Rol de las imágenes en la patología arterial no ateroesclerótica poco frecuente en el abdomen / Role of images in rare vascular pathology in the abdomen

Resumen La patología arterial no ateromatosa constituye un grupo de patologías poco frecuentes, pero de gran importancia debido a su morbilidad y mortalidad asociadas. La presentación clínica de estas entidades es inespecífica, por lo que el estudio inicial con imágenes es fundamental. Debido a esto, es muy importante reconocer los signos radiológicos característicos de cada una en las diversas modalidades imagenológicas para poder sospechar y orientar el diagnóstico.

Abstract Non-atheromatous arterial pathology constitutes a group of infrequent pathologies but of great importance due to their associated morbidity and mortality. In general, the clinical presentation of these pathologies is quite nonspecific, so the clinician decides to perform imaging studies thinking of more common entities. It is for this reason that it is very important to recognize characteristic radiological signs in the various imaging modalities to be able to suspect and orient a pathology of this kind.

Surviving complications of retroperitoneal fibrosis with intermittent relapses over a period of 20 years: case report and literature review / Sobrevivir a las complicaciones de la fibrosis retroperitoneal con recaídas intermitentes durante un período de 20 años: reporte de un caso y revisión de la literatura

Idiopathic retroperitoneal fibrosis is a rare fibro-inflammatory disease of varied etiology which usually originates around aorta and spreads caudally along Iliac vessels into adjacent retroperitoneum causing ureteral obstruction as the most frequent complication.A 53-year-old male patient presented with complaint of mild pain in both the legs off and on. On investigating further, we found that he had been struggling with intermittent relapses every 3-4 years for last 20 years since he was first diagnosed with Idiopathic Retroperitoneal Fibrosis. He was 33-year-old when he first developed the symptoms of anuria for 48 hours and was diagnosed with Idiopathic retroperitoneal fibrosis. This was followed by atrophy of left kidney and hypertension 6 years later, then hypothyroidism after another 3years and finally involvement of Inferior Vena Cava and acute Deep Vein Thrombosis of lower limbs after another 3-4 years. His deep vein thrombosis was well managed in time. He was put on glucocorticoids everytime he had a relapse and a complication.We did a review of literature to understand recent advances about its pathogenesis, diagnosis, investigations and management. We searched in PubMed using terms like retroperitoneal fibrosis alone and in combination with related terms such as Inferior Vena Cava thrombosis, Deep Vein Thrombosis, Tamoxifen, Methotrexate. This case is unique as it is very rare to find acute Deep Vein Thrombosis in Idiopathic retroperitoneal fibrosis without development of any collaterals when Inferior Vena Cava lumen is compromised to almost complete obstruction.After a follow up of 20 years patient is doing well in terms of physical activity and psychological wellbeing with anti-hypertensives, thyroxine and anti-coagulants. Is the disease-free interval actually free of the disease or it just subsided with immunosuppressants to become active after some time?

