherry-red spot in the left eye. Optical coherence tomography showed inner retinal infiltration and loss of normal architecture. Surgery for tissue biopsy was discussed and declined due to risk. Instead, multimodal imaging and anterior chamber fluid sampling were used as a surrogate for tissue biopsy and helped rule out infectious uveitis and retinal vascular disease. The patient was empirically treated with intravitreal methotrexate with rapid improvement in vision, exam, and quality of life. Conclusions and importance Multimodal imaging can support a presumed diagnosis of secondary vitreoretinal lymphoma in order to proceed with intravitreal methotrexate treatment, which can result in rapid clinical and visual improvement.

Purpose: To describe the efficacy of belzutifan as a treatment for juxtapapillary retinal hemangioblastomas in patients with von Hippel-Lindau disease. Methods: A case and its findings were analyzed, and a systematic literature review was conducted using PubMed and Ovid MEDLINE. Results: At a routine follow-up, a 63-year-old woman with a history of von Hippel-Lindau disease and slowly progressive bilateral juxtapapillary retinal hemangioblastomas presented with decreased visual acuity (VA) in the right eye resulting from significant lesion growth and an increase in central macular edema and exudate. Oral belzutifan therapy was initiated. A significant bilateral regression and decrease in tumor size, improved macular thickening and edema, and improved VA were seen over a 6-month period. Conclusions: The current literature on the therapeutic effects of oral belzutifan is limited; however, recent reports have been promising. This case shows the potential efficacy of belzutifan as a first-line treatment for vision-threatening juxtapapillary retinal hemangioblastomas in patients with von Hippel-Lindau disease.

Abstract Objective Uveitis is an inflammatory process of the eye that can lead to severe vision loss. Non-infectious forms of uveitis constitute the majority of cases and chronic disease is typically treated with immunosuppressive drugs. A minority of uveitis cases are of an infectious cause, and in these cases, the use of immunosuppressive agents may aggravate the underlying infection. Design: Retrospective case series. Result. Five patients who initially presented with ocular findings suspicious for non-infectious uveitis and who were treated with corticosteroids. After failing to respond to corticosteroid therapy, and with a history of injection drug use (IDU) eventually reported by all five patients, there was a high degree of clinical suspicion for an underlying infectious process. All five were later classified as having endogenous endophthalmitis related to IDU, and experienced clinical improvement after antibiotic therapy and pars plana vitrectomy. Conclusions and Importance. Given the ongoing opioid crisis, this study demonstrates the importance of considering IDU-associated endogenous endophthalmitis in any individual with intraocular inflammation and history of IDU presenting with new-onset vision loss, irrespective of time since last reported use.

We describe the clinical features and treatment outcome of a patient with combined central retinal vein occlusion and cilioretinal artery occlusion. A 52-year-old female presented to our clinic with decreased vision in the right eye for 4 days. Visual acuity and intraocular pressure were count fingers at 2&1/2M and 14 mm Hg in the right and 20/20 and 16 mm Hg in the left eye, respectively. Funduscopic exam and optical coherence tomography (OCT) of the right eye confirmed the diagnosis of concurrent cilioretinal artery occlusion and central retinal vein occlusion with segmental macular pallor in the territory of the cilioretinal artery, corresponding marked inner retina thickening on OCT and signs of vein occlusion. The patient received an intravitreal injection of bevacizumab and at 1-month follow-up, vision improved to 20/30 with corresponding anatomical improvement. It is very important to recognize combined central retinal vein occlusion and cilioretinal artery occlusion as they could be treated with intravitreal injections of anti-vascular endothelial growth factors with favorable treatment outcomes.

We report the case of a 35-year-old female with combined Chiari 1 malformation and basilar invagination, who presented with intermittent conjunctival chemosis and unilateral chorioretinal folds that were temporally correlated. She denied any flashes, floaters, eye redness, or pain. She also denied nausea or vomiting. Clinical exam and optical coherence tomography imaging revealed conjunctival chemosis and chorioretinal folds in the left eye. Subsequent magnetic resonance imaging of the brain and the orbits were consistent with combined Chiari 1 malformation and basilar invagination. The unilateral and intermittent chorioretinal folds and conjunctival chemosis presentation of combined Chiari 1 malformation and basilar invagination is unusual. To the best of our knowledge, this is the first case to be reported with this unique clinical presentation. It is most important to be aware that unilateral and intermittent chorioretinal folds associated with conjunctival chemosis may be signs of intracranial disease.

