Intraocular sarcoid-like reaction in patients with chronic lymphocytic leukemia.

OBJECTIVE: To investigate the frequency and clinical features of intraocular paraneoplastic sarcoid-like reaction (SLR) in patients with chronic lymphocytic leukemia (CLL). METHODS: Retrospective review of patients with CLL from January 1, 1980, to December 31, 2020. Eye examinations were searched for 22 keywords suggestive of SLR, and charts were manually reviewed. RESULTS: Of 4209 unique patients with CLL, 1021 (24%) had at least 1 eye examination on record, and 324 (8%) had 1 or more keyword eye examination findings. After manual review, 12 patients (<1%) were identified as having probable SLR with characteristic features (n = 7), possible but not classic (n = 1), or suspect but less likely (n = 4). All patients (n = 8) with probable or possible SLR were White, and half (n = 4) were male. Intraocular SLR was diagnosed a mean of 49.7 months after the CLL diagnosis (n = 7) or preceded the CLL diagnosis by 1 month (n = 1). Involvement was bilateral in 5 patients, with 13 total affected eyes and mean presenting Snellen visual acuity of 20/50. Common characteristic features on initial examination included vitreous cell (n = 13), anterior-chamber cell (n = 10), keratic precipitates (n = 9), posterior synechiae (n = 6), chorioretinal lesions (n = 5), and vitreous haze (n = 5). Treatment included topical corticosteroids alone (n = 5), with sub-Tenon corticosteroids (n = 1), or with steroid-sparing immunosuppressive agents (n = 1) or oral corticosteroids alone (n = 1). After a mean follow-up of 19.8 months, final mean visual acuity was 20/30. CONCLUSION: Intraocular SLRs affect fewer than 1% of patients with CLL. SLR should be on the differential diagnosis list for any CLL patient with ocular complaints, and most patients can be managed successfully with corticosteroids.

BILATERAL EYE PATCHING MAY IMPROVE CLINICAL OUTCOMES FOR ACUTE PRIMARY RHEGMATOGENOUS RETINAL DETACHMENT.

PURPOSE: Evaluate preoperative bilateral eye patching (BEP) on subretinal fluid and vision in acute primary rhegmatogenous retinal detachments (RRDs). METHODS: Retrospective nonrandomized interventional study of 335 patients with RRD undergoing BEP until surgery (BEP cohort) and separated by the percentage of full-time compliance: high (≥90%), medium (>90% but ≥50%), and low (<50%). Those declining BEP were included (control). All underwent surgery and were followed for ≥3 months. Imaging was obtained immediately before surgery. Best-corrected visual acuity was measured at the longest follow-up and immediately before surgery. SRF and foveal status immediately before surgery were analyzed. RESULTS: Two hundred and forty and 95 patients were in BEP and control cohorts, respectively. Thirty patients presented immediately before surgery for analysis. High (64%) and medium (35%) compliance showed significantly greater ( P < 0.01) SRF reduction compared with low (4%) and control (3%). Mac-off RRD showed significantly greater ( P < 0.01) foveal reattachment with high (29%) and medium (8%) compliance compared with low (2%) and control (1%). Mac-on RRD demonstrated no significant differences ( P ≥ 0.51) in final best-corrected visual acuity among high (0 logarithm of the minimum angle of resolution [logMAR] [median], 20/20 Snellen), medium (0.10 logMAR, 20/25 Snellen), low (0.10 logMAR), and control cohorts (0.10 logMAR). Mac-off RRD demonstrated significantly better final best-corrected visual acuity with high compliance (0.30 logMAR, 20/40 Snellen) compared with low (0.40 logMAR, 20/50 Snellen; P = 0.04) and control (0.60 logMAR, 20/80 Snellen; P = 0.02). CONCLUSION: Preoperative BEP can stabilize or improve subretinal fluid in acute primary RRD. Patients with BEP >50% of the time experienced the greatest benefits.

Ocular Whipple Disease: Cases Diagnosed Over Four Decades.

PURPOSE: To describe ocular involvement in subjects with Whipple's disease (WD). METHODS: Retrospective review of documented WD cases seen at Mayo Clinic between 1980 and 2021 with ocular involvement. RESULTS: Of 217 patients with WD, 30 had eye exams and four (two female, median age 58.5 years) had ocular involvement. Findings included anterior/intermediate uveitis (n = 2), intermediate uveitis and phlebitis (n = 1), and chorioretinitis with vitritis (n = 1). The diagnosis was confirmed by vitreous biopsy in three of four cases. In two cases, WD diagnosis was unconfirmed prior to the ocular diagnosis. Systemic manifestations included gastrointestinal symptoms in all patients, synovitis (n = 3), weight loss (n = 2), and pericarditis (n = 1). Mean time from onset of ocular symptoms to ocular diagnosis was 11 months (range 2-28 months). Prior systemic symptoms were present as long as 3 years. CONCLUSIONS: WD is uncommon and ocular involvement is even more rare. However, WD should be considered in the differential for all patients with chronic recalcitrant uveitis, especially in the setting of polyarthralgias and/or gastrointestinal symptoms. Vitreous biopsy is a reliable method to diagnose ocular WD.Abbreviations and Acronyms: Whipple's disease (WD), intestinal lipodystrophy (IL), polymerase-chain reaction (PCR), periodic acid-Schiff (PAS), trimethoprim/sulfamethoxazole (TMP/SMX).

