Non-Healing Corneal Ulcer and Uveitis Following Monkeypox Disease: Diagnostic and Therapeutic Challenges.

PURPOSE: The ocular manifestations of Monkeypox virus (Mpox) infection remain incompletely characterized. Our goal is to present a case series of non-healing corneal ulcers with associated uveitis caused by Mpox infection as well as management recommendations for Mpox-related ophthalmic disease (MPXROD). METHODS: Retrospective case series. RESULTS: Two male patients with recent hospitalization for systemic Mpox infection presented with non-healing corneal ulcer associated with anterior uveitis and severe IOP elevation. Despite initiation of conservative medical treatment including corticosteroid treatment for uveitis, in both cases, there was clinical progression with enlarging cornea lesions. Both cases received oral tecovirimat with complete healing of the corneal lesion. CONCLUSIONS: Corneal ulcer and anterior uveitis are rare complications of Mpox infection. Although Mpox disease is generally anticipated to be self-limited, tecovirimat may be an effective intervention in poorly healing Mpox keratitis. Corticosteroids should be used with caution in Mpox uveitis, as they might lead to worsening infection.

Drug-induced Sarcoid Uveitis with Biologics.

PURPOSE/OBJECTIVES: to evaluate new onset uveitis or reactivated uveitis by biologic agents and characterize their features. MATERIALS AND METHODS: This is a multicenter, retrospective case series. Patients under biologic therapy were included if they developed uveitis for the first time or experienced intraocular inflammation which was different in location or laterality to previous inflammation. RESULTS: Sixteen patients were identified. The underlying disorders included ankylosing spondylitis, juvenile idiopathic arthritis, rheumatoid arthritis, and Behçet's Disease. The biologic agents associated with a first episode of uveitis (n = 11) or with a new recurrence of uveitis (n = 5) were etanercept, adalimumab, abatacept, infliximab, and golimumab. Sarcoidosis based on bihilar lymphadenopathy, other computer tomography-findings, or biopsy was diagnosed in five patients under therapy with etanercep, adalimumab, and abatacept. Additionally, seven patients developed clinical changes in their uveitis pattern, suggesting sarcoid uveitis. CONCLUSIONS: Biologic treatment-induced uveitis often presents as granulomatous disease.

Cancer-associated retinopathy preceding the diagnosis of a pulmonary carcinoid tumour.

Cancer-associated retinopathy (CAR) belongs to the paraneoplastic retinopathy syndromes and manifests itself by rapidly progressive vision loss, scotoma and photopsia. We herein reported the case of a 77-year-old woman without a cancer history who presents typical CAR symptoms. A complete workup followed by lung biopsy enabled the detection of a pulmonary carcinoid tumour. Treatment of oral cortisone was then initiated with dramatic improvements in the symptoms.

The Collaborative Ocular Tuberculosis Study (COTS) Consensus (CON) Group Meeting Proceedings.

An international, expert led consensus initiative was set up by the Collaborative Ocular Tuberculosis Study (COTS) group to develop systematic, evidence, and experience-based recommendations for the treatment of ocular TB using a modified Delphi technique process. In the first round of Delphi, the group identified clinical scenarios pertinent to ocular TB based on five clinical phenotypes (anterior uveitis, intermediate uveitis, choroiditis, retinal vasculitis, and panuveitis). Using an interactive online questionnaires, guided by background knowledge from published literature, 486 consensus statements for initiating ATT were generated and deliberated amongst 81 global uveitis experts. The median score of five was considered reaching consensus for initiating ATT. The median score of four was tabled for deliberation through Delphi round 2 in a face-to-face meeting. This report describes the methodology adopted and followed through the consensus process, which help elucidate the guidelines for initiating ATT in patients with choroidal TB.

Standardization of Nomenclature for Ocular Tuberculosis - Results of Collaborative Ocular Tuberculosis Study (COTS) Workshop.