La fibrosis retroperitoneal idiopática es una enfermedad fibroinflamatoria rara, de etiología variada que generalmente se origina alrededor de la aorta y se propaga caudalmente a lo largo de los vasos ilíacos en retroperitoneo adyacente causando obstrucción ureteral como la complicación más frecuente.Reportamos el caso de un paciente varón de 53 años que se presentó con un dolor leve en ambas piernas. Al investigar más a fondo, descubrimos que había estado luchando con recaídas intermitentes cada 3-4 años durante los últimos 20 años desde que se le diagnosticó por primera vez fibrosis retroperitoneal idiopática. Tenía 33 años cuando desarrolló por primera vez los síntomas de anuria durante 48 horas y se le diagnosticó fibrosis retroperitoneal idiopática. Esto fue seguido por atrofia del riñón izquierdo e hipertensión 6 años después, luego hipotiroidismo después de otros 3 años y finalmente afectación de la vena cava inferior y trombosis venosa profunda aguda de las extremidades inferiores después de otros 3-4 años. Su trombosis venosa profunda se controló bien a tiempo. Le recetaron glucocorticoides cada vez que tenía una recaída y una complicación.Hicimos una revisión de la literatura para comprender los avances recientes sobre su patogenia, diagnóstico, investigaciones y manejo. Se realizaron búsquedas en PubMed utilizando términos como fibrosis retroperitoneal sola y en combinación con términos relacionados como trombosis de la vena cava inferior, trombosis venosa profunda, tamoxifeno, metotrexato. Este caso es único, ya que es muy raro encontrar trombosis venosa profunda aguda en fibrosis retroperitoneal idiopática sin desarrollo de colaterales cuando la luz de la vena cava inferior está comprometida hasta una obstrucción casi completa.Después de un seguimiento de 20 años, el paciente se encuentra bien en términos de actividad física y bienestar psicológico con antihipertensivos, tiroxina y anticoagulantes. ¿El intervalo libre de enfermedad está realmente libre de la enfermedad o simplemente disminuyó con inmunosupresores para activarse después de algún tiempo?

Tumor retroperitoneal en el espectro de enfermedades asociadas a igg4 / Retroperitoneal tumor in the clinical spectrum of igg4 related disease

Presentamos el caso de una mujer de 55 años sin antecedentes, que acude por dolor abdominal, se realiza una tomografía abdominopélvica evidenciando un tumor retroperitoneal de aspecto quístico en la periferia de la aorta abdominal, que afecta el riñón izquierdo. Se le realizó una laparotomía exploratoria, nefrectomía izquierda, tumorectomía retroperitoneal y una resección de un fragmento de la aorta abdominal con reporte histopatológico, corroborado por una prueba inmunohistoquímica de fibrosis retroperitoneal y aortitis por IgG4. El espectro de enfermedades relacionadas con IgG4 es de origen inmunológico con la capacidad de involucrar casi cualquier órgano. La epidemiología no es precisa, aunque se observa mayor predilección por la raza asiática, siendo más común durante la séptima década de vida. El diagnóstico radica en la coexistencia de varios parámetros clínicos, laboratoriales e histopatológicos, sin ser ninguno de estos patognomónicos. El objetivo del presente artículo es exponer el reporte de un caso con manifestación inusual de fibrosis retroperitoneal y dar a conocer que la importancia del diagnóstico radica en la decisión terapéutica, ya que cursa con buena respuesta al tratamiento inmunosupresor.

We present the case of a 55-year-old woman, with noclinical pathological history, who presented with abdominal pain, abdominopelvic tomography showing retroperitoneal tumoral in the periphery of the abdominal aorta, which affects the left kidney producing hydronephrosis. Exploratory laparotomy, left radical nephrectomy, retroperitoneal tumorectomy and resection of a fragment of abdominal aorta were performed, with histopathological report, corroborated by immunohistochemistry of retroperitoneal fibrosis and IgG4 aortitis. The spectrum of diseases related to IgG4, are of immunological origin that can involve almost any organ. The epidemiology is not precise, although a greater predilection for the Asian race is observed, being more common in the seventh decade of life, it should be suspected in patients with unexplained pain in one or more organs.The diagnosis lies in the coexistence of several clinical, laboratorial and histopathological parameters, without being any of these pathognomonic. We present a case with unusual presentation of retroperitoneal fibrosis and the importance of the diagnosis lies in the therapeutic decision, since it responds well to immunosuppressive treatment, without requiring surgical management.

