RESUMEN
Gorham-Stout disease (GSD) is a rare angiomatous disorder characterized by osteolysis. We report the case of a 66-year-old woman who developed sudden left eye proptosis and facial pain. Neuroimaging demonstrated a destructive mass involving the roof and lateral wall of the left orbit with adjacent abnormal orbital soft tissue. An initial biopsy of the soft tissue was nondiagnostic; however, transcranial biopsy of the orbital roof revealed an intraosseous cavernous angioma, with infiltration of orbital fat by angiomatous tissue. Despite resection of the orbital roof and lateral wall, the orbital lesion continued to expand, leading to signs of a compressive optic neuropathy. The patient then reported severe back pain, prompting imaging that demonstrated disseminated bony involvement of the axial skeleton, eventually leading to the diagnosis of GSD. The patient was treated with a bisphosphonate and a vascular endothelial growth factor inhibitor with stabilization of disease.
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Exoftalmia/diagnóstico , Osteólisis Esencial/diagnóstico , Enfermedad Aguda , Anciano , Inhibidores de la Angiogénesis/uso terapéutico , Bevacizumab/uso terapéutico , Difosfonatos/uso terapéutico , Combinación de Medicamentos , Exoftalmia/tratamiento farmacológico , Dolor Facial/diagnóstico , Femenino , Humanos , Imagen por Resonancia Magnética , Osteólisis Esencial/tratamiento farmacológico , Factor A de Crecimiento Endotelial Vascular/antagonistas & inhibidores , Agudeza VisualRESUMEN
PURPOSE: To evaluate baseline characteristics, microbiological spectrum, management, and outcomes of patients with culture-proven fungal keratitis. METHODS: Retrospective review of all patients with culture-proven fungal keratitis seen over 6 years at a tertiary referral center. RESULTS: The present study included 62 eyes from 62 patients. Infection with filamentous organisms was more common than with yeast (66.1% vs 27.4%). The most common filamentous organisms were Fusarium (17.7%) and Aspergillus (16.1%), while the most common yeast was Candida (24.2%). The main predisposing factor for filamentous keratitis was contact lens use. Yeast keratitis is most associated with an immunocompromised host and ocular surface disease. Corneal perforation (20.0%) and surgical interventions (46.8%) were common, with 27.4% of eyes requiring at least one penetrating keratoplasty. Filamentous keratitis is more likely than yeast keratitis to require urgent penetrating keratoplasty or enucleation and to receive more than one topical and systemic antifungal agent. Visual outcomes were poor with nearly half of the eyes remaining at 20/200 or worse upon resolution of infection. Worse visual outcomes were associated with poor vision at presentation and a history of ocular surface disease. Antifungal susceptibility testing was not routinely performed, but it demonstrated a relatively high minimum inhibitory concentration for at least one antifungal drug in 90% of cases when performed (16.1%) and guided the direction of treatment for 80% of the cases. CONCLUSION: Fungal keratitis is visually devastating. Infections with filamentous fungi predominated over yeast and were generally treated more aggressively both medically and surgically. Filamentous and yeast keratitis had similar durations of infections and visual outcomes. Antifungal susceptibility testing influenced treatment in 80% of cases in which it was performed.
RESUMEN
BACKGROUND/PURPOSE: To describe a case of a rare, late scleral buckle-related complication, which was masquerading as ocular inflammation. METHODS: Case report. RESULTS: A 63-year-old woman with ocular history significant for cataract extraction with intraocular lens placement and retinal detachment repaired through pars plana vitrectomy and scleral buckle presents with anterior chamber inflammation and debris in the left eye. Uveitis workup was negative for any associated systemic disease. Anterior chamber washout and biopsy were nondiagnostic. At postoperative Month 1, she had recurrent anterior chamber debris and new debris noted in the vitreous cavity. A pars plana vitrectomy was performed, and during intraoperative scleral depression, copious strands of whitish material were expressed into the vitreous cavity. The scleral buckle was removed with additional whitish material noted within the encapsulated area. Papanicolaou stain of the material demonstrated a mixture of epithelial cells, histiocytes, lymphocytes, plasma cells, and neutrophils consistent with an epithelial cyst. CONCLUSION: Epithelial inclusion cysts are a rare complication of scleral buckle placement. These cysts are lined by a nonkeratinized, stratified squamous epithelium with or without goblet cells and most commonly occur after ocular trauma, inflammation, or surgical procedures. To the best of our knowledge, this is the first reported case of erosion of an epithelial inclusion cyst into the vitreous cavity.
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Quistes , Curvatura de la Esclerótica , Quistes/diagnóstico , Quistes/etiología , Diagnóstico Diferencial , Femenino , Humanos , Inflamación/diagnóstico , Persona de Mediana Edad , Curvatura de la Esclerótica/efectos adversosRESUMEN
PURPOSE: To determine the prevalence and reasons for delays in diagnosis in patients with Acanthamoeba keratitis (AK) presenting to Wilmer Eye Institute, Baltimore, Maryland. METHODS: This retrospective study analysed all patients with culture-positive AK seen between 2012 and 2019 at a tertiary referral centre. Patient demographic information, clinical history, risk factors, symptom duration, referral patterns, slit lamp examination findings, visual acuity and need for surgery were collected. RESULTS: The study included 45 eyes of 43 patients. On average, patients were symptomatic for 52.6 days before culture collection. Thirty-one percent of patients were diagnosed within 28 days of symptom onset while 69% were diagnosed after 28 days. Before presentation to a tertiary care centre, 69% of patients were evaluated by an ophthalmologist outside of this institution and 27% were evaluated by a provider other than an ophthalmologist. AK was most commonly misdiagnosed as herpetic keratitis, occurring in 38% of patients. The strongest risk factor for AK was contact lens use. Only 11% of patients presented with the classic ring infiltrate and 82% had pain. Patients with an early versus late diagnosis had a mean Snellen visual acuity (VA) of 20/224 versus 20/296 at presentation (p = 0.33) and a mean Snellen VA of 20/91 versus 20/240 at final visit (p = 0.07). 11% of patients required a therapeutic penetrating keratoplasty. CONCLUSION: Delayed diagnosis of AK in our cohort occurred due to a misdiagnosis as herpetic keratitis, non-specific clinical signs including the lack of pain in a number of patients, and a delay in referral to a tertiary care centre. Any contact lens wearer with an atypical keratitis should be referred promptly for Acanthamoeba cultures.
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Queratitis por Acanthamoeba/diagnóstico , Acanthamoeba/aislamiento & purificación , Córnea/parasitología , Diagnóstico Tardío , Infecciones Parasitarias del Ojo/diagnóstico , Centros de Atención Terciaria/estadística & datos numéricos , Agudeza Visual , Queratitis por Acanthamoeba/epidemiología , Queratitis por Acanthamoeba/parasitología , Adulto , Córnea/diagnóstico por imagen , Infecciones Parasitarias del Ojo/epidemiología , Infecciones Parasitarias del Ojo/parasitología , Femenino , Estudios de Seguimiento , Humanos , Masculino , Microscopía Confocal , Prevalencia , Estudios Retrospectivos , Factores de Riesgo , Estados Unidos/epidemiologíaRESUMEN
Congenital corneoscleral epithelial cysts can be associated with scleral thinning secondary to pressure from the expanding cyst. We report a congenital scleral epithelial cyst associated with a likely primary partial-thickness scleral defect. The defect appeared as a full-thickness communication between the cyst and posterior chamber on ultrasound biomicroscopy, most likely because the scleral remnant was too thin to be appreciated on imaging. The cyst was treated surgically by aspiration, excision of the anterior wall, and fibrin glue closure of the cyst cavity, with no recurrence after 14 months of follow-up.
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Quistes , Recurrencia Local de Neoplasia , Humanos , Microscopía Acústica , EscleróticaRESUMEN
A previously healthy 16-year-old adolescent boy presented with pallor, blurry vision, fatigue, and dyspnea on exertion. Physical examination demonstrated hypertension and bilateral optic nerve swelling. Laboratory testing revealed pancytopenia. Pediatric hematology, ophthalmology and neurology were consulted and a life-threatening diagnosis was made.
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Mutación Missense , Pancitopenia/diagnóstico , Papiledema/etiología , Receptores de Trombopoyetina/genética , Adolescente , Diagnóstico Diferencial , Fatiga/etiología , Trasplante de Células Madre Hematopoyéticas , Humanos , Masculino , Disco Óptico/patología , Pancitopenia/complicaciones , Pancitopenia/genética , Pancitopenia/terapia , Retina/patología , Trastornos de la Visión/etiologíaRESUMEN
PURPOSE: To describe the incidence of ocular hypertension (OHT) and secondary glaucoma (SG) in JIA-associated uveitis, identify risk factors for development of these complications, and describe their effect on visual outcomes. METHODS: A retrospective cohort of 108 patients (196 eyes) with JIA-associated uveitis seen over 30 years at an academic practice. RESULTS: Of examined eyes, 40% had OHT or SG at presentation. These eyes had a nearly three-fold higher incidence of legal blindness during follow-up, compared with eyes without OHT or SG. An additional 41 eyes developed OHT or SG during follow-up. Presenting with anterior uveitis, active inflammation, and using systemic corticosteroids were risk factors for developing OHT, while use of immunosuppressive medication at presentation reduced this risk. Risk factors for developing SG included anterior uveitis and use of systemic corticosteroids. CONCLUSIONS: OHT and SG were common in patients with JIA-associated uveitis. Use of immunosuppressive drugs may decrease the risk of developing OHT.
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Artritis Juvenil/complicaciones , Glaucoma/etiología , Hipertensión Ocular/etiología , Uveítis/complicaciones , Adolescente , Artritis Juvenil/fisiopatología , Niño , Preescolar , Femenino , Estudios de Seguimiento , Glaucoma/epidemiología , Glaucoma/fisiopatología , Glucocorticoides/uso terapéutico , Humanos , Inmunosupresores/uso terapéutico , Incidencia , Lactante , Presión Intraocular/fisiología , Masculino , Hipertensión Ocular/epidemiología , Hipertensión Ocular/fisiopatología , Estudios Retrospectivos , Factores de Riesgo , Tonometría Ocular , Uveítis/fisiopatología , Agudeza Visual/fisiologíaRESUMEN
OBJECTIVE: To describe risk factors for hypotony in patients with juvenile idiopathic arthritis (JIA)-associated uveitis. DESIGN: Retrospective cohort study. METHODS: All patients with JIA-associated uveitis (N = 108; affected eyes = 196) evaluated and followed at the Wilmer Eye Institute from July 1984 through June 2014 were included in this study. Prevalence and incidence of hypotony (intraocular pressure [IOP] <5 mm Hg) and low IOP (5 mm Hg ≤ IOP < 8 mm Hg) and risk factors for developing hypotony were analyzed. RESULTS: At presentation, 9.3% of patients (7.1% of affected eyes) had hypotony. During a median follow-up of 5.3 years, the rate of developing hypotony and low IOP were 0.04 per eye-year (/EY; 95% confidence interval [CI]: 0.02/EY, 0.05/EY) and 0.06/EY (95% CI: 0.04/EY, 0.08/EY), respectively. Risk factors for development of hypotony during follow-up appeared to be associated with more severe uveitic disease, such as the presence of panuveitis (adjusted hazard ratio [aHR], 43.1; P = .004), anterior chamber cells or flare ≥ 3+ (aHR, 25.6, P < .001), posterior synechiae (aHR, 5.9, P = .02), and the use of oral corticosteroid (aHR 28.9; P = .003) at the presenting examination. Receiving immunosuppressive drug therapy at the time of presentation was associated with a lower risk of development of hypotony (aHR, 0.02; P = .002). CONCLUSIONS: Hypotony affects a small but significant proportion of patients with JIA-associated uveitis and is associated with signs of active and severe uveitis. Immunosuppression was associated with significantly lower risk of hypotony, suggesting that aggressive control of the inflammation may reduce risk of hypotony in JIA-associated uveitis.