Purpose: To standardize a nomenclature system for defining clinical phenotypes, and outcome measures for reporting clinical and research data in patients with ocular tuberculosis (OTB).Methods: Uveitis experts initially administered and further deliberated the survey in an open meeting to determine and propose the preferred nomenclature for terms related to the OTB, terms describing the clinical phenotypes and treatment and reporting outcomes.Results: The group of experts reached a consensus on terming uveitis attributable to tuberculosis (TB) as tubercular uveitis. The working group introduced a SUN-compatible nomenclature that also defines disease "remission" and "cure", both of which are relevant for reporting treatment outcomes.Conclusion: A consensus nomenclature system has been adopted by a large group of international uveitis experts for OTB. The working group recommends the use of standardized nomenclature to prevent ambiguity in communication and to achieve the goal of spreading awareness of this blinding uveitis entity.

Intraocular T-cell Lymphoma: Clinical Presentation, Diagnosis, Treatment, and Outcome.

PURPOSE: To report on the clinical data of seven patients with T-cell intraocular lymphoma (IOL). METHODS: Retrospective case series. RESULTS: Seven immunocompetent patients, 12 eyes, 6 women, with T-cell-IOL were included from five countries. Mean age was 53.5 years (range: 25-82). Four patients had systemic-ocular lymphoma, two had CNS-ocular lymphoma, and one had systemic-CNS- ocular lymphoma. Vitritis was the most frequent clinical sign, followed by anterior uveitis and serous retinal detachment. Cytopathologic examination was performed on all ocular specimens (vitreous in six and iris mass biopsy in one patient). Adjunctive diagnostic procedures included immunohistochemistry, molecular tests, and cytokine profiling of vitreous samples. Treatment modalities included systemic chemotherapy (five patients), intravitreal methotrexate (three patients), globe radiotherapy, and intrathecal chemotherapy. Mean survival from diagnosis was 21.7 months (range: 2-69). Two patients are still alive. CONCLUSIONS: T-cell IOL has variable clinical manifestations and prognosis. Systemic involvement, SRD, and vitreoretinal involvement were frequently observed.

Response of Postoperative and Chronic Uveitic Cystoid Macular Edema to a Dexamethasone-Based Intravitreal Implant (Ozurdex).

PURPOSE: To survey the clinical responses to treatment of chronic postoperative and uveitic cystoid macular edema (CME) with a dexamethasone-based intravitreal implant (Ozurdex(®)). METHODS: This retrospective, interventional case series reports on patients with chronic CME after uncomplicated vitrectomy for epiretinal gliosis or phacoemulsification (group 1: 12 eyes) or secondary to noninfectious endogenous uveitis (group 2: 36 eyes). Central retinal thickness (CRT), best-corrected visual acuity (BCVA, logMAR), and intraocular pressure (IOP) throughout follow-up were gleaned from the medical records. RESULTS: In group 1, CRT decreased, compared with baseline, from 519 ± 43 to 297 ± 23 and 356 ± 49 µm by the 1- and 3-month visit (P = 0.02) and to 429 ± 57 µm before reimplantation. In group 2, CRT decreased from 460 ± 31 to 300 ± 21 and 312 ± 26 µm by the 1- and 3-month follow-up, respectively (P = 0.001), and to 373 ± 32 µm before reimplantation. Complete resolution of CME was achieved in 67% and 94% (groups 1 and 2, respectively) by 1 month and in 42% and 80% by 3 months after injection. In group 1, BCVA improved from 0.46 ± 0.08 to 0.27 ± 0.09 and 0.20 ± 0.06 (P = 0.01) by the 1- and 3-month follow-up, respectively, and to 0.32 ± 0.08 before reimplantation. In group 2, BCVA improved from 0.47 ± 0.06 to 0.34 ± 0.09, 0.26 ± 0.07, and 0.29 ± 0.08 (P < 0.05) at 1 and 3 months of follow-up and before reimplantation, respectively. A significant IOP increase was not observed in either group. Mean time to reimplantation of Ozurdex was 6.4 ± 5.7 and 6.6 ± 3.4 months for postoperative and uveitic CME, respectively. CONCLUSION: Ozurdex seems to achieve a sustained effect over up to 6 months in postsurgical and uveitic CME.