Fibrosis retroperitoneal como causa de uropatía obstructiva. Reporte de caso / Retroperitoneal fibrosis as a cause of obstructive uropathy. Case report

Resumen La fibrosis retroperitoneal es una patología rara, en la mayoría de los casos idiopática, aunque se ha asociado a medicamentos, neoplasias y otras enfermedades de tejido conectivo. Histopatológicamente se evidencia inflamación y depósito de tejido fibrótico en el retroperitoneo y se caracteriza por cubrir los uréteres provocando lesión renal aguda obstructiva siendo ésta, la manifestación más frecuente; el diagnóstico definitivo se obtiene únicamente con biopsia y la base del tratamiento es la corticoterapia, aunque en casos severos y en resistencia a los corticoides se han usado otras terapias como los inmunomuladores. En ocasiones son necesarias las intervenciones quirúrgicas para el manejo de las complicaciones. Se presenta el caso de un hombre de 50 años que ingresó al servicio de urgencias del Hospital San José por dolor abdominal, los paraclínicos demostraron elevación de los azoados y en las imágenes diagnósticas hidronefrosis izquierda con componente de tejido blando interaortocava y periaórtico, se realizó biopsia retroperitoneal y se hizo diagnóstico de fibrosis retroperitoneal idiopática, se instauraron nefrostomías bilaterales y se inició manejo con corticoide.

Abstract Retroperitoneal fibrosis is a rare, in most cases idiopathic, pathology, although it has been associated with medications, neoplasms and other connective tissue diseases. In terms of histopathology, inflammation and deposits of fibrotic tissue in the retroperitoneum are observed and, characteristically, this covers the urethra, provoking acute obstructive kidney damage, the most frequent manifestation of the disease. The definitive diagnosis is obtained solely via biopsy, and the basis of treatment is corticotherapy, although in severe cases, and where resistance to corticosteroids exists, other treatments have been used, such as immunomodulators. Occasionally, surgical interventions are necessary to manage complications. The case of a 50-year-old man who came to the Hospital emergency service due to abdominal pain is presented. Paraclinical studies showed azotemia, and diagnostic images showed left hydronephrosis with a component of interaortocaval and periaortic soft tissue. A retroperitoneal biopsy was conducted, and a diagnosis of idiopathic retroperitoneal fibrosis was made. Bilateral nephrostomies were put in place and treatment with corticosteroids was initiated.

Antecedentes históricos de la enfermedad relacionada con IgG4 / Historical history of IgG4­related disease

El concepto de enfermedad relacionada a IgG4 se introdujo en la literatura médica hace menos de dos décadas. Este surgió a raíz del registro creciente de manifestaciones extrapancreáticas asociadas a la pancreatitis esclerosante linfoplasmocitaria y a la detección concomitante de niveles séricos aumentados de IgG4 en estos pacientes. Por siglos, muchas manifestaciones de esta enfermedad fueron designadas por epónimos y consideradas idiopáticas. Ya hace más de setenta años, Norman Barrett unió dentro de un mismo grupo patológico algunas de las distintas manifestaciones de esta enfermedad al observar las similitudes macro­ y microscópicas en ellas, aunque no se pudo corroborar una misma etiopatogenia hasta comienzos de este siglo. Se revisó aquí la evolución histórica de esta entidad. (AU)

Imágenes en Medicina: fibrosis peritoneal / Medical images: retroperitoneal fibrosis

Se reporta el caso de un paciente varón de 36 años con antecedente de litiasis renal que visita al urólogo por lumbalgia crónica y astenia. Se solicita laboratorio que arroja aumento de reactantes de fase aguda y leucocitosis. El diagnóstico por imagen comprende tomografía (TC) de abdomen y pelvis con contraste endovenoso (EV), TC tórax, ecografía testicular con doppler color y Resonancia Magnética de abdomen y pelvis con gadolinio EV. El servicio de diagnóstico por imágenes plantea la posibilidad de fibrosis retroperitoneal como primer diagnóstico diferencial. Se solicita biopsia de la lesiónpara su confirmación, que demuestra tejido fibrótico, confirmando el diagnóstico de fibrosis retroperitoneal, en este caso idiopática

