Malignancy Risk Associated With the Use of Systemic Immunomodulatory Therapy in the Management of Noninfectious Uveitis.

PURPOSE: Patients with noninfectious uveitis (NIU) can require treatment with systemic immunomodulatory therapy (IMT), but it is unclear whether IMT drug categories increase the risk of malignancy in NIU patients. The purpose of this study is to determine if the use of systemic IMT in patients with NIU is associated with an increased risk of malignancy. DESIGN: Clinical cohort study. METHODS: Patients were identified from a US administrative medical claims database including some Medicare Advantage and commercial plans, from 2000 to 2022. About 318,498 NIU patients were identified. Enrollees were included in the analysis if they met the following criteria: continuous enrollment in the plan for at least 1 year, and at least 2 consecutive visit diagnoses of any type of NIU, after initiation of systemic IMT. We compared the rates of incident malignancy in NIU patients treated with IMT versus the rates among NIU patients not treated with IMT. Multivariable Cox regression models were used to predict the hazard of developing incident cancer. RESULTS: Of the 318,498 patients with NIU identified over a 15-year period, 318,006 did not develop malignancy, and 492 did develop malignancy. Of the patients that developed a malignancy, 280 (57%) were treated with systemic corticosteroids; 204 (41%) were treated with antimetabolites; 44 (9%) were treated with T cell inhibitors; 108 (22%) were treated with TNF alpha inhibitors; 2 (0.004%) were treated with interleukin-6 (IL-6) inhibitors; and 1 was treated with CD-20 antibodies. There were no malignancies reported in the group treated with alkylating agents. There was no association between any of the drug classes and incidence of malignancy. CONCLUSIONS: This study suggests that there is no increased risk of malignancy associated with the use of systemic IMT for patients with NIU.

Carbonic anhydrase inhibitors limit complications in X-linked retinoschisis.

Purpose: Carbonic anhydrase inhibitors (CAIs) reduce macular schisis in patients with X-linked retinoschisis (XLRS). The purpose of this study was to determine if CAIs reduce the incidence of complications from XLRS, including macular atrophy, retinal tears, and retinal detachment (RD), the most common causes of vision loss in patients with XLRS. Methods: For this retrospective interventional case series, a chart review of patients examined at Cincinnati Children's Hospital Medical Center [CCHMC] and Cincinnati Eye Institute [CEI] between 1/1/2015 and 1/16/2023 was performed. Male patients were included based on genetically-confirmed RS1 or typical clinical presentation with known family history of XLRS with at least two follow-up visits. Results: Twenty-eight patients (56 eyes) with XLRS were included. There were 10 RS1 variants among the 21 genotyped patients. Median age at clinical diagnosis was 10.4 years old (range: 0.4-55.7 years) with median follow-up time of 4.7 years (range: 0.2-38.3 years). Median presenting Snellen visual acuity was 20/60 (logMAR 0.48, range: 0.18-3). In 26 eyes of 15 patients treated with CAIs, median CST pre-treatment was 416 microns (range: 198-701 microns), and median percentage decrease in CST on treatment was 21.8% (range: 0-74.5%) from highest pre-treatment CST. Reduction in CST with CAI use was statistically significant (p = 0.02), but not logMAR VA (p = 0.64). There was no significant difference in CST between patients treated with topical vs. oral CAI (p = 0.95) or between patients with partial or complete CAI adherence (p = 0.60). Ten eyes of seven patients had an RD requiring surgical intervention. No treated eyes developed new macular atrophy, peripheral retinoschisis, retinal tears, or RD; two eyes on topical CAIs had spontaneous resolution of bullous peripheral retinoschisis. Conclusion: During the follow-up period, patients taking CAIs reduced macular schisis and did not experience new complications of macular atrophy, retinal tears, or RD. This is a relatively large cohort with long-term follow-up periods for patients with XLRS. Reduced macular schisis may not require perfect adherence with CAIs. A large, prospective, randomized, controlled clinical trial is needed to determine the potential of CAIs to improve visual function, reduce retinoschisis, and prevent RD.