PURPOSE:To describe multiple ocular (and non-ocular) manifestations of disease that can present in a person who injects drugs (PWID). We report a case of a patient consecutively presenting across multiple visits to an ambulatory eye care clinic as the initial point of contact for endogenous endophthalmitis, fungal keratitis, bacteremia, and psoas abscess with vertebral osteomyelitis within a matter of weeks.OBSERVATIONS:A 51-year-old male with past medical history of alcohol use disorder and injection drug use was initially seen in an eye clinic three days after suffering vision loss in the left eye associated with floaters, photophobia, and eye pain. After initial workup and treatment for panuveitis, endogenous endophthalmitis was suspected. A pars plana vitrectomy was performed, and intravitreal medications were given. A pathogen was never isolated from vitreous samples. Two weeks later, the patient presented with complaints of pain, blurry vision, and foreign body sensation in his opposite (right) eye. Examination revealed a corneal ulcer later identified as a Paecliomyces fungal infection. Two weeks after this, he developed fever, chills, and right-sided flank pain radiating to his testicles. Following evaluation by the emergency department and subsequent hospitalization after bacteremia was noted, he was found to have a right-sided psoas abscess with lumbar vertebral osteomyelitis. Fluid was drained, cultured, and grew methicillin-sensitive Staphylococcus aureus (MSSA). At his last visit, his best-corrected visual acuity was 20/20 OS and 20/30 OD despite central corneal scarring. It was only after hospitalization that he affirmed recent injection drug use, despite being queried about it through the course of his infections.CONCLUSIONS AND IMPORTANCE:Injection drug use is an increasingly common concern for all healthcare providers as the opioid crisis in the United States remains widespread. This case highlights multiple potential infectious processes which may impact persons who inject drugs when seen by eye care providers. It also describes difficulties in caring for people who inject drugs who may not provide critical and timely information relating to their injection drug use and/or may delay care even when faced with potentially vision- and/or life-threatening conditions.

PURPOSE OF REVIEW:The United States has experienced a dramatic rise in opioid and injection drug use over the past 2 decades. A public health emergency was declared in 2017 and subsequently, there have been several new reports on the rise of endogenous endophthalmitis specifically associated with injection drug use. The purpose of this review is to provide a current perspective of the ocular harms posed by injection drug use.RECENT FINDINGS:The opioid epidemic has prompted several new studies from New England, one of the US regions most heavily affected, that examine the trends and characteristics of injection drug use-associated endogenous endophthalmitis. Patients may delay seeking care and may be infected with a variety of rare and atypical microbes, and as a result clinical appearance may vary widely. Injection drug use also leads to embolic phenomena such as talc retinopathy and septic emboli from endocarditis. HIV is highly associated with injection drug use and although HAART has drastically reduced the morbidity and mortality of HIV-associated infections, a variety of ocular disease may accompany an immunocompromised patient.SUMMARY:Healthcare providers must remain vigilant in the recognition of injection drug use patients with vision loss and ocular inflammation to ensure prompt medical and/or surgical treatment.

most commonly selected preferred treatment, many respondents indicated that more evidence was needed to guide treatment choices. 4The MUTT I result was driven primarily by ulcers caused by Fusarium, which had the worst response to voriconazole. 1 After the results of MUTT I and 2 subsequent trials, 2,3 only a few experts indicated that they would prefer to treat Fusarium keratitis with voriconazole.Limitations of this study include that respondents self-reported practice patterns and it was not possible to validate the survey against actual practice patterns.Respondents may have underreported voriconazole use because of social desirability bias.The decrease in preference for voriconazole over time may indicate that randomized clinical trials led to changes in practice patterns and integration of evidence-based practices into the treatment of fungal keratitis.

PURPOSE. Traumatic brain injury (TBI) frequently leads to chronic visual dysfunction. The purpose of this study was to investigate the effect of TBI on retinal ganglion cells (RGCs), and to test whether treatment with the novel neuroprotective compound P7C3-S243 could prevent in vivo functional deficits in the visual system.METHODS. Blast-mediated TBI was modeled using an enclosed over-pressure blast chamber. The RGC physiology was evaluated using a multielectrode array and pattern electroretinogram (PERG). Histological analysis of RGC dendritic field and cell number were evaluated at the end of the study. Visual outcome measures also were evaluated based on treatment of mice with P7C3-S243 or vehicle control.RESULTS. We show that deficits in neutral position PERG after blast-mediated TBI occur in a temporally bimodal fashion, with temporary recovery 4 weeks after injury followed by chronically persistent dysfunction 12 weeks later. This later time point is associated with development of dendritic abnormalities and irreversible death of RGCs. We also demonstrate that ongoing pathologic processes during the temporary recovery latent period (including abnormalities of RGC physiology) lead to future dysfunction of the visual system. We report that modification of PERG to provocative postural tilt testing elicits changes in PERG measurements that correlate with a key in vitro measures of damage: the spontaneous and light-evoked activity of RGCs. Treatment with P7C3-S243 immediately after injury and throughout the temporary recovery latent period protects mice from developing chronic visual system dysfunction.CONCLUSIONS. Provocative PERG testing serves as a noninvasive test in the living organism to identify early damage to the visual system, which may reflect corresponding damage in the brain that is not otherwise detectable by noninvasive means. This provides the basis for developing an earlier diagnostic test to identify patients at risk for developing chronic CNS and visual system damage after TBI at an earlier stage when treatments may be more effective in preventing these sequelae. In addition, treatment with the neuroprotective agent P7C3-S243 after TBI protects from visual system dysfunction after TBI.