Presentaciones de la sífilis anorrectal: la gran simuladora / Anorectal syphilis presentations: the great pretender

Antecedentes: La sífilis es una infección sexualmente transmisible sistémica crónica que afecta a docenas de millones de personas al año. A nivel anorrectal, su manifestación polimórfica obliga al diagnóstico diferencial con enfermedades anorrectales benignas y malignas. Objetivo: Describir las diferentes presentaciones de la sífilis anorrectal a propósito de 5 casos clínicos. Método: Estudio observacional, retrospectivo, descriptivo. Resultados: La mayoría de los pacientes fueron VIH positivos en edad sexual activa. Las manifestaciones registradas, al igual que las reportadas en la bibliografía fueron las fisuras, úlceras perianales y pseudotumores. Conclusiones: La sífilis es considerada "la gran simuladora". En la localización anorrectal se requiere una alta sospecha diagnóstica para diferenciarla de presentaciones similares de otras enfermedades anales benignas, la enfermedad inflamatoria intestinal y el cáncer anorrectal, con el fin de evitar el consiguiente riesgo de sobretratamiento. (AU)

Background: Syphilis is a chronic systemic sexually transmitted infection that affects tens of millions of people annually. At the anorectal level, its polymorphic manifestation requires differential diagnosis with benign and malignant anorectal diseases. Objective: To review the presentation of anorectal syphilis from 5 clinical cases. Methods: Observational, retrospective, descriptive study. Results: Most of the patients were HIV positive in sexually active age. The manifestations recorded and reported in the literature were fissures, perianal ulcers, and pseudotumors. Conclusions: Syphilis is considered "the great pretender". In anorectal syphilis, a high diagnostic suspicion is needed to differentiate it from similar presentations due to other anal conditions, inflammatory bowel disease, and anorectal cancer, to avoid the consequent risk of overtreatment. (AU)

Pseudotumor inflamatorioocular asociado a IgG4. Reporte de un caso / IgG4-Associated Ocular Inflammatory Pseudotumor. A Case Report

Resumen Introducción: El pseudotumor inflamatorio ocular asociado a IgG4 es un proceso inflamatorio no neoplásico y poco frecuente con una incidencia de 0.28-1.08 por cada 100,000 personas. El diagnóstico de esta patología es de exclusión debiendo cumplir criterios histopatológicos, agrandamiento de un órgano en específico y concentraciones serias de IgG4. El tratamiento tiene como objetivo evitar las complicaciones secundarias. Caso clínico: Mujer de 54 años de edad sin patologías previas referidas, acude refiriendo que 6 meses previos presenta de forma súbita y sin causa aparente prurito a nivel de globo ocular y párpado izquierdo, además de disminución de la agudeza visual y dolor retroocular, motivo por el que acude a valoración. Al examen físico presentó movimientos oculares normales, proptosis izquierda, dolor a la palpación, hiperemia subconjuntival, opacidad corneal, ausencia de reflejo rojo y más renitente a la presión el ojo izquierdo en relación con el contralateral. Tomografía cráneo-toraco-abdominal reporta a nivel ocular izquierdo imágenes sugestivas de melanoma coroideo sin hallazgos de actividad metastásica. Se realiza enucleación de ojo izquierdo. Se envía pieza a patología la cual reporta infiltrado difuso linfoplasmocítico positivas para IgG4. Conclusión: La enfermedad por IgG4 ocular es una patología muy infrecuente de clínica inespecífica y diagnóstico complejo. Sin embargo, un abordaje rápido y correcto es fundamental para evitar complicaciones.

Abstract Introduction: IgG4-associated ocular inflammatory pseudotumor is a rare, non-neoplastic inflammatory process with an incidence of 0.28-1.08 per 100,000 people. The diagnosis of this pathology is one of exclusion, having to meet histopathological criteria, enlargement of a specific organ, and serious concentrations of IgG4. Treatment aims to avoid secondary complications. Clinical case: A 54-year-old woman with no previous reported pathologies, reports that for the past 6 months she has itching at the level of the eyeball and the left eyelid, suddenly and without apparent cause, in addition to decreased visual acuity and retro-ocular pain; this is why she asked for the evaluation. The physical examination revealed normal eye movements, left proptosis, pain on palpation, subconjunctival hyperemia, corneal opacity, absence of red reflex, and the left eye being more resistant to pressure in relation to the contralateral eye. The cranio-thoraco-abdominal tomography reported images at the left ocular level that were suggestive of choroidal melanoma without findings of metastatic activity. Enucleation of the left eye was performed. The specimen was sent to pathology which reported diffuse lymphoplasmacytic infiltrate positive for IgG4. Conclusion: Ocular IgG4 disease is a very rare pathology with non-specific symptoms and complex diagnosis. However, a quick and correct approach is essential to avoid complications.