Intraocular penetration of penciclovir after oral administration of famciclovir: a population pharmacokinetic model.

OBJECTIVES: We developed a population model that describes the ocular penetration and pharmacokinetics of penciclovir in human aqueous humour and plasma after oral administration of famciclovir. METHODS: Fifty-three patients undergoing cataract surgery received a single oral dose of 500 mg of famciclovir prior to surgery. Concentrations of penciclovir in both plasma and aqueous humour were measured by HPLC with fluorescence detection. Concentrations in plasma and aqueous humour were fitted using a two-compartment model (NONMEM software). Inter-individual and intra-individual variabilities were quantified and the influence of demographics and physiopathological and environmental variables on penciclovir pharmacokinetics was explored. RESULTS: Drug concentrations were fitted using a two-compartment, open model with first-order transfer rates between plasma and aqueous humour compartments. Among tested covariates, creatinine clearance, co-intake of angiotensin-converting enzyme inhibitors and body weight significantly influenced penciclovir pharmacokinetics. Plasma clearance was 22.8±9.1 L/h and clearance from the aqueous humour was 8.2×10(-5) L/h. AUCs were 25.4±10.2 and 6.6±1.8 µg·h/mL in plasma and aqueous humour, respectively, yielding a penetration ratio of 0.28±0.06. Simulated concentrations in the aqueous humour after administration of 500 mg of famciclovir three times daily were in the range of values required for 50% growth inhibition of non-resistant strains of the herpes zoster virus family. CONCLUSIONS: Plasma and aqueous penciclovir concentrations showed significant variability that could only be partially explained by renal function, body weight and comedication. Concentrations in the aqueous humour were much lower than in plasma, suggesting that factors in the blood-aqueous humour barrier might prevent its ocular penetration or that redistribution occurs in other ocular compartments.

A shared HLA-DRB1 epitope in the DR beta first domain is associated with Vogt-Koyanagi-Harada syndrome in Indian patients.

PURPOSE: Vogt-Koyanagi-Harada (VKH) disease and sympathetic ophthalmia (SO) are two distinct entities that share common clinical and histopathological features; however, it remains unknown whether they have a common genetic susceptibility. Several studies have shown an association of human leukocyte antigen (HLA)-DR4 with VKH disease in patients of different ethnic backgrounds. We present in this paper the HLA-DRB1 genotyping analysis of a large cohort of VKH patients from southern India and compare these patients to patients with SO and to healthy individuals from the same geographic area. METHODS: VKH patients were diagnosed according to the revised criteria of the International Committee on VKH disease. Patients with granulomatous uveitis after ocular trauma or multiple eye surgeries were diagnosed as having SO. Genomic DNA was extracted from all patients and controls. Samples were analyzed for HLA-DRB1 alleles by reverse polymerase chain reaction (PCR) sequence-specific oligonucleotide (SSO) hybridization on microbeads, using the Luminex technology, and by PCR sequence-specific primers (SSP) typing for DRB1*04 allele determination. Strength of associations was estimated by odds ratios (OR) and 95% confidence intervals (CI) and frequencies were compared using the Fisher's exact test. RESULTS: HLA-DRB1 alleles were determined in 94 VKH patients, 39 SO patients, and 112 healthy controls. HLA-DRB1*04 frequency was higher in VKH patients (20.2% versus 10.3% in controls; OR=2.2, p=0.005, pc=0.067). This association was lower than the association of HLA-DRB1*04 frequency in cohorts of patients from different origins. No significant DR4 association with SO was detected. HLA-DRB1*0405 and HLA-DRB1*0410 alleles were significantly increased in VKH patients (8.5% versus 0.9% in controls; OR=10.3, 95% CI=2.34-45.5, p<0.001). These two alleles share the epitope S57-LLEQRRAA (67-74) in the third hypervariable region of the HLA-DR molecule. None of the DRB1 alleles was significantly associated with SO. CONCLUSIONS: Based on the association of HLA-DRB1*0405 and HLA-DRB1*0410 alleles with VKH disease, we propose that the epitope S57-LLEQRRAA (67-74) in the third hypervariable region of the HLA-DRbeta1 molecule is the relevant susceptibility epitope. This genetic component seems specific to VKH disease since no correlation could be identified in SO patients. The weaker association with HLA-DR4 in this VKH patient cohort compared to VKH patients from northern India is probably related to the lower frequency of HLA-DRB1*0405 in our study group. The HLA-DRB1 association with susceptibility to VKH syndrome seems weaker in Indian patients compared to Japanese or Hispanic patients, suggesting a different non-HLA immunogenetic background in Indian VKH patients.

VZV retinal vasculitis without systemic infection: diagnosis and monitoring with quantitative Polymerase Chain Reaction.

To report a case of unilateral varicella zoster virus (VZV) retinal vasculitis aspect in an immunocompetent child without systemic infection. Clinically, no signs of retinal necrosis or frosted branch vasculitis were present. This is an observational case report. Quantitative PCR was performed on the aqueous humor (AH) using primers specific for herpes virus (cytomegalovirus, Epstein-Barr virus, herpes simplex virus 1-2, and VZV). The patient was treated with intravenous acyclovir, intravitreous ganciclovir, and oral valacyclovir. A positive quantitative PCR result was found for VZV DNA (1.72 x 10(6) viral copies/ml) in the AH. After 6 months, PCR of the AH was negative. Herpes viruses are involved in the pathogenesis of isolated retinal vasculitis. This case demonstrates that quantitative PCR is useful to detect viral DNA in AH and to monitor the viral activity and the therapeutic response.

Bacterial keratitis after nonpenetrating glaucoma surgery.

A 68-year-old woman had uneventful deep sclerectomy with a collagen implant in the left eye that was complicated by infectious keratitis 2 weeks later. Corneal scraping revealed the presence of Staphylococcus aureus. The patient responded to topical antibiotic treatment, and the corneal infiltration resolved, leaving a corneal scar. Bacterial keratitis may occur after nonpenetrating glaucoma surgery and should be included in the list of early postoperative complications.

Multiple evanescent white dot syndrome following simultaneous hepatitis-A and yellow fever vaccination.

PURPOSE: To report a case of multiple evanescent white dot syndrome (MEWDS) following simultaneous hepatitis-A virus (HAV) and yellow fever (YF) vaccination. METHODS: Review of the clinical, laboratory, photographic, and angiographic records of a patient suffering from MEWDS. RESULTS: A healthy 50-year-old woman presented with rapidly progressive left-eye visual loss, associated with photopsias and a para-central scotoma, one week after receiving simultaneous HAV and YF vaccination. Both anterior segments and right-eye fundus were unremarkable. Fundus examination of the left-eye disclosed papillitis with multiple, small, white, outer-retinal lesions. Angiographic tests were pathognomonic for MEWDS. Perimetry revealed left-eye blind spot enlargement. Initial inflammatory/infectious work-up was negative. Signs and symptoms resolved spontaneously within 6 weeks, with concomitant normalization of ancillary exams. CONCLUSIONS: The clinical presentation and the benign course were consistent with the diagnosis of MEWDS. No other aetiopathogenic factor than simultaneous HAV and YF immunization was identified, suggesting an autoimmune basis for MEWDS in predisposed patients.

Indocyanine green angiographic features in endogenous Candida chorioretinis.

BACKGROUND: To describe indocyanine green (ICG) angiography (ICGA) findings and clinical features of endogenous mycotic endophthalmitis. PATIENTS AND METHODS: Two patients (a female 62 years, a male 31 years) were addressed to investigate a progressive unilateral visual loss. Slit-lamp examination disclosed a macular chorioretinitis. A clinical work-up revealed a mycotic infection (Candida albicans). Before treatment an ICGA was performed. RESULTS: ICGA early frames disclosed hypofluorescent lesions. Progressively, the lesions were surrounded by a slight hyperfluorescence, although the centre of the lesions was still hypofluorescent. CONCLUSIONS: The presence and persistence of a hypofluorescent lesion after introducing a specific treatment, led us to suspect a necrotic/ischaemic process affecting the choroidal vascular bed. ICGA provided additional information regarding the pathophysiological process and the patient's functional visual recovery.

[Cerebral sinus thrombosis in Behçet disease: case report and review of the literature]. / Thrombose des sinus veineux cerébraux dans la maladie de Behçet: description d'un cas et revue de la litérature.

BACKGROUND: Behçet's disease is a chronic inflammatory disorder, clinically characterised by multisystemic vasculitis. Primary neurologic involvement in childhood is quite exceptional. We report a case of a child with recurrent venous thrombosis of the dural sinus. Behçet's disease was diagnosed. HISTORY AND SIGNS: A 13-year-old boy from Eritrea was referred to our clinic with severe headaches and photophobia one month after onset. Ophthalmological examination disclosed a bilateral papilledema. Recurrent oral ulcers and pseudo-folliculitis were present. Pathergy test was positive. THERAPY AND OUTCOME: Complementary investigations disclosed an inflammatory syndrome. Computertomography was normal. Cranial magnetic resonance venography disclosed multiple cerebral sinus thrombosis (superior sagittal sinus, sigmoid sinus and lateral left sinus). There was no evidence of infectious, inflammatory or hypercoagulation disorder. A Behçet's disease was diagnosed based on the medical history and clinical examination. Whilst his general health deteriorated rapidly before treatment, he improved quickly after the onset of therapy with immunosuppression (Prednisone and Ciclosporine) in combination with anticoagulation (coumarin/Sintrom). CONCLUSION: Behçet's disease is uncommon and difficult to diagnose in children. Neurologic involvement as a first presentation is rarely described, but might be underestimated. We emphasise, therefore, the systematic application of magnetic resonance venography in children with unclear persistent headaches.

Chronic hyposphagma revealing primary ocular amyloidosis.

BACKGROUND: Amyloidosis is an extracellular accumulation of a clear substance called amyloid in different organs. Fragments of identical proteinic chains are the components of this substance. Amyloidosis can be primary, secondary (chronic inflammation, multiple myeloma, tumour), senile or hereditary. HISTORY AND SIGNS: A 59-year-old healthy patient was referred with recurrent subconjunctival haemorrhages in his right eye since one year. The clinical examination disclosed the presence of yellowish subconjunctival deposits associated with haemorrhages. THERAPY AND OUTCOME: Histopathologic examination of these deposits revealed the presence of amyloid. A complete work-up to exclude a systemic disease gave negative results. CONCLUSIONS: Primary conjunctival amyloidosis is a rare clinical entity that is mainly diagnosed histopathologically. In the presence of a recurrent hyposphagma of unknown aetiology the diagnosis of amyloidosis should be excluded.

[Morganella morganii endophthalmitis after vitrectomy: case report and review of the literature]. / Endophtalmie à Morganella morganii après vitrectomie: cas clinique et revue de la litérature.

BACKGROUND: Postoperative bacterial endophthalmitis are caused in 80 % of the cases by the patient's own flora. Most of the time, bacterial agents are Gram-positive ((2/3) of cases) and more rarely Gram-negative ((1/3) of cases). Usually, Pseudomonas sp, Proteus sp or Klebsiella sp are isolated, but very rarely Morganella morganii. HISTORY AND SIGNS: We describe a case of a Morganella morganii endophthalmitis which occurred after a vitrectomy. THERAPY AND OUTCOME: Bacterial examinations disclosed the presence of Morganella morganii in the vitreous. An aggressive treatment (intravitreous [ceftazidim, vancomycin], topical [gentamycin, chloramphenicol] and intravenous [imipenem, ofloxacin] antibiotics) was introduced. In spite of this treatment, the outcome was not favorable. CONCLUSIONS: Post-vitrectomy endophthalmitis is very rare and the isolation of a Gram-negative bacteria, in this case Morganella morganii, is infrequent. The outcome of these infections is often poor despite the introduction of a rapid, specific and aggressive treatment.