VITREORETINAL INVOLVEMENT BY INDOLENT T-CELL LYMPHOPROLIFERATIVE DISORDER OF THE GASTROINTESTINAL TRACT DIAGNOSED BY FLUORESCENCE IN SITU HYBRIDIZATION.

PURPOSE: The purpose of this study was to describe an exceedingly rare presentation of secondary vitreoretinal involvement by the uncommon entity "indolent T-cell lymphoproliferative disorder of the gastrointestinal tract" and illustrate the utility of fluorescence in situ hybridization for diagnosis. METHODS: This is a case report. RESULTS: A 57-year-old woman with presumed iritis on chronic topical prednisolone acetate presented with increased vitreous opacities in the right eye. She had a history of biopsy-confirmed indolent T-cell lymphoproliferative disorder of the gastrointestinal tract involving the stomach and duodenum, JAK2 -rearrangement positive, controlled on maintenance oral methotrexate. Vitreous biopsy was unremarkable with small CD3-positive and CD4-positive and CD20-negative lymphocytes, along with histiocytes and fibroblasts. Immunostains showed CD4 positivity, and fluorescence in situ hybridization revealed a JAK2 gene rearrangement, consistent with the patient's previously diagnosed indolent T-cell lymphoproliferative disorder of the gastrointestinal tract. Intravitreal methotrexate injections were started in the right eye. MRI of the brain and lumbar puncture with cytology, MYD88 , IL10, and flow cytometry performed at the time of right eye vitreoretinal lymphoma diagnosis revealed no evidence of central nervous system lymphoma, but subsequent bone marrow biopsy demonstrated 5% involvement by indolent T-cell lymphoproliferative disorder of the gastrointestinal tract, JAK2 -rearrangement positive, with a lung nodule on PET computed tomography. She returned 4 months later with fatigue, night sweats, and blurry vision in the left eye with vitreous and anterior chamber cellular infiltration and retinal vasculitis. CONCLUSION: T-cell vitreoretinal lymphoma is rare, and diagnosis can be challenging. Despite inconclusive cytology in this case, interphase fluorescence in situ hybridization detected a JAK2 gene rearrangement, which confirmed the involvement by indolent T-cell lymphoproliferative disorder of the gastrointestinal tract and prompted appropriate treatment and workup for recurrent systemic or central nervous system lymphoma.

Combined Ophthalmic Artery and Central Retinal Vein Occlusion Secondary to Advanced Mantle Cell Lymphoma.

This case report describes a diagnosis of ophthalmic artery and central retinal vein occlusion in a female patient with a history of end-stage mantle cell lymphoma.

Clinical Outcomes Following Implementation of a Formalized "Flashes and Floaters" Emergency Department Triage Protocol.

PURPOSE: To report outcomes of patients presenting to the emergency department (ED) with new-onset visual flashes and/or floaters following implementation of a formalized triage protocol allowing eligible patients to be discharged for prompt outpatient ophthalmic examination. DESIGN: Retrospective consecutive case series. METHODS: Patient characteristics, protocol eligibility, and clinical outcomes were recorded for adult patients triaged within a formal "flashes and floaters" protocol at a single academic ED. RESULTS: A total of 457 patients presented for 471 unique ED encounters with a chief complaint of visual flashes and/or floaters between October 2014 and May 2018. In all, 61% of patient encounters (287/471) met protocol criteria for prompt outpatient ophthalmic examination, of whom 94% (269/287) were examined within 48 hours. Final diagnoses of protocol-eligible patients were posterior vitreous detachment only (73%, 197/269), retinal break(s) (10%, 26/269), migraine (5%, 14/269), and no cause or new cause found (10%, 27/269). No protocol-eligible patients had retinal detachment or diagnoses requiring emergent diagnostic or therapeutic care (0%, 95% CI = 0%-1.1%). Final diagnoses following 175 encounters not meeting criteria for deferred examination included posterior vitreous detachment only (25%, 43/175), retinal break(s) (19%, 33/175), macula-involving retinal detachment (13%, 22/175), macula-sparing retinal detachment (11%, 19/175), retinal arterial occlusion (2%, 3/175), and stroke (0.6%, 1/175). The Cohen kappa for agreement on protocol eligibility between the ED physician and ophthalmologist was 0.85. CONCLUSIONS: A formalized ED "flashes and floaters" triage protocol may help identify patients for whom prompt outpatient ophthalmic examination may be more safely considered.

The Continuing Ophthalmic Challenge of Bartonella henselae.

Purpose: To better understand the history and epidemiology of Bartonella henselae infections of the eye and adnexa, and their relationship to cat scratch disease (CSD). We also assess B. henselae infection as a public health threat. Methods: We reviewed the available literature concerning B. henselae infections of the eye and CSD, and attempted calculation of the incidence and prevalence of both B. henselae eye infections and CSD from the database of the Rochester Epidemiology Project. Results: It took nearly a century of determined effort to reveal that Henri Parinaud's oculoglandular syndrome (POGS) (1889) and Leber's stellate retinitis (1916) were the result of B. henselae infection and are subtypes of CSD. These ocular infections remain of clinical, epidemiologic, and public health concern to ophthalmologists with many unanswered questions. Their incidence and prevalence have yet to be accurately determined. Our attempt to achieve this through the Rochester Epidemiology Project database suggests a major obstacle is inconsistent with nonunanimous diagnostic terminology and coding. Conclusions: Modern serologic testing and molecular diagnostic techniques offer ophthalmologists the opportunity to make B. henselae infection of the eyes an area of "precision medicine." For this to happen, greater awareness and teaching about this disease, updated terminology, and a greater clinical and research effort are required.