The case of a 36-year-old male patient with a history of renal lithiasis who visits the urologist for chronic low back pain and asthenia is reported. Laboratory is requested to show an increase in acute phase reactants and leukocytosis. The imaging diagnosis includes tomography (CT) of the abdomen and pelvis with intravenous contrast, chest CT, testicular ultrasound with color doppler and Magnetic Resonance of the abdomen and pelvis with gadolinium. The diagnostic imaging service raises the possibility of retroperitoneal fibrosis as the first differential diagnosis. Biopsy of the lesion is requested for confirmation, which demonstrates fibrotic tissue, confirming the diagnosis of idiopathic retroperitoneal fibrosis, in this case

Hipofisitis linfoplasmocitaria y fibrosis retroperitoneal asociadas a un síndrome poliglandular autoinmune: enfermedades relacionadas con IgG4. Caso clínico / Hypophysitis and retroperitoneal fibrosis associated with autoimmune polyglandular syndrome and IgG4-related disease: report of one case

We report a 23 year old woman presenting with a nephrotic syndrome due to minimal change disease, central diabetes insipidus, primary hypothyroidism, vitiligo and universal alopecia. Eleven years later, she presented secondary amenorrhea due to hypogonadotropic hypogonadism, with mild hyperprolactinemia and central adrenal insufficiency. A magnetic resonance imaging of the sella turcica showed a pituitary mass with suprasellar extension that was resected using a transsphenoidal approach. Pathology confirmed the presence of a lymphoplasmacytic hypophysitis. She needed a second surgical resection due to mass growth and neuro-ophthalmologic impairment. One year later, systemic lupus erythematosus, arterial hypertension and type 2 diabetes mellitus were diagnosed. Two years later, due to back pain, constipation and renal failure, retroperitoneal fibrosis was found, satisfactorily treated with glucocorticoids and colchicine. Hence, this clinical vignette shows the coexistence of autoimmune polyglandular syndrome with retroperitoneal fibrosis and lymphoplasmacytic hypophysitis. Tissue analysis showed the presence of IgG4 producing plasma cells in the pituitary and retroperitoneum, which constitute a basis for the diagnosis of IgG4 related disease.

A propósito de un caso de lipoma retroperitoneal / Retroperitoneal Lipoma: A case report

Los lipomas retroperitoneales son infrecuentes y suelen representar el 2.9% de los tumores primarios de origen en retroperitoneo1 . Pueden alcanzar gran tamaño y el diagnóstico diferencial debe hacer con el liposarcoma bien diferenciado, aunque es muy difícil de realizar en el preoperatorio2 . Se presenta el caso de un paciente con lipoma retroperitoneal cuyo diagnóstico se realizó por tomografía axial computada (TAC) y que fue extirpado en su totalidad.

Retroperitoneal lipomas are infrequent and can represent a 2.9 % of the primary tumors originated in the retroperitoneum. They can reach a great size and the differential diagnosis must be performed with the well differentiated liposarcoma, even though it is very difficult to establish it during the preoperative time. We describe herein a case of a patient with a retroperitoneal lipoma. The diagnosis was established with a computed axial tomography (CAT) and it was totally surgically removed.

Espectro clínico de la enfermedad relacionada con IgG4 en Colombia / Clinical spectrum of IgG4-related disease in Colombia

RESUMEN La enfermedad relacionada con IgG4 es una condición inflamatoria sistémica, caracterizada por la infiltración de diversos órganos por complejos formados por células plasmáticas IgG4 positivas, asociadas con niveles elevados de IgG4 en el suero. El diagnóstico de esta enfermedad es complejo y los hallazgos clínicos no son patognomónicos. En esta serie de casos, describimos un amplio espectro clínico en 4 pacientes, en quienes, después de descartar otros diagnósticos, se confirmó la enfermedad relacionada con IgG4. A pesar de que esta enfermedad no es común, se debe considerar entre los diagnósticos diferenciales de enfermedades con afectación de múltiples órganos. Este reporte de pacientes con enfermedad relacionada con IgG4, en Colombia, resalta un amplio espectro de presentaciones clínicas, incluyendo estenosis subglótica, pancreatitis autoinmune, fibrosis retroperitoneal y compromiso sistémico.