Multimodal Imaging of Panuveitis with Multifocal Retinitis Resulting from Post-Streptococcal Uveitis Syndrome.

BACKGROUND: Post-streptococcal uveitis syndrome is a rare inflammatory sequela following streptococcus infection most commonly occurring in young patients. Patients most typically present with bilateral anterior nongranulomatous uveitis, but posterior involvement occurs frequently. CASE PRESENTATION: We report an immunosuppressed patient presenting with asymmetrically bilateral acute onset, panuveitis with diffuse multifocal retinitis resulting from post-streptococcal uveitis syndrome. CONCLUSION: Post-streptococcal uveitis syndrome can present as a panuveitis with multifocal retinitis. A high clinical suspicion is needed for this diagnosis especially in patients under the age of 30 years old with bilateral non-granulomatous uveitis.

CASE OF TORPEDO MACULOPATHY WITH TWO DISTINCT ZONES OF THE RETINAL PIGMENT EPITHELIUM.

BACKGROUND/PURPOSE: To report a case of torpedo maculopathy with two distinct zones of the retinal pigment epithelium visualized on optical coherence tomography. METHODS: Observational case report. RESULTS: A 6-year-old female presented for a routine examination. Visual acuity was 20/20 bilaterally. Dilated fundus examination was normal in the right eye. Dilated fundus examination of the left eye showed a wedge-shaped area of hypopigmentation in the temporal macula. Optical coherence tomography macula of the left eye showed outer retinal cavitation with segmentation of the foveal retinal pigment epithelium into a superficial fluffy zone and a deeper hyperreflective zone. CONCLUSION: This case helps contribute to the growing body of the torpedo maculopathy literature that may reveal different stages of the same disease evolving over time.

BILATERAL ENDOGENOUS ENDOPHTHALMITIS FROM STREPTOCOCCUS PNEUMONIAE.

PURPOSE: To report a case of bilateral endogenous endophthalmitis from Streptococcus pneumoniae with devastating sequelae. METHODS: Interventional case report. RESULTS: A 56-year-old man presented with acute bilateral blurred vision and floaters with fever and hemodynamic instability. Previously, he was diagnosed with acute otitis externa and reported manually extracting several of his own teeth. He underwent a vitreous tap and intravitreal antibiotic injections. Blood and vitreous cultures were positive for S. pneumoniae. The patient later developed life-threatening medical sequelae. His final visual acuity was no light perception in the right eye and 20/25 in the left eye. CONCLUSION: Diagnosing endogenous endophthalmitis early is essential to initiating a systemic evaluation for potentially life-threatening medical conditions, including sepsis, endocarditis, and osteomyelitis. A high degree of suspicion, expeditious treatment, and interdisciplinary collaboration are essential to maximizing patient outcomes.

Bilateral pulmonary emboli following macular hole surgery with postoperative prone positioning.

PURPOSE: To report a rare case of bilateral pulmonary emboli following pars plana vitrectomy with postoperative prone positioning. OBSERVATIONS: A 60 year-old female presented with a fourmonth history of unilateral distorted vision. Ocular coherence tomography revealed a full thickness macular hole. Two weeks later, the patient underwent a 23-gauge pars plana vitrectomy with internal limiting membrane peeling and 12% perfluoropropane gas tamponade. Postoperatively, she completed two weeks of prone positioning. Five days later, she presented with a two day history of abdominal pain and shortness of breath. Computed tomography angiography revealed bilateral pulmonary emboli. The patient received six months of anticoagulation to prevent further thromboembolic events. CONCLUSIONS: Life threatening blood clots can form due to prolonged immobilization from prone positioning. Patients should be educated to perform light exercise postoperatively to prevent complications of blood stasis.