Pacientes con compromiso orbitario y renal: Diagnósticos diferenciales poco frecuentes / Patients with orbitary and kidney commitment: Rare differential diagnoses

Se comunica una serie de tres casos clínicos que consultaron al servicio de Reumatología por compromiso orbitario y renal. Uno de ellos presentó pseudotumor orbitario con proteinuria en rango nefrótico; se realizó biopsia y se encontró infiltrado linfoplasmocitario denso y fibrosis estoriforme con inmunohistoquímica: 15 células IgG4+ por campo de alto poder y relación IgG/IgG4 ≤40%, concluyendo diagnóstico de enfermedad relacionada por IgG4. El segundo y tercer caso presentaron compromiso ocular con "ojos de mapache" y lesiones amarillentas en párpados, ambos con proteinuria >500 mg/24 h, con biopsia de piel rojo Congo positiva y birrefringencia verde manzana con luz polarizada. Se discuten distintos diagnósticos diferenciales poco frecuentes a tener en cuenta en estos pacientes.

A series of three cases that consulted the rheumatology service due to orbital and renal involvement is reported. One of them presented orbital pseudotumor with proteinuria in the nephrotic range, a biopsy was performed, finding dense lymphoplasmacytic infiltrate and storiform fibrosis with immunohistochemistry: 15 IgG4 positive cells per HPF and IgG/IgG4 ratio ≤40%, concluding diagnosis of IgG4 related disease. The second and third cases presented ocular involvement with raccoon eyes and yellowish lesions on the eyelids, both with proteinuria greater than 500 mg/24 h, with apple-green birefringence of amyloid on congo red staining. Different rare differential diagnoses to take into account in these patients are discussed.

Tumor primario quístico de la órbita: A propósito de un caso de hidatidosis infantil / Primary orbital cystic tumor: A case of hydatidosis in a child

RESUMEN Los tumores quísticos primarios de la órbita, causados por infestaciones parasitarias como el Equinococcus granulosus son raros. Al ser el Perú zona endémica para este parásito, presentamos el caso de una niña de 10 años de edad, proveniente de una zona rural de Huancavelica, con proptosis axial irreductible del globo ocular derecho, de curso progresivo, con pérdida de la agudeza visual e imágenes tomográficas de la órbita en relación a masa tumoral de apariencia quística. Los exámenes serológicos fueron negativos, la paciente fue sometida a resección quirúrgica con resultado histopatológico de membranas anhistas correspondientes a quiste hidatídico. Al año de seguimiento no se evidencia recurrencia local ni sistémica. En conclusión, ante una tumoración orbitaria quística en pacientes pediátricos, se debe de considerar como diagnóstico diferencial la hidatidosis orbitaria, sobre todo si proceden de zonas endémicas.

ABSTRACT Primary orbital cystic tumors caused by Echinococcus granulosus are rare. As this parasite is endemic in Peru, we report the case of a 10-year-old girl from a rural area of Huancavelica, who presented with progressive and irreducible axial proptosis of the right eye and loss of vision. Orbital tomographic images demonstrated a tumor mass with a cystic appearance. Serologic tests were negative. The patient underwent surgical resection, and histopathologic analysis revealed anhistic membranes, corresponding to hydatid cysts. During one year of follow-up, no evidence of local or systemic recurrence was observed. In conclusion, orbital hydatidosis should be considered in the differential diagnosis of a cystic orbital tumor in pediatric patients, especially if the patient is from an endemic area.

Fibroma de Tarso / Tarsal Fibroma

RESUMO O objetivo dos autores é relatar um caso de fibroma de tarso, uma tumoração na reborda orbitária súpero-temporal direita com crescimento lento ao longo de anos. A análise da peça obtida da região tarsal mostrou uma lesão bem delimitada, constituída por deposição de fibras colágenas densas, com proliferação de células fusiformes, sem atipias, com revestimento de células conjuntivais, caracterizando fibroma da placa tarsal.

ABSTRACT The objective of the authors is to report the case of a tarsal fibroma, a tumoration on the superotemporal side of the right orbit with slow growth over the years. Analysis of the anatomical specimen obtained from the tarsal region showed a well-defined lesion, consisting of deposition of dense collagen fibers, with fusiform cells without atypia, with coating conjunctival cells, featuring fibroma of the tarsal plate.

Orbital Dirofilariasis

No abstract available.

A prevalencia de complicacoes orbitarias entre criancas e adultos com rinossinusite aguda / The prevalence of orbital complications among children and adults with acute rhinosinusitis

OBJETIVO: Investigar as complicações orbitais em crianças e adultos com sinusite. MÉTODO: Os pacientes que frequentam clínica de Otorrinolaringologia com sinusite de janeiro de 2010 até janeiro de 2012 foram incluídos. Os pacientes foram classificados em dois grupos, de acordo com sua idade. O primeiro incluiu crianças idade inferior a 16 anos e, o segundo, pacientes com mais de 16 anos. Quadro clínico, seio envolvido, gestão e resultados foram comparados. RESULTADOS: O número total de pacientes foi 616. Complicações orbitais foram observadas em 36 pacientes (5,8%). Destes, 26 pacientes (72,2%) eram crianças (21 tinham presseptal e cinco tiveram celulite orbitária) e dez pacientes (27,8%) eram adultos (cinco com pré-septal, três com celulite orbitária e dois com abscesso). A complicação mais comum foi celulite orbital presseptal (72,2%), seguida de celulite e abcesso orbital (22,2% e 5,6%, respectivamente). O seio mais comum envolvido foi etmoidal em crianças e sinusopatia mista em adultos. A maioria dos pacientes respondeu ao tratamento médico. CONCLUSÃO: As complicações orbitárias da sinusite são mais comuns em crianças do que em adultos e têm prognóstico favorável. .

OBJECTIVE: To investigate orbital complications in children and adult with sinusitis. METHOD: Patients attending ENT clinic with sinusitis from January 2010 until January 2012 were included. Patients were classified into two groups according to their age. First involved children aged less than 16 and second included adults older than 16 years. Clinical picture, sinus involved, management and outcome were compared. RESULTS: The total number of patients were 616. Orbital complications were seen in 36 patients (5.8%). Twenty six patients (72.2%) were children (21 had preseptal and 5 had orbital cellulitis) and ten patients (27.8%) were adults (5 with preseptal, three with orbital cellulitis and 2 with abscess). The most common orbital complication was preseptal cellulitis (72.2%) followed by orbital cellulitis and abscess (22.2% and 5.6% respectively). The commonest sinus involved was ethmoidal in children and mixed sinus pathology in adults. The majority of patients responded to medical treatment. CONCLUSION: Orbital complications of sinusitis are commoner in children than adults and have favorable prognosis. Keywords: Sinusitis, cellulitis, preseptal, abscess. .

Magnetic resonance imaging for the ophthalmologist: A primer.

Magnetic resonance imaging (MRI) and computerized tomography (CT) have added a new dimension in the diagnosis and management of ocular and orbital diseases. Although CT is more widely used, MRI is the modality of choice in select conditions and can be complimentary to CT in certain situations. The diagnostic yield is best when the ophthalmologist and radiologist work together. Ophthalmologists should be able to interpret these complex imaging modalities as better clinical correlation is then possible. In this article, we attempt to describe the basic principles of MRI and its interpretation, avoiding confusing technical terms.

Primary localized amyloidosis presenting as diffuse amorphous calcified mass in both orbits: case report / Amiloidose primária localizada se apresentando como massa tumoral amorfa e calcificada em ambas as órbitas: relato de caso

Primary localized amyloidosis is rare in the orbit. We report the case of a 63-year-old woman that presented with bilateral proptosis and ophthalmoplegia. A computed tomography scan revealed an infiltrative amorphous and markedly calcified mass in both orbits while a magnetic resonance scan showed a heterogeneous hypointense signal on T2-weighted images. A biopsy was performed through an anterior orbitotomy. Microscopy revealed extracellular amorphous and eosinophilic hyaline material which stained pink with Congo red and displayed green birefringence on polarized microscopy, leading to a diagnosis of amyloidosis. The results of the systemic workup were completely normal. A two-year follow-up period without any treatment disclosed no worsening of the condition. While calcification of nonvascular orbital lesions has often been regarded as suggestive of malignant disease, our case is a reminder that it can also be a characteristic presenting sign of orbital amyloidosis.

Amiloidose primária e localizada na órbita é rara. Relatamos o caso de paciente do sexo feminino, 63 anos que se apresentou com proptose bilateral e oftalmoplegia. A tomografia computadorizada mostrou uma massa infiltrativa amorfa e calcificada em ambas as órbitas enquanto que a imagem por ressonância magnética mostrou imagem heterogênea hipointensa ponderada em T2. Uma biópsia foi realizada por orbitotomia anterior. A microscopia revelou um material extracelular amorfo, hialino e eosinofílico que corou com vermelho Congo e mostrou cor verde birrefringente à microscopia com luz polarizada, levando ao diagnóstico de amiloidose. O resultado da investigação sistêmica foi inteiramente negativo. Um período de seguimento de dois anos sem qualquer tratamento não evidenciou piora da condição. Enquanto que a presença de calcificação de lesão não vascular da órbita frequentemente é considerada sugestiva de doençamaligna, o nosso caso serve para lembrar que pode também ser um sinal característico de amiloidose orbitária.

Update in pathological diagnosis of orbital infections and inflammations

Orbital infections and inflammations include a broad spectrum of orbital diseases that can be idiopathic, infectious, from primary or secondary inflammatory processes. Being able to properly diagnose and manage these orbital diseases in a timely manner can avoid permanent vision loss and possibly save a patient's life. When clinicians are faced with such patients, quite often the exact diagnosis cannot be made just based on clinical examination, various laboratory tests and imaging are needed. Moreover, orbital biopsies with histopathological analyses are often required, especially for the atypical cases. Thus, it is important for the clinicians to be familiar with the pathological features and characteristics of these orbital diseases. This review provides a comprehensive update on the clinical and pathological diagnosis of these orbital infections and inflammations

Orbital Rosai-Dorfman disease in a five-year-old boy

Rosai-Dorfman disease [RDD] is characterized by histiocytic proliferation and massive cervical lymphadenopathy, although some patients have extra-nodal involvement. We report a case of extranodal RDD in a five-year-old child, initially misdiagnosed as orbital inflammatory disease and treated with oral steroids. A subsequent orbital biopsy three years later confirmed the diagnosis of Rosai Dorfman disease

A rare and an unusually delayed presentation of orbital actinomycosis following avulsion injury of the scalp.

We report a rare case of orbital swelling presenting one year after head trauma. An initial fine needle aspiration cytology revealed it to be an infected organizing hematoma. However, broad-spectrum antibiotics did not resolve the infection and the orbital lesion continued to grow in size, as evaluated by magnetic resonance imaging. Incisional biopsies were done, which were reported as orbital actinomycosis. Patient has responded well to treatment with penicillin. This case is of interest due to the delayed presentation of an orbital complication of head trauma and the rare infection with actinomyces. It also highlights the importance of using appropriate antibiotics, as well as the need for long-term treatment.

Disseminated orbital actinomycetoma: A case report.

Mycetoma is a chronic granulomatous infection. Lower extremities are commonly involved. A 20-year-old male came with complaints of multiple sinuses on scalp, left eyelid swelling with a sinus and dystopia, since one year. On examination there was relative proptosis in left eye of 2 mm. Computed tomography scan showed soft tissue swelling of the pre-septal area of the left upper eyelid with orbital involvement. Magnetic resonance imaging showed increased left orbital volume and evident dystopia. Microbiology testing of the erosive scalp and lid lesions showed genus Nocardia, suggestive of actinomycetoma. This case is presented as it shows an unusual involvement of the orbit.

Mucormicosis en una paciente diabética: caso clínico y revisión de la literatura / Mucormycosis in a diabetic patient: case report and literature review

Presentamos un caso de Mucormicosis Rinoorbitaria en una mujer de 48 años, diabética, en cetoacidosis, a quien se hizo el diagnóstico por histopatología. Tratada con anfotericina B intravenosa y un agresivo debridamiento quirúrgico para eliminar tejidos infartados y necróticos de gran parte de la hemicara y ojo derecho, logra sobrevivir a esta catastrófica infección. La mucormicosis es una infección aguda y extremadamente grave causada por hongos oportunistas y ubicuos del orden Mucoroles pertenecientes a la clase Phycomycetes, que afecta principalmente a un grupo de pacientes cuyas condiciones inmunológicas y metabólicas favorecen el desarrollo del hongo. Hacemos una definición del grupo de "pacientes en riesgo", lo que es extremadamente importante, ya que de la sospecha clínica se puede abordar un diagnóstico precoz para evitar la alta mortalidad y morbilidad que esta enfermedad provoca. Analizamos la patogenia de la enfermedad, así como una revisión de la literatura sobre modalidades de diagnóstico y terapéutica. El diagnóstico de urgencia sigue siendo de responsabilidad del patólogo, y el tratamiento de mayor éxito es la anfotericina B.

We present a case of rhinoorbital mucormycosis in a diabetic, developing ketoacidose, 48-year old woman. Diagnosis was established through histopathology. The patient was successfully treated and survives with a combination of anphotericin B and an aggressive surgery in order to eliminate all the ischemic and necrotic tissues affecting almost the entire right side of the face and intraorbital structures surviving to this catastrophic infection. Mucormycosis is an acute and often fatal infection caused by opportunistic fungus of the class Zygomycetes, order Mucoroles that affects a select group of patients associated with underlying metabolic and immunological disorders. A definition of "risk-group" is proposed. This definition is of extreme importance for a prompt diagnosis avoiding the high rate of morbidity and mortality associated with this condition. We review both pathogenic mechanism and literature related to diagnostic modalities and new therapeutical approaches. Emergency diagnosis is still in hands of pathologists and the most successful treatment is with anphotericin B.

Metastatic Esophageal Squamous Cell Carcinoma to the Orbit and Periorbit Masquerading as Periorbital Abscess

A 61-year-old man with no history of malignancy presented with a rapidly expanding left periorbital mass, first noticed one month prior to presentation. The mass was firm, and a pus-like discharge drained spontaneously from the center of the lesion. A biopsy was performed, and histopathology confirmed squamous cell carcinoma. Systemic evaluation revealed that the patient had a primary esophageal squamous cell carcinoma with multiple metastases. The prognosis of orbital metastasis is generally poor, and this patient expired after one month of conservative treatment.

Application of tele-ophthalmology in remote diagnosis and management of adnexal and orbital diseases.

Purpose: To assess the feasibility of making a diagnosis of adnexal and orbital diseases by Tele-ophthalmological means. Materials and Methods: Tele-consultation for eye diseases was done for 3497 patients from remote areas of Tamilnadu as part of the rural tele-ophthalmology project of a tertiary eye care hospital during a period of nine months from October 2004 to June 2005. These patients were comprehensively examined on-site by optometrists. Using digitized images sent by store and forward technique and videoconferencing, the ophthalmologist made a diagnosis and advised treatment. Results: Adnexal or orbital diseases were detected in 101 out of 3497 patients (2.88%). Medical treatment was advised to 13 of 101 patients (12.8%). Surgery was advised in 62 of 101 patients (61.28%) whereas 18 of 101 patients (17.8%) required further investigations at a tertiary center. Conclusion: It was feasible to apply the satellite based tele-ophthalmology set-up for making a presumptive diagnosis and planning further management of adnexal and orbital diseases based on live interaction and digital still images of the patients.

Liver cirrhosis and rhino-orbital mucormycosis, a possible but rare association: description of a clinical case and literature review

Only few cases of rhino-orbital mucormycosis in patients with liver cirrhosis are described in the literature and most of these patients showed an associated diabetes mellitus. We describe a case of rhino-orbital mucormycosis in a patient with liver cirrhosis without other risk factors.