ABSTRACT IgG4-related disease is an inflammatory systemic condition noted by the infiltration of different organs by IgG4-bearing plasma cells, as well as elevated serum IgG4 levels. Diagnosis of this condition is complex, and clinical findings are not particularly indicative. In this case series, a description is presented on 4 patients with a wide spectrum of clinical manifestations, in whom, after ruling out different options, a diagnosis of IgG4 related disease was confirmed. Despite this disease not being common, it should be considered among the options whenever multiple organs are affected. This report of patients with IgG4-related disease in Colombia highlights a wide spectrum of clinical presentations, including subglottic stenosis, autoimmune pancreatitis, retroperitoneal fibrosis, and systemic compromise.

A case of retroperitoneal fibrosis responding to steroid therapy

ABSTRACT A 69-year-old man presented at the hospital with complaints of prolonged stomach pain extending from the week prior. Enhanced computed tomography (CT) revealed a low density area in the retroperitoneal space. A radiologist diagnosed the patient with retroperitoneal fibrosis. One week later, an enhanced CT revealed an exponential increase of the low density area and slight right hydronephrosis. Upon admission, prednisolone administration was initiated at a dose of 40mg/day. The size of the retroperitoneal soft tissue mass decreased gradually. Although the dose of prednisolone was tapered to 5mg, the patient is doing well without any sign of recurrence.

Uropatía obstructiva recurrente en arteritis de Takayasu / Recurrent upper urinary tract obstruction in takayasu arteritis

La arteritis de Takayasu (AT) es una enfermedad inflamatoria infrecuente de vasos grandes. A menudo, el daño crónico originado en las grandes arterias requiere de revascularización mediante prótesis vasculares. Excepcionalmente la implantación de las prótesis origina lesiones en otros órganos, como los uréteres, los cuales pueden obstruirse por compresión extrínseca por el tejido fibroso retroperitoneal originado como reacción a la presencia de la prótesis vascular.Se comunica el caso de una mujer que presentó hidronefrosis bilateral en forma asincrónica después de los 2 años de la instalación de una prótesis aorto-ilíaca con insuficiencia renal aguda, la cual pudo revertirse mediante. (AU)

Takayasu's arteritis is a rare inflammatory disease of large vessels. Often, chronic damage resulting in large arteries stenosis requires revascularization with vascular prostheses. Exceptionally, the presence of prostheses causes lesions in other organs, such as ureters, which are subject to extrinsic compression caused by retroperitoneal fibrous tissue originated as a reaction to the presence of vascular prostheses. We report the case of a woman who presented bilateral asynchronous hydronephrosis after 2 years of the installation of an aorto- iliac graft with acute renal failure, which could be reversed by releasing the ureter from the periprosthetic fibrous retroperitoneal tissue.(AU)

Retroperitoneal fibrosis: case series of five patients and review of the literature / Fibrose retroperitoneal: série de cinco casos e revisão da literatura

ABSTRACT Chronic periaortitis (CP) is an umbrella term used to describe a group of nosologically allied conditions that include idiopathic retroperitoneal fibrosis (Ormond's disease), inflammatory abdominal aortic aneurysm, and perianeurysmal retroperitoneal fibrosis. Retroperitoneal fibrosis encompasses a range of diseases characterized by the presence of a fibro-inflammatory tissue, which usually surrounds the abdominal aorta and the iliac arteries and extends into the retroperitoneum to envelop neighboring structures-ureters. Retroperitoneal fibrosis is generally idiopathic, but can also be secondary to the use of certain drugs, malignant diseases, infections, and surgery. Here we describe a 5 years follow up (2006-2011) of 5 patients admitted to our hospital with symptoms, laboratory, imaging and pathologic finding compatible with retroperitoneal fibrosis. We review our clinical course of our patient with respect to the literature.

RESUMO Periaortite crônica (PC) é um termo genérico usado para descrever um grupo de condições nosologicamente ligadas que incluem a fibrose idiopática retroperitoneal (doença de Ormond), o aneurisma da aorta abdominal inflamatório e a fibrose retroperitoneal perianeurismática. O termo fibrose retroperitoneal engloba uma gama de doenças que se caracterizam pela presença de um tecido fibroinflamatório que geralmente envolve a aorta abdominal e as artérias ilíacas, se estende ao retroperitôneo e envolve estruturas ureterais vizinhas. A fibrose retroperitoneal geralmente é idiopática, mas pode também ser secundária ao uso de determinados fármacos, doenças malignas, infecções e cirurgia. Este estudo descreve o seguimento por cinco anos (2006-2011) de cinco pacientes internados em nosso hospital que apresentavam sintomas e achados laboratoriais, de imagem e patológicos compatíveis com a fibrose retroperitoneal. Revisou-se a evolução clínica dos pacientes, que foi comparada com os achados da literatura.

Fibrosis retroperitoneal idiopática / Idiopathic retroperitoneal fibrosis

Mujer de 36 años de edad quien consulta por cuadro clínico de ocho meses de evolución consistente en dolor abdominal, asociado a diarrea y emesis, siendo manejada inicialmente como cuadro de colon irritable. Ante la persistencia del dolor solicitan estudios imagenológicos con hallazgos sugestivos de masa retroperitoneal por lo cual remiten para valoración por cirugía. Al examen físico se encuentra dolor a la palpación abdominal generalizada sin adenopatías o masas. Paraclínicos con resonancia abdominal evidenciando masa sólida retroperitoneal de localización paraaórtica izquierda y espacio interaortocavo, englobando de forma circunferencial la aorta abdominal a la emergencia de arterias renales comprometiendo tejidos adyacentes, con disminución del tamaño del riñón izquierdo, compensando con hipertrofia del riñón derecho. Marcadores tumorales negativos, colonoscopia sin evidencia de masas. Es llevada a biopsia guiada por TAC con patología conclusiva de fibrosis retroperitoneal idiopática. Conclusión: se presenta un caso de fibrosis retrooperitoneal idiopática, enfermedad de muy bajafrecuencia de presentación en la práctica clínica diaria. (Acta Med Colomb 2015: 40: 260-262).

The case of a 36-year-old woman who consults for history of abdominal pain associated to diarrhea and emesis of eight months of evolution, being initially handled as irritable bowel syndrome, is presented. Given the persistence of pain, imaging studies suggestive of retroperitoneal mass were requested and were referred for assessment by surgery. Physical examination showed generalized pain at abdominal palpation without lymphadenopathy or masses. Abdominal resonance showed solid retroperitoneal mass of left para-aortic location and interaortocaval space, circumferentially encompassing the abdominal aorta at the emergence of renal arteries engaging adjacent tissue with decreased size of the left kidney, compensated by right kidney hyperthrophy. Tumor markers were negative, and colonoscopy did not show evidence of masses. A carried CT-guided biopsy was performed with conclusive pathology of idiopathic retroperitoneal fibrosis. Conclusion: a case of idiopathic retroperitoneal fibrosis, disease with very low frequency in everyday clinical practice, is presented. (Acta Med Colomb 2015: 40: 260-262).

Fibrosis retroperitoneal asociada a neoplasias malignas: Serie de casos clínicos / Retroperitoneal fibrosis associated with malignancies: Report of eight cases

Retroperitoneal fibrosis (RPF) is uncommon. Eight percent of cases are associated with malignancies. The pathogenesis of cancer related RPF (MRPF) is unknown. It may be originated from a desmoplastic reaction of the retroperitoneum to the presence of malignant cells, to the action of cytokines secreted by the tumor or a reactive inflammation. MRPF may also be a consequence of antineoplastic therapy (surgery, radiotherapy and chemotherapy). We report eight cases of MRPF associated with breast, cervix, thyroid, kidney and retroperitoneal cancer. In four patients, retroperitoneