Masquerade syndrome in ocular surface squamous neoplasia / Síndrome mascarada em neoplasia escamosa da superfície ocular

ABSTRACT The aim of this study was to alert the ophthalmic community to an atypical manifestation of ocular surface squamous neoplasia, which may delay diagnosis and treatment and result in a guarded visual prognosis and significant sequelae. A 61-year-old immunocompetent man presented with an initial diagnosis of necrotizing scleritis in the right eye for 3 months. He was treated with systemic prednisone but experienced persistent pain and low visual acuity. Conjunctival biopsy of the affected region confirmed the diagnosis of invasive ocular surface squamous neoplasia, which progressed with intraocular and orbital invasion; thus, exenteration was performed. Masquerade syndrome should be suspected in patients with nodulo-ulcerative lesions of the conjunctiva and sclera. This clinical can be more aggressive, with a greater likelihood of intraocular and orbital involvement. The earlier the diagnosis and treatment, the better the patient prognosis.

RESUMO O objetivo é alertar a comunidade oftalmológica sobre uma manifestação atípica de neoplasia escamosa da superfície ocular (OSSN) que pode levar a um atraso no diagnóstico e tratamento, evoluindo com prognóstico reservado e significativas sequelas. Homem, imunocompetente, 61 anos com diagnóstico inicial de esclerite necrosante em olho direito há 3 meses, em tratamento com prednisona sistêmica porém com persistência da dor e baixa acuidade visual. Realizado biópsia conjuntival em região acometida e diagnosticado como neoplasia escamosa da superfície ocular invasiva. Evolui com invasão intraocular e orbital sendo submetido a exenteração. Assim sendo, deve-se suspeitar de síndrome mascarada frente a um paciente com lesões nódulo-ulcerativas da conjuntiva e esclera. Essa forma clínica pode ser mais agressiva, com maior chance de comprometimento intraocular e orbital. Quanto mais precoces o diagnóstico e o tratamento, melhor o prognóstico para o paciente.

Posterior segment involvement in systemic lupus erythematosus – A series from South India

Purpose: To report the spectrum of posterior segment manifestations and visual outcomes in a large series of patients with systemic lupus erythematosus (SLE). Methods: Retrospective study at a tertiary referral eye center in south India between 2016 and 2022. Results: Charts of 109 patients diagnosed to have SLE were retrieved from our medical database. Only nine cases of SLE (8.25%) had posterior segment involvement. The male: female ratio was 1:8. The mean age was 28 years. Unilaterality was the most common presentation in eight cases (88.89%). Lupus nephritis was the most common systemic presentation in five cases (55.56%). Antiphospholipid antibodies (APLA) positivity was seen in two cases (22.22%). Ocular manifestations included microangiopathy (cotton wool spots) in one case, occlusive retinal vasculitis with cotton wool spots in four cases (five eyes), optic disc edema with combined venous and arterial occlusion (one case), central retinal vein occlusion with cotton wool spots and hemorrhages (one case), macular edema (four cases), posterior scleritis with optic disc edema and exudative retinal detachment in the posterior pole (one case), and tubercular choroidal granuloma (one case). Treatment included systemic steroids, hydroxychloroquine sulfate (HCQS), and immunosuppression in all cases, blood thinners in two cases, and laser photocoagulation in four cases. HCQS?related retinal toxicity was not seen in any of the 109 cases. Ocular manifestation was the initial presentation of SLE in one case. Visual outcome was poor in three cases. Conclusion: Presence of posterior segment findings in cases with SLE may suggest a severe systemic disease. Early detection and aggressive treatment result in better visual outcomes. Ophthalmologists could play a vital role in guiding systemic therapy.