Optic nerve sheath fenestration: A second lease at sight

Purpose: To study the safety and efficacy of optic nerve sheath fenestration surgery in patients with optic disc edema due to different etiologies. Methods: Records of 18 eyes of 15 patients who underwent optic nerve sheath fenestration for vision threatening optic disc edema were reviewed retrospectively, and results were analyzed. Improvement of visual acuity was the main measure of outcome. Improved visual fields, resolution of optic disc edema, diplopia, and headache were other benefits that were observed. Results: Fifteen patients between 13 and 54 years of age were included in the study. Three patients underwent successive bilateral surgery. Idiopathic intracranial hypertension was the most common cause for optic disc edema and was found in 80% of the patients. Mean preoperative logMAR acuity was ?1.9789 ± 1.46270, which improved to ?0.9022 ± 1.23181 (p < 0.005) in the operated eye, and mean logMAR acuity of contralateral eye improved from ?1.3378 ± 1.50107 to ?1.0667 ± 1.33813 (p < 0.05). Conclusion: Early optic nerve sheath fenestration is an effective modality for treating optic disc edema due to a wide myriad of causes and helps resolve the associated symptoms.

Tumor inflamatorio miofibroblástico gastrointestinal. Reporte de caso / Gastrointestinal myofibroblastic inflammatory tumor. Case report

Introducción. El tumor miofibroblástico inflamatorio es una enfermedad proliferativa rara, de etiología incierta, caracterizada por la proliferación de miofibroblastos epitelioides o fusionados mezclados con células inflamatorias, predominantemente mononucleares. En general se considera una lesión benigna, aunque en algunos casos esta neoplasia ha mostrado un comportamiento agresivo en cuanto a recidiva local y metástasis. El tratamiento definitivo es la resección quirúrgica completa. Caso clínico. Paciente de 67 años con dos meses de evolución de fiebre y masa abdominal, en quien se realizó una tomografía computarizada de abdomen que identificó una lesión de aspecto infiltrativo tumoral, comprometiendo la grasa retroperitoneal en la transcavidad de los epiplones. Por vía percutánea se tomó una biopsia que informó un pseudotumor inflamatorio retroperitoneal. Fue llevado a cirugía radical abdominal, con patología quirúrgica final que describió un tumor miofibroblástico inflamatorio de compromiso multifocal, adherido a la serosa del estómago e intestino delgado, sin compromiso muscular. Discusión. El tumor inflamatorio miofibroblástico es una entidad rara, de etiología por esclarecer y difícil diagnóstico. Presentamos el caso clínico de un paciente con tumor inflamatorio miofibroblástico gastrointestinal.Conclusión. Se describe el caso clínico de un paciente con un tumor inflamatorio miofibroblástico gastrointestinal, de presentación rara en nuestro medio. Es importante la comparación con casos similares para poder hacer conclusiones útiles en la práctica clínica

Introduction. Inflammatory myofibroblastic tumor is a rare proliferative disease of uncertain etiology, characterized by the proliferation of epithelioid or fused myofibroblasts mixed with predominantly mononuclear inflammatory cells. In general, it is considered a benign lesion, although in some cases this neoplasm has shown aggressive behavior in terms of local recurrence and metastasis. The definitive treatment is complete surgical resection. Clinical case. A 67-year-old patient with a two-month history of fever and an abdominal mass underwent a computed tomography scan of the abdomen that identified an infiltrative tumor, compromising the retroperitoneum fat in the lesser cavity. A biopsy was taken percutaneously, which reported a retroperitoneal inflammatory pseudotumor. He was taken to radical abdominal surgery, with final surgical pathology describing an inflammatory myofibroblastic tumor with multifocal involvement attached to the serosa of the stomach and small intestine without muscle involvement. Discussion. Inflammatory myofibroblastic tumor is a rare entity, of unknown etiology and difficult to diagnose. We present a clinical case of gastrointestinal myofibroblastic inflammatory tumor to better understand this entity.Conclusion. The clinical case of a patient with a gastrointestinal myofibroblastic inflammatory tumor, a rare presentation in our environment, is described. Comparison with similar cases is important to draw useful conclusions in clinical practice

Síndrome de hipertensión intracraneana como manifestación inhabitual del síndrome de Sjögren. Caso clínico

Neurological manifestations such as polyneuropathy are reported in 8-49% of cases with Sjögren's Syndrome (SjS), but central nervous system involvement is seldom described. We report a 46-year-old woman with a history of SjS with distal renal tubular acidosis and autoimmune thyroiditis. She consulted in the emergency room for a five-days history of holocranial headache and explosive vomiting. Fundoscopy showed bilateral papilledema. Brain computed tomography (CT) without contrast showed diffuse encephalic edema, with effacement ofsulci and restriction ofperitruncal cisterns. Brain AngioCT ruled out thrombosis, and brain magnetic resonance (MRI) was without structural alterations or hydrocephalus. Lumbar puncture had increased cerebrospinal fluid output pressure but without cytochemical alterations, and negative gram, cultures and filmarray. The diagnosis of Intracranial Hypertension Syndrome (ICHTS) ofprobable autoimmune etiology in the context of SjS was proposed, and management with high-dose corticosteroids was initiated with favorable clinical and imaging response.

Sclerosing angiomatoid nodular transformation of spleen with calcifying fibrous pseudotumor: A rare case with unusual association

Sclerosing angiomatoid nodular transformation (SANT) of the spleen is a rare benign vascular lesion of the spleen that was first described in 2004. SANT is associated with other concurrent diseases, mostly malignancies. Calcifying fibrous pseudotumor (CFPT) is a tumor-like lesion usually arising from soft tissue or peritoneal sub-serosa. Both SANT and CFPT are considered to be variants of the inflammatory myofibroblastic tumor. We report the rare case of a 24-year-old female presenting with a left abdominal mass that was clinically diagnosed as an extraintestinal gastrointestinal stromal tumor (GIST). Histopathological examination revealed SANT of spleen and CFPT. We report this case due to its rarity of occurrence and unusual association of SANT with CFPT.

Papiledema bilateral por síndrome do pseudotumor cerebral em paciente com achado fortuito de microadenoma hipofisário / Bilateral papilledema by cerebral pseudotumor syndrome in a patient with a fortuitous finding of pituitary microadenoma

RESUMO O presente relato descreve um raro caso de papiledema bilateral decorrente da síndrome do pseudotumor cerebral relacionado com achado de microadenoma hipofisário. Relatamos um caso de paciente do sexo feminino, 21 anos, referindo baixa acuidade visual para longe, associada à cefaleia de início há 2 meses. Ao exame oftalmológico, a fundoscopia mostrou presença de papiledema bilateral significativo. A ressonância magnética de encéfalo evidenciou imagem nodular na hipófise, medindo 7 mm, sem qualquer evidência de compressão intracraniana, insuficiente para causar os sintomas do quadro clínico. Após diagnóstico de síndrome do pseudotumor cerebral, instituiu-se tratamento clínico com acetazolamida e orientou-se perda de peso, com evolução satisfatória. A descrição de ambas as patologias presentes de forma mútua foi citada apenas uma vez durante extensa revisão da literatura. Dessa forma, chamamos a atenção para essa ocorrência incomum, enfatizando as características de ambas as patologias, a fim de facilitar o diagnóstico diferencial, bem como elucidar a melhor abordagem terapêutica. Ressaltamos que o achado de pequenos adenomas hipofisários não deve confundir o diagnóstico etiológico em pacientes com queixas visuais e papiledema bilateral.

ABSTRACT The present report describes a rare case of bilateral papilledema due to cerebral pseudotumor syndrome (CPT) related to an occasional finding of pituitary microadenoma. We report the case of a 21-year-old female patient presenting low far visual acuity, associated with headache beginning 2 months ago. On ophthalmological examination, fundoscopy showed significant bilateral papilledema. Magnetic resonance imaging of the brain showed symmetrical and normal-sized ventricles, besides a nodular imaging in the pituitary, measuring 7 mm, without any evidence of intracranial compression, insufficient to cause the symptoms of the clinical presentation. After the diagnosis of cerebral pseudotumor syndrome, clinical treatment with acetazolamide was instituted and weight loss was advised, with satisfactory evolution. The description of both pathologies mutually present was mentioned only once during extensive literature review. Thus, we call attention to this unusual occurrence, emphasizing the characteristics of both pathologies in order to facilitate the differential diagnosis, as well as to elucidate the best therapeutic approach. We emphasize that the finding of small pituitary adenomas should not confuse the etiological diagnosis in patients with visual complaints and bilateral papilledema.

Pseudotumeur inflammatoire colique / Colic inflammatory pseudotumor

Introduction : Les pseudotumeurs inflammatoires coliques sont des lésions bénignes très rares, d'étiologie inconnue et dont le diagnostic différentiel est difficile avec les adénocarcinomes. Résultat : Nous rapportons le cas d'une patiente de 20 ans, admise en urgence dans un contexte d'occlusion intestinale aiguë chez qui en per opératoire, nous avons retrouvé une tumeur ulcéro -nécrotique du côlon droit suspecte de malignité. Mais l'examen histologique de la pièce d'hémi colectomie a affirmé sa bénignité. Conclusion : Les pseudo-tumeurs inflammatoires bien que rares, ne sont pas exceptionnelles. Elles sont de diagnostic difficile car peuvent simuler des tumeurs malignes et l'examen histologique est indispensable pour affirmer le diagnostic

Introduction: Colonic inflammatory pseudotumors are rare benign lesions, they are of unknown etiology and the differential diagnosis is difficult with adenocarcinomas. Result: We report the case of a 20-year-old patient admitted urgently in an acute intestinal occlusion in which laparotomy has objectified an ulcerative-necrotic tumor of the right colon suspected of malignancy; it is the histological examination of the piece of hemicolectomy which affirmed its benignity. Conclusion: Inflammatory pseudo-tumors, although rare, are not exceptional; they are difficult to diagnose because they can simulate malignant tumors and histological examination is essential to assert the diagnosis

CT features of inflammatory pseudotumor like follicular dendritic cell sarcoma of the spleen / 中华肝胆外科杂志

Objective:To explore the CT features of inflammatory pseudotumor like follicular dendritic cell sarcoma (FDCS) of the spleen.Methods:The clinical data of 12 patients with splenic inflammatory pseudotumor like FDCS admitted to 3 central hospitals including Yongjia People's Hospital in Zhejiang Province from January 2015 to December 2022 were retrospectively analyzed, including 4 males and 8 females, with a median age of 60 years old. The number, shape, size and CT features of the lesions were analyzed based on patient's CT image data.Results:CT scans of 12 patients showed 15 lesions, including 10 single lesions and 2 multiple lesions. The lesions were circular in 5 cases, elliptical in 4 cases, and irregular in 3 cases. The median maximum diameter of the mass is 6.5 cm. On plain scan, all 12 tumors showed low density or slightly low density. The CT value is (41.3±7.2) HU; 8 cases had uneven density and 4 cases had uniform density. There were 8 cases with clear tumor boundaries and 4 cases with unclear boundaries. There were 8 cases with tumor necrosis and cystic transformation, and 5 cases showed patchy bleeding lesions in the center of the tumor. Enhancement: the arterial phase shows small patches or flocculent enhancement at the edges or parenchymal parts of the tumor, with CT value of (56.0±3.8) HU. Among them, there were 7 cases of mild enhancement, 4 cases of moderate enhancement, and 1 case of significant enhancement. During the portal phase, there was mild to moderate persistent small patchy uneven enhancement, with CT value of (62.0±4.3) HU. Among them, there were 8 cases of mild enhancement and 4 cases of moderate enhancement. The delayed phase showed a slow withdrawal of enhancement, with CT value of (45.0±8.2) HU. All 12 cases underwent complete resection and were diagnosed with FDCS through pathological examination.Conclusion:FDCS plain scan shows circular or elliptical uneven low-density masses, with small patches or flocculent light to moderate uneven enhancement in the arterial phase, continuous enhancement in the portal phase, and slow withdrawal in the delayed phase as the main characteristics.

Cerebrospinal fluid levels of sortilin-1, lipocalin-2, autotaxin, decorin and interleukin-33 in patients with idiopathic intracranial hypertension / Níveis de sortilina-1, lipocalina-2, autotaxina, decorina e interleucina-33 no líquido cefalorraquidiano em pacientes com hipertensão intracraniana idiopática

Abstract Background Idiopathic intracranial hypertension (IIH) is characterized by increased cerebrospinal fluid (CSF) pressure of unknown cause. It has been suggested that the inflammatory process plays a role in the pathophysiology of the disease. Sortilin-1, lipocalin-2, autotaxin, decorin, and interleukin-33 (IL-33) are among the factors involved in inflammatory processes. Objective To investigate the CSF levels of sortilin-1, lipocalin-2, autotaxin, decorin, and IL-33 in patients with IIH. Methods A total of 24 IIH patients and 21 healthy controls were included in the study. Demographic characteristics of the patients and of the control group as well as CSF pressures were evaluated. Sortilin-1, lipocalin-2, autotaxin, decorin and IL-33 levels in the CSF were measured. Results The CSF levels lipocalin-2, sortilin-1, autotaxin, IL-33 and CSF pressure were significantly higher in the patients group compared with the control group (p < 0.001). Decorin levels were reduced in patients (p < 0.05). There was no correlation between the autotaxin and IL-33 levels and age, gender, CSF pressure, and body mass index. The results of our study showed that inflammatory activation plays an important role in the development of the pathophysiology of IIH. In addition, the fact that the markers used in our study have never been studied in the etiopathogenesis of IIH is important in explaining the molecular mechanism of this disease. Conclusion Studies are needed to evaluate the role of these cytokines in the pathophysiology of the disease. It is necessary to evaluate the effects of these molecules on this process.

Resumo Antecedentes A hipertensão intracraniana idiopática (HII) é caracterizada pelo aumento da pressão do líquido cefalorraquidiano (LCR) de causa desconhecida. Tem sido sugerido que o processo inflamatório desempenha um papel na fisiopatologia da doença. Sortilina-1, lipocalina-2, autotaxina, decorina e interleucina-33 (IL-33) estão entre os fatores envolvidos nos processos inflamatórios. Objetivo Investigar os níveis de sortilina-1, lipocalina-2, autotaxina, decorina e IL-33 no LCR de pacientes com HII. Métodos Um total de 24 pacientes com HII e 21 controles saudáveis foram incluídos no estudo. Foram avaliadas as características demográficas dos pacientes e do grupo controle, bem como as pressões liquóricas. Os níveis de sortilina-1, lipocalina-2, autotaxina, decorina e IL-33 no LCR foram medidos. Resultados Os níveis no líquido cefalorraquidiano lipocalina-2, sortilina-1, autotaxina, IL-33 e pressão liquórica foram significativamente maiores no grupo de pacientes em comparação com o grupo controle (p < 0,001). Os níveis de decorina foram reduzidos nos pacientes (p < 0,05). Não houve correlação entre os níveis de autotaxina e IL-33 e idade, sexo, pressão liquórica e índice de massa corporal. Os resultados do nosso estudo mostraram que a ativação inflamatória desempenha um papel importante no desenvolvimento da fisiopatologia da HII. Além disso, o fato de os marcadores utilizados em nosso estudo nunca terem sido estudados na etiopatogenia da HII é importante para explicar o mecanismo molecular dessa doença. Conclusão Estudos são necessários para avaliar o papel dessas citocinas na fisiopatologia da doença. É necessário avaliar os efeitos dessas moléculas nesse processo

Pseudotumor inflamatorio hepático, una entidad de difícil diagnóstico / Inflammatory pseudotumor of the liver: a condition hard to diagnose

RESUMEN El pseudotumor inflamatorio hepático es una lesión muy infrecuente, sin una etiología ni patogenia claras. Su diagnóstico preoperatorio no es habitual pero, en caso de realizarse, puede evitar la cirugía. Presentamos el caso de un paciente joven, con antecedente de lupus cutáneo que, tras debutar con una pancreatitis aguda, presenta episodios de colangitis y cuyos hallazgos radiológicos no permiten descartar la presencia de un colangiocarcinoma, por lo que se realiza hepatectomía izquierda, siendo el diagnóstico histológico final de pseudotumor inflamatorio hepático.

ABSTRACT Inflammatory pseudotumors of the liver are rare and lack clear etiology and pathogenesis. The preoperative diagnosis is seldom made but it avoids unnecessary surgery. We report the case of a young male patient with a history of cutaneous lupus and episodes cholangitis after an acute pancreatitis. As the imaging tests could not rule out cholangiocarcinoma, a left liver resection was performed, and the final histologic diagnosis was inflammatory pseudotumor of the liver.

Idiopathic intracranial hypertension: an illustrated guide for the trainee radiologist / Hipertensão intracraniana idiopática: um guia ilustrado para o residente de radiologia

Abstract Idiopathic intracranial hypertension is characterized by increased intracranial pressure, headache, and visual perturbations. Although the pathophysiology of idiopathic intracranial hypertension is obscure, several mechanisms have been proposed, such as increased cerebral blood volume, excessive cerebrospinal fluid volume (due to high production or impaired resorption), and inflammatory mechanisms as a likely cause of or contributor to impaired cerebrospinal fluid circulation. It predominantly affects women of reproductive age who are overweight or obese. The most common symptoms are daily headache, synchronous pulsatile tinnitus, transient visual perturbations, and papilledema with visual loss. The main neuroimaging findings are a partially empty sella turcica; flattening of the posterior sclera; transverse sinus stenosis (bilateral or in the dominant sinus); a prominent perioptic subarachnoid space, with or without optic nerve tortuosity; and intraocular protrusion of the optic nerve head. The main complication of idiopathic intracranial hypertension is visual loss. Within this context, neuroimaging is a crucial diagnostic tool, because the pathology can be reversed if properly recognized and treated early.

Resumo A hipertensão intracraniana idiopática é caracterizada por aumento da pressão intracraniana, cefaleia e manifestações visuais. Apresenta fisiopatologia incerta, porém, alguns mecanismos já foram propostos, como o aumento do volume sanguíneo cerebral, o excesso de líquor por aumento da produção ou a redução da reabsorção, e mecanismos inflamatórios como fator causal ou mesmo determinando limitação na circulação do líquor. Predomina em mulheres obesas em idade reprodutiva. Os sintomas e sinais mais comuns são cefaleia diária, zumbido síncrono ao pulso, obscurecimentos visuais transitórios e papiledema com perda visual. Os principais achados em neuroimagem são: sela turca vazia, achatamento posterior do globo ocular/esclera, estenose do seio transverso bilateral ou do seio dominante, distensão do espaço liquórico perióptico com ou sem tortuosidade do nervo óptico e protrusão intraocular da cabeça do nervo óptico. A principal complicação da hipertensão intracraniana idiopática é a perda visual. Nesse contexto, o papel da neuroimagem no diagnóstico é fundamental, pois a doença pode ser revertida se devidamente reconhecida e precocemente tratada.

Measuring optic nerve sheath diameter using ultrasonography in patients with idiopathic intracranial hypertension / Medição do diâmetro da bainha do nervo óptico por ultrassonografia em pacientes com hipertensão intracraniana idiopática

Abstract Background: Idiopathic intracranial hypertension (IIH) is primarily a disorder of obese young women characterized by symptoms associated with raised intracranial pressure in the absence of a space-occupying lesion. Objective: To compare the mean optic nerve sheath diameter (ONSD) measured using ultrasonography (USG) in patients with idiopathic intracranial hypertension (IIH) and normal healthy individuals. Methods: A prospective study. Ninety-seven participants aged 18-80 years were divided into two groups as patients with IIH (n=47) and the control group (n=50). The ONSD was measured using ultrasound with a 10-MHz probe. ONSD was measured 3 mm behind the optic disc. Receiver operating characteristic (ROC) curve analysis was performed to determine patients with IIH using ONSD. Results: Body mass index was higher in the IIH group compared with the control group (p=0.001). The mean ONSD was statistically significantly thicker in the IIH group (6.4 mm) than in the control group (4.90 mm). The cut-off value of ONSD in patients with IIH was measured as 5.70 mm. There was a significant negative correlation between ONSD and age (r:-0.416 and p<0.001). There was a positive correlation between BMI and ONSD (r: 0.437 and p<0.001). Conclusions: Ultrasound can be a reliable, non-invasive and rapid tool to measure ONSD in monitoring patients with IIH. After the first diagnosis of IIH, based on neuroimaging and measuring intracranial pressure using invasive methods, ONSD can be used in treatment and follow-up.

Resumo Antecedentes: A hipertensão intracraniana idiopática (HII) é primariamente um distúrbio de mulheres jovens obesas caracterizado por sintomas e sinais associados à pressão intracraniana elevada na ausência de uma lesão ocupante de espaço. Objetivo: Comparar o diâmetro médio da bainha do nervo óptico (ONSD) medido por ultrassonografia (USG) em pacientes com hipertensão intracraniana idiopática (HII) e indivíduos normais e saudáveis. Métodos: Estudo prospectivo. Noventa e sete participantes com idade entre 18-80 anos foram divididos em dois grupos: pacientes com HII (n=47) e o grupo controle (n=50). O ONSD foi medido por ultrassonografia com uma sonda de 10 MHz. O ONSD foi medido 3 mm atrás do disco óptico. A análise da curva ROC foi realizada para determinar pacientes com HII usando-se o ONSD. Resultados: O índice de massa corporal foi maior no grupo HII comparado ao grupo controle (p=0,001). O ONSD médio foi estatística e significativamente mais espesso no grupo HII (6,4 mm) do que no grupo controle (4,90 mm). O valor de corte do ONSD em pacientes com HII foi medido em 5,70 mm. Houve correlação negativa significativa entre ONSD e idade (r:-0,416 ep<0,001). Houve correlação positiva entre IMC e ONSD (r: 0,437 ep<0,001). Conclusões: A ultrassonografia pode ser uma ferramenta confiável, não invasiva e rápida para medir o ONSD no monitoramento de pacientes com HII. Após o primeiro diagnóstico de HII, com base em neuroimagem e na medida da pressão intracraniana por métodos invasivos, o ONSD pode ser utilizado no tratamento e acompanhamento.

Pseudotumor hemofílico secundario a fractura expuesta de tibia / Hemophilic pseudotumor secondary to an open fracture of the tibia

Introducción: El pseudotumor hemofílico consiste en un hematoma encapsulado de crecimiento progresivo debido a repetidos episodios de hemorragia en pacientes con hemofilia. Objetivo: Evaluar la recuperación de un paciente con una lesión compleja y poco frecuente que le causaba limitaciones a su vida cotidiana. Presentación de caso: Paciente masculino de 36 años de edad, con antecedentes patológicos personales de hemofilia A grave, que presentaba aumento de volumen en la pierna derecha posterior a un trauma sufrido a ese nivel. Llevó tratamiento conservador por más de 10 años, período en que la lesión continúo aumentando de tamaño, el cual progresó considerablemente en los últimos 3 años, por lo que requirió tratamiento quirúrgico. Conclusiones: La complejidad del paciente no solo radica en la afección en la región medial de la tibia, sino por la enfermedad de base, demuestra que se requiere un diagnóstico lo más certero y precoz posible para adoptar una conducta adecuada y rápida(AU)

Introduction: Hemophilic pseudotumor consists of an encapsulated hematoma with progressive growth, due to repeated bleeding episodes in patients with hemophilia. Objective: To evaluate the recovery of a patient with a complex and rare injury that already caused limitations to his daily life. Case presentation: 36-year-old male patient with a personal pathological history of severe hemophilia A, who presented an increase in volume in the right leg after a trauma that he suffered at that level. As time went by, he began to present an increase in volume in the distal middle region of his right leg for more than 10 years, which progressed considerably in the last 3 years, requiring surgical treatment. Conclusions: The complexity of the patient is not only given by the condition in the medial region of the tibia, but by the underlying disease shows that a diagnosis as accurate and early as possible is required, to adopt an adequate and rapid behavior(AU)

Headaches and obesity / Obesidade e cefaleia

ABSTRACT Obesity and headache disorders are two very common conditions in the general population that have been increasing in incidence over the last decades. Recent studies have shown a significant relationship between obesity and headaches, particularly migraine, with an important role in whether the disease is chronic. On the other hand, no such association was found with tension-type headaches. Studies showing an overlapping of hunger-control pathways and those involved in the pathophysiology of migraine may justify the close association between obesity and migraine. Moreover, a secondary headache for which obesity is a strong risk factor is idiopathic Intracranial Hypertension (pseudotumor cerebri), with several studies showing the impact of weight reduction/bariatric surgery in the treatment of the disease. In conclusion, since obesity is a modifiable risk factor, it is important for physicians treating patients with headaches, and particularly migraine, to be aware of the association between these two disorders.

RESUMO Obesidade e cefaleias são duas condições muito frequentes na população geral e que vem aumentando em incidência nas últimas décadas. Estudos recentes têm demonstrado uma significativa relação entre obesidade e cefaleia, particularmente na enxaqueca, com papel importante na cronificação da doença. Por outro lado, não se observa tal associação com cefaleia do tipo tensão. Trabalhos que mostram uma sobreposição das vias de controle da fome e daquelas envolvidas na fisiopatologia da enxaqueca podem justificar a íntima associação entre obesidade e enxaqueca. Além disso, uma cefaleia secundária para a qual a obesidade é um forte fator de risco é a Hipertensão Intracraniana Idiopática (pseudotumor cerebral), inclusive com diversos estudos mostrando o impacto da redução do peso/cirurgia bariátrica no tratamento da doença. Concluindo, visto que a obesidade é um fator de risco modificável, é importante para os médicos que tratam de pacientes com cefaleia e, particularmente, enxaqueca, estarem cientes da associação entre essas duas comorbidades.

Update on Idiopathic Intracranial Hypertension Management / Atualização no manejo da Hipertensão Intracraniana Idiopática

ABSTRACT Background: Idiopathic Intracranial Hypertension (IIH) is a secondary headache with a steadily growing incidence. Currently, there is little evidence to guide the treatment of IIH. Objective: To review the pathophysiology of IIH, with focus on the role of obesity as a risk factor, and the implications for new therapeutic perspectives. Methods: in this narrative review, we summarized the current knowledge on treatment options highlighting available evidence for managing intracranial hypertension, obesity, and headache. Results: Clinical Presentation: headache is the most common symptom and a significant cause of quality-of-life impairment. Visual loss is common in the diagnosis. Pathophysiology: there is no unified theory able to explain all symptoms and the evolution of the disease. There is growing data pointing to metabolic changes and obesity with a central role in IIH pathophysiology. Treatment: most published data on IIH treatment is related to pressure control and protection from visual loss. Acetazolamide and cerebrospinal fluid diversion are the best options available. Optic nerve sheath fenestration might be useful to temporally control the pressure over the optic nerve and thus protect from visual deterioration. Recently, venous sinus stenting has proven to be a safe option in selected cases. Finally, bariatric surgery has proven to effectively control elevated intracranial pressure. Conclusion: IIH is a potential cause of high disability. Early recognition is important, and treatment should be tailored to the needs of each case. There is a lack of research on headache management, which might persist after ICP control.

RESUMO Antecedentes: A Hipertensão Intracraniana Idiopática (HII) é uma cefaleia secundária com incidência crescente. Atualmente há pouca evidência disponível na literatura referente ao manejo da HII. Objetivo: O entendimento da fisiopatologia e o papel central da obesidade como fator de risco para HII abriu novas perspectivas de tratamento. Métodos: nessa revisão narrativa, objetivamos revisar as principais opções de tratamento disponíveis atualmente para o manejo da HII, controle da obesidade e da cefaleia. Resultados: Apresentação clínica: a cefaleia é o sintoma mais comum e uma importante causa de impacto na qualidade de vida, e o déficit visual é um achado comum no diagnóstico. Fisiopatologia: atualmente não existe uma teoria unificada capaz de explicar satisfatoriamente os sintomas e a evolução da doença. Um crescente corpo de evidências aponta para o papel central das alterações metabólicas e da obesidade na fisiopatologia da HII. Tratamento: a maioria dos dados publicados sobre HII estão relacionados a medidas para controle da hipertensão intracraniana e proteção da visão. Acetazolamida e Derivação Ventriculo Peritoneal são as principais alternativas com esse fim. A fenestração do nervo óptico pode ser útil como medida termporaria de controle da pressão sobre o nervo óptico e proteção contra a progressão do déficit visual. Cirurgia bariátrica se mostrou efetiva no controle da pressão intracraniana. Conclusão: A HII é uma causa importante de incapacidade cujo reconhecimento precoce é importante. O tratamento deve ser individualizado. Atualmente há uma importante defasagem de evidências sobre o manejo da cefaleia nesse grupo de pacientes.

Differential Diagnoses of Diseases Involving the Extrinsic Ocular Musculature ­ A Pictorial Essay

Introduction There are some inflammatory, infectious, and neoplastic diseases affecting the extrinsic orbital musculature (EOM) that present with pain, decreased visual acuity, and proptosis. Imaging is fundamental to the differential diagnoses of these diseases with similar clinical presentations. The present case series report has as main objective to illustrate and discuss the main pathologies that affect the orbit. Material and Methods The present series of cases discusses the main pathologies that can affect the extraocular musculature that can be characterized by computed tomography (CT) or magnetic resonance imaging (MRI) using cases from our institution. Results and Discussion The present study compiled several cases of ophthalmopathy from our institution to illustrate and address some of these pathologies, such as orbital lymphoma, Grave disease, metastases, periorbital cellulitis, and idiopathic orbital inflammatory syndrome. The diseases are discussed according to the presentation of clinical cases with emphasis on the main imaging findings of each pathology. Conclusion Computed tomography and MRI can help in the diagnosis and follow-up of the diseases that affect the EOM. We must be conversant with the main characteristics of the pathologies presented in the present case series report, since such findings together with clinical data can confirm the diagnosis of these diseases or at least help to narrow the differential diagnoses.

A Case of Hemophilia B with Abdominal and Pelvic Pseudotumor / 罕见病研究

Hemophilia B is a genetic disorder caused by coagulation factor Ⅸ(FⅨ) deficiency, mainly manifesting as joint, muscle and deep tissue bleeding. Hemophilia pseudotumor is a mass formed by soft tissue liquefaction and necrosis caused by repeated bleeding. Most pseudotumors occur in the bone and muscle. We report a case of hemophilia B with pseudotumor formation in the pelvis and abdomen, where lesion location is relatively rare. After active and effective hemostasis, the patient's hematuria symptom gradually improved. This case suggests that early and timely hemostatic treatment is crucial for patients with hemophilia.

Pseudotumor toraco-abdominal asociado a Bartonella henselae y Eikenella corrodens en un paciente pediátrico inmunocompetente / Thoraco-abdominal pseudotumor associated with Bartonella henselae and Eikenella corrodens in an immunocompetent patient

Resumen Los pseudotumores inflamatorios son poco frecuentes y escasamente descritos en la literatura y han sido asociados a infecciones polimicrobianas. Se presenta el caso de un niño de 9 años, procedente de Bolivia, quien consultó por dolor abdominal, baja de peso y vómitos, diagnosticándose un pseudotumor tóraco-abdominal. El laboratorio clínico, mediante el estudio de biología molecular en tejido, permitió la identificación de uno de los probables agentes etiológicos.

Abstract Inflammatory pseudotumors are a rare pathology and scarcely reported in the literature and have been associated with polymicrobial infections. Here, we present the case of a 9 years old boy from Bolivia, who presented with abdominal pain, weight loss and vomiting, who was diagnosed with a thoraco-abdominal pseudotumor. The micro-biology and molecular laboratories in tissue allowed the identification of one of the probable etiological agents.

Unilateral papilledema and peripapillary polypoidal choroidal vasculopathy as the presenting manifestations of intracranial hypertension / Papiledema unilateral e vasculopatia polipoidal da coroide peripapilar como sinais de apresentação de hipertensão intracraniana

ABSTRACT We have reported here the case of a 54-year-old woman with intracranial hypertension that presented with the unique features of unilateral papilledema and peripapillary polypoidal choroidal vasculopathy. Our investigations lead to the diagnosis of idiopathic intracranial hypertension and an incidental small right frontal meningioma. The patient was accordingly treated with oral acetazolamide, followed by three consecutive monthly intravitreal injections of bevacizumab, which resulted in the inactivation of the polypoidal choroidal vasculopathy, marked reduction of lipid exudation, and complete absorption of the subretinal fluid. This case serves as the first documentation of polypoidal choroidal vasculopathy associated with papilledema. It also demonstrates that choroidal vascular abnormalities may occur even when optic disk edema is unilateral, which is an uncommon manifestation of increased intracranial pressure. Prompt recognition of such findings and its appropriate management are essential for adequate treatment and prevention of irreversible visual loss.

RESUMO Relatamos um caso de uma paciente de 54 anos com hipertensão intracraniana que apresentava achados atípicos de papiledema unilateral e vasculopatia polipoidal da coroide peripapilar. A investigação levou ao diagnóstico de hipertensão intracraniana idiopática e de um pequeno meningioma incidental. A paciente foi tratada com acetazolamida por via oral, seguida de três injeções intravítreas mensais de bevacizumabe, resultando em inatividade da vasculopatia polipoidal da coroide, redução da exsudação e completa absorção do líquido subretiniano. A apresentação deste caso serve para documentar pela primeira vez vasculopatia polipoidal da coroide associada a papiledema. Ele também demonstra que podem ocorrer anormalidades vasculares da coroide mesmo quando o edema do disco óptico é unilateral, uma manifestação incomum do aumento da pressão intracraniana. O reconhecimento imediato desses achados e seu manejo adequado são essenciais para o tratamento adequado e para prevenção da perda visual irreversível.