Bilateral interstitial keratitis in a patient with presumed brain tuberculoma / Ceratite intersticial bilateral em um paciente com tuberculoma cerebral presumido

ABSTRACT Interstitial keratitis is an inflammation of the corneal stroma without epithelium or endothelium involvement. The underlying causes are mostly infectious or immune mediated. Brazil has one of the highest incidence rates of tuberculosis in the world. Tuberculosis is considered one of the causes of interstitial keratitis. Malnutrition and anemia are risk factors of the disseminated disease. This is a case report of a 10-year-old child who presented with decreased visual acuity and a clinical diagnosis of bilateral interstitial keratitis and sclero-uveitis. The patient had been treated with topical steroids with partial improvement. Examinations revealed severe iron deficiency anemia, negative serologies for human immunodeficiency virus and syphilis, positivity for cytomegalovirus- and herpes simplex-specific IgG, and purified protein derivative of 17 mm. During the follow-up, the patient presented with tonic-clonic seizures, and magnetic resonance imaging findings suggested a central nervous system tuberculoma. Interstitial keratitis improvement was observed after specific tuberculosis treatment. This is the first case report describing the association of interstitial keratitis and central nervous system tuberculoma.

RESUMO A ceratite intersticial é uma inflamação do estroma corneano sem envolvimento epitelial ou endotelial causada principalmente por doenças infecciosas e imunomediadas. O Brasil tem altas taxas de tuberculose que deve ser lembrada como causa de ceratite intersticial. Desnutrição e anemia são fatores de risco da forma disseminada da tuberculose. Este é um relato de uma criança de 10 anos com redução de acuidade visual e diagnóstico clínico de ceratite intersticial bilateral e esclerouveíte. O paciente obteve melhora parcial da ceratite com corticoide tópico. Exames laboratoriais mostraram anemia ferropriva grave, sorologias negativas para HIV e sífilis; IgM negativo e IgG positivo para citomegalovírus e herpes simplex e PPD positivo (17 mm). Ele evoluiu com crises tônico-clônicas e a ressonância nuclear magnética revelou tuberculoma do sistema nervoso central. A melhora da ceratite intersticial foi observada após tratamento para tuberculose. Este é o primeiro caso que descreve a associação de ceratite intersticial e tuberculoma do sistema nervoso central.

Posterior scleritis following COVID-19 vaccination in an elderly patient

We report a case of posterior scleritis masquerading as choroidal melanoma following COVID-19 vaccination. An 86-year-old Caucasian male presented to a retina specialist with a 2-month history of blurred vision and pain right eye (OD). He received his fourth dose of the Pfizer-BioNTech COVID-19 vaccine before developing ocular symptoms. An intraocular mass was found OD and he was referred to our Ocular Oncology Service for potential choroidal melanoma with exudative retinal detachment. On examination, there was a 360-degree episcleral injection and no evidence of choroidal mass OD. Multimodal imaging confirmed no abnormality. Previous ultrasonography demonstrated an echolucent choroidal mass, likely representing choroidal effusion and minimal episcleral Tenon’s edema, suggesting posterior scleritis with spontaneous resolution over 1 week.Posterior scleritis following COVID-19 vaccination can masquerade as choroidal melanoma. In our case, the scleritis resolved spontaneously with no treatment and minimal consequences

Clinical profile and management of granulomatosis with polyangiitis–associated scleritis from a tertiary care hospital in South India

Purpose: To describe the clinical features and management of patients with scleritis associated with granulomatosis with polyangiitis (GPA) at a tertiary eye care center in South India. Methods: The clinical profile and management of patients presenting to a tertiary eye care center in South India with scleritis secondary to GPA from 2003 to 2021 were analyzed retrospectively. Scleritis was classified into anterior diffuse, nodular, and necrotizing scleritis with inflammation according to Watson and Hayreh’s classification. Demographic characteristics, clinical features, anti?neutrophil cytoplasmic antibody (ANCA) positivity, treatment response, ocular complications, and status at the last follow?up were analyzed. Statistical analysis of data was performed using Microsoft Excel 2019. Results: Nineteen eyes of 17 patients (15 cytoplasmic staining ANCA [c?ANCA], two p?ANCA positive) were included. Fifteen eyes had necrotizing scleritis, two had diffuse anterior scleritis, and two had nodular scleritis. Remission was induced using a combination of steroids and cyclophosphamide or rituximab. Maintenance therapy was instituted using tapering steroids and immunosuppressants like cyclophosphamide, mycophenolate mofetil, methotrexate, or rituximab. Three eyes required a scleral patch graft. Fourteen patients had good anatomical and visual outcomes, and three were lost to follow?up. Conclusion: GPA is a rare disease, while it is the most common ANCA?associated vasculitis with scleritis. As scleritis may be the presenting sign of the disease, ophthalmologists must be aware of the various features suggestive of GPA. GPA?associated scleritis can have a good prognosis when diagnosed promptly and managed aggressively in the acute stage, and remission is maintained with adequate systemic immunosuppression.

A challenging case of keratoconjunctivitis medicamentosa with necrotizing scleritis

We report a case of keratoconjunctivitis medicamentosa in a 64-year-old female. She was operated for cataract 4 months back and treated for viral keratitis at present. Ocular examination revealed inferior sterile corneal melt with ischemic necrotizing scleritis and ischemic necrosis of tarsal and lower fornix conjunctiva. Keratoconjunctivitis medicamentosa was diagnosed. Tenonplasty with multilayered amniotic membrane grafting and mucous membrane grating of the tarsal plate was done to which she responded well. Chemical irritation of the ocular surface by topical drops may result in keratoconjunctivitis medicamentosa. A high index of suspicion is required for the accurate diagnosis of such a visually debilitating condition.

Semilunar sign of cornea: A multimodal analysis of the posterior corneal opacity in non-infectious anterior scleritis

Purpose: To analyze the morphological outcomes of the posterior corneal opacity or 搒emilunar sign� in noninfectious anterior scleritis using multimodal imaging. Methods: This was a prospective observational case series. Patients with anterior scleritis from January 2018 to January 2019 were included. Clinical and demographic data were collected. Posterior cornea was visualized using the digital slit lamp photography (Elite, mega digital vision), spectral domain optical coherence tomography (MS39), and specular count analyzer (EM?3000). 揝emilunar sign� was defined by the (1) presence of posterior corneal opacity, (2) concave semilunar pattern, (3) absence of blood vessels, and (4) normal anterior cornea. Incidence, clinical characteristics and significance, correlation with Mantoux sensitivity, and role of multimodal valuation were assessed. Results: Overall 76 eyes of 72 patients were recruited with anterior scleritis. Fifteen eyes of 11 patients (15.3%) presented with semilunar sign. The scleritis was both nonnecrotizing (n = 8) and necrotizing (n = 7). The semilunar configuration appeared as isolated (n = 9) and continuous lesion (n = 6). The extent was directly related to the scleral disease extent (P = 0.002). The mean thickness measured 212.5 � 129.3 ?m. The mean central endothelial cell density (ECD) was 2540.8 � 351.7 cells/mm2, which was significantly higher than the involved peripheral cornea (P = 0.05). The mean surface area of the semilunar sign was 7.7 � 5.2 mm2. There was no significant correlation between the opacity thickness and the best?corrected visual acuity (P = 0.895, r = ?0.39), ECD (P = 0.52, r = ?0.188), and Mantoux (P = 0.696, r =? 0.142). Conclusion: Corneal semilunar sign of scleritis affected the peripheral cornea and caused no functional abnormality in early presentation. Multimodal analysis can aid in clinical assessment and severity.

Dos manifestaciones oculares de la artritis reumatoide: epiescleritis y escleritis / Two ocular manifestations of Rheumatoid Arthritis: episcleritis and scleritis

La artritis reumatoide es un trastorno inflamatorio sistémico que afecta a las articulaciones, que puede tener varias manifestaciones extraarticulares, incluida la enfermedad ocular inflamatoria. El propósito de este estudio es hacer una descripción general sobre el diagnóstico y el tratamiento de dos manifestaciones oculares de la artritis reumatoide: la epiescleritis y la escleritis, incluida una actualización de las perspectivas del tratamiento. Las manifestaciones oculares, que incluyen epiescleritis, escleritis, queratitis ulcerosa periférica y enfermedad del ojo seco, se pueden encontrar hasta en el 39 por ciento de los pacientes con artritis reumatoide. Si bien no existen consensos ni guías, muchas opciones emitidas desde la práctica reumatológica estarán disponibles para el tratamiento de la escleritis refractaria, una vez descartadas las causas infecciosas, lo que permite un rápido control de la inflamación y evita tanto el daño estructural del ojo como las complicaciones del uso prolongado de esteroides(AU)

Rheumatoid arthritis is a systemic inflammatory disorder that affects the joints, which can have several extra-articular manifestations, including inflammatory eye disease. To provide an overview of the diagnosis and treatment of two ocular manifestations of rheumatoid arthritis: episcleritis and scleritis, including an update on treatment prospects. Ocular manifestations, including episcleritis, scleritis, peripheral ulcerative keratitis, and dry eye disease, can be found in up to 39 percent of rheumatoid arthritis patients. Although there is no consensus or guidelines, many options issued from rheumatology practice will be available for the treatment of refractory scleritis, once the infectious causes have been ruled out, allowing rapid control of inflammation, and avoiding both structural damage to the eye and complications from long-term steroid use(AU)

Pay attention to the diagnosis and treatment of fundus complications related to tumor immunotherapy / 中华眼底病杂志

Cancer immunotherapy refers to the therapeutic effect of controlling or eliminating tumor cells by interfering with the immune system to restore the anti-tumor immune response. Immune checkpoint inhibitor therapy that blocks programmed death -1/programmed cell death ligand-1/cytotoxic T lymphocyte-associated antigen 4 is one of the most commonly used tumor immunotherapies, with good efficacy and wide application. These drugs cause immune-related ocular complications such as uveitis, autoimmune retinopathy, and scleritis, which represent a new etiology of ocular inflammation. The ophthalmologist's grasp of the clinical characteristics of these diseases is helpful for timely diagnosis. At the same time, the ophthalmologist will work closely with the oncologist to make a comprehensive judgment based on the patient's primary tumor, survival prognosis, severity of adverse reactions related to ocular immunotherapy, and visual prognosis, and develop suitable therapeutic strategie, thereby saving the patients' vision and improving the quality of life.

Nodular anterior scleritis associated with Berger's disease / Esclerite anterior nodular associada à doença de Berger

ABSTRACT A 45-year-old female patient presented with a complaint of right eye redness and pain for 7 days. She was under investigation for urinary abnormalities and reported a previous history of recurrent oral ulcers and ocular hyperemia in both eyes. Best-corrected visual acuity was 20/30 and 20/20 in the right and left eyes, respectively. Slit-lamp biomicroscopy of the ocular surface of the right eye revealed nasal scleral hyperemia that persisted after instillation of topical phenylephrine 10%, reinforcing the diagnosis of anterior scleritis. Renal biopsy showed immunoglobulin A immune complexes and confirmed the suspected diagnosis of Berger's disease. Maintenance immunosuppressive therapy with azathioprine following a 6-month induction of remission with cyclophosphamide was necessary after pulse therapy with methylprednisolone. Scleritis is usually related to systemic autoimmune diseases, such as rheumatoid arthritis, and polyangiitis. Herein, we describe a rare case of unilateral anterior scleritis associated with Berger's disease.

RESUMO Paciente de 45 anos, sexo feminino queixava-se de hiperemia e dor no olho direito há sete dias. Encontrava-se sob investigação de alterações urinárias e relatou história pregressa de úlceras orais e hiperemia ocular bilateral recorrentes. A acuidade visual corrigida era de 20/30 no olho direito e 20/20 no esquerdo. A biomicroscopia da superfície ocular do olho direito revelou intensa hiperemia escleral em região nasal que persistiu após a instilação de fenilefrina tópica a 10%, reforçando o diagnóstico clínico de esclerite anterior unilateral. A biópsia renal revelou a presença de imunocomplexos de IgA e confirmou a hipótese de doença de Berger. Uma terapia imunossupressora de manutenção com azatioprina após 6 meses de indução de remissão com ciclofosfamida foi necessária após pulsoterapia com metilprednisolona. A esclerite geralmente está relacionada a doenças autoimunes sistêmicas, como artrite reumatoide e poliangeite. Descrevemos aqui um caso raro de esclerite anterior unilateral associada à doença de Berger.

Oral mucosa for reconstructive surgery in a case of severe inflammatory necrotizing sclero-uveitis

The purpose of this case is to show the efficacy of buccal mucosa as an alternative to treat a case of severe necrotizing sclero-uveitis (NSU) associated with ocular perforation. We show a severe inflammatory NSU case that did not improve with topical treatment and scleral patch. We performed a buccal mucosa graft taken from the lower lip with excellent functional and anatomical result, with no signs of relapse of the NSU after 2 years of follow-up. Buccal mucosa can be a safe, useful, and effective alternative for the reconstruction of the scleral wall.

Can cryotherapy be used as an adjunct in select cases of recalcitrant endophthalmitis? - A case report

We report two cases of recalcitrant endophthalmitis, a delayed postoperative fungal endophthalmitis following cataract surgery and an atypical mycobacterial endogenous endophthalmitis associated with infective scleritis. Due to recalcitrant nature of the endophthalmitis, one or more sittings of double freeze and thaw cryotherapy was applied over and adjacent to the infective foci in each case. This cryotherapy was used as an adjunct alongside conventional endophthalmitis management following which resolution of infection was observed in both cases.

Tubercular posterior scleritis: A case report and review of literature

A 47-year-old female presented with optic disc oedema, peripapillary subretinal fluid and scattered areas of choroiditis. Her ultrasound B-scan showed sclerochoroidal thickening with widening of sub-Tenon space. Subsequent investigations revealed a positive Mantoux test and high-resolution computed tomography of the chest was suggestive of pulmonary involvement. She responded well to antitubercular treatment and systemic corticosteroid. A review of the literature was conducted to identify additional reports on similar cases and discussed. A high index of suspicion and appropriate laboratory work-up can aid in the diagnosis of tuberculous posterior scleritis.

A case of idiopathic necrotizing scleritis with secondary glaucoma treated successfully with golimumab and Ahmed valve implantation

A 52-year-old woman presented with idiopathic active scleritis not responding to oral cyclophosphamide, azathioprine, and oral steroid. Her intraocular pressure in the left eye was 45 mm of Hg in spite of using combination of brinzolamide 1% and brimonidine 0.2% (thrice a day), timolol maleate 0.5% (twice a day) eye drops in both eyes and oral acetazolamide. She was administered subcutaneous golimumab 50 mg injection every 4 weeks along with oral methotrexate 15 mg/week. The scleral inflammation responded and she underwent Ahmed glaucoma valve implantation after two months of initiation of golimumab therapy. After one week of surgery her IOP in left eye was the reduced to 8 mm of Hg. The index case showed that Golimumab can be a useful drug in the management of necrotizing scleritis refractory to the conventional therapy.

Alteraciones anatomopatológicas en la escleritis anterior nodular necrotizante / Pathological alterations in necrotizing nodular anterior scleritis

RESUMEN La presencia de la escleritis necrotizante es rara; necesita de un diagnóstico y de un tratamiento oportuno, ya que su curso es rápido, severo y destructivo de la integridad del globo ocular. Se presenta una paciente de 43 años de edad, quien requirió atención oftalmológica al presentar el ojo derecho rojo con intenso dolor. Se le encontraron 3 nódulos en la región antero-inferior escleral. Se le realizó punción-aspiración con aguja fina en los nódulos esclerales y se obtuvo un fragmento de uno de ellos para el corte histológico. En ambas muestras se observó infiltrado inflamatorio de linfocitos y neutrófilos con algunas células plasmáticas. Se le impuso tratamiento con prednisona oral (0,5 mg/kg/día), prednisolona colirio y azatioprina a dosis de 1,5 mg/kg/día. La evolución al mes fue satisfactoria con mejoría gradual de las lesiones. El tratamiento se mantuvo por 3 meses con chequeos hematológicos reiterados. Se disminuyeron posteriormente las dosis medicamentosas hasta su eliminación a los 6 meses. La curación de las lesiones fue total. No se encontró enfermedad sistémica en el chequeo clínico efectuado(AU)

ABSTRACT The presence of necrotizing scleritis is rare; it needs a diagnosis and an opportune treatment, since its course is fast, severe and destructive of the integrity of the eyeball. We present a 43-year-old patient who required ophthalmological attention when presenting the red right eye with intense pain. Three nodules were found in the antero-inferior scleral region. Fine needle aspiration was performed on the scleral nodules and a fragment of one of them was obtained for the histological section. In both samples, inflammatory infiltrate of lymphocytes and neutrophils with some plasma cells was observed. He was prescribed treatment with oral prednisone (0.5 mg /kg/day), prednisolone eye drops and azathioprine at a dose of 1.5 mg/kg/day. The evolution to the month was satisfactory with gradual improvement of the lesions. The treatment was maintained for 3 months with repeated blood tests. Drug doses were subsequently reduced until their elimination at 6 months. The healing of the injuries was total. No systemic disease was found during the clinical check-up(AU)

Esclerite posterior / Posterior scleritis

Resumo Esclerite Posterior (EP) é uma inflamação do segmento escleral posterior, de etiologia inflamatória ou infecciosa, pouco diagnosticada, com evolução rápida, progressiva e irreversível com severo comprometimento visual, principalmente se o diagnóstico e o tratamento da (EP) não forem realizados a tempo hábil. A dor ocular, dor à movimentação ocular, cefaléia e embaçamento visual são os principais sinais e sintomas. Pode ser de causa Idiopática ou associada a doença sistêmica em até 45% dos caso. A Artrite Reumatoide é descrita como a associação mais comum. Causas infecciosas podem estar presente tais como: Herpes Simples, Herpes Zoster Oftálmico, Sífilis e Tuberculose. A bilateralidade pode ocorrer em até 35% dos casos sendo mais prevalente em mulheres a partir da quinta década de vida.

Abstract Posterior Scleritis (PS) is an inflammation of the posterior scleral segment, of low inflammatory or infectious etiology, with a rapid, progressive and irreversible evolution with severe visual impairment, especially if the diagnosis and treatment of (PS) are not performed in time skillful. Ocular pain, eye movement pain, headache and visual haze are the main signs and symptoms. It can be of idiopathic cause or associated with systemic disease in up to 45% of the cases. Rheumatoid arthritis is described as the most common association. Infectious causes may be present such as: Simple Herpes, Ophthalmic Herpes Zoster, Syphilis and Tuberculosis. Bilaterality can occur in up to 35% of cases being more prevalent in women from the fifth decade of life.

Clinical Features and Treatment Outcomes in Patients with Infectious Scleritis

PURPOSE: To investigate risk factors, clinical features, pathogenic organisms, and outcomes in patients with infectious scleritis. METHODS: This study was a retrospective review of 20 patients with infectious scleritis who were admitted from January 2011 to December 2018 in a single tertiary hospital, with at least 3 months of follow-up. We analyzed age, risk factors, clinical manifestations, pathogenic organisms, treatment, and outcomes of infectious scleritis. RESULTS: The mean patient age was 69.2 ± 8.4 years and the mean duration of hospitalization was 11.3 ± 5.8 days. Furthermore, the mean duration of symptoms before presentation was 16.8 ± 13.9 days; patients were followed for a mean duration of 23.3 ± 25.4 months. All patients had prior pterygium surgery. Eighteen patients (90%) were culture-positive and Pseudomonas aeruginosa (P. aeruginosa) was identified in 12 eyes. In the acute stages, adjuvant surgical intervention was performed for 18 patients (90%) for these patients, the mean duration of hospitalization before surgery was 4.1 ± 4.4 days. CONCLUSIONS: The most common risk factor and pathogenic organism for infectious scleritis were prior pterygium surgery and P. aeruginosa, respectively. Identification of specific causative organisms and corresponding antibiotic treatment with adjuvant surgical intervention may improve visual prognosis in patients with infectious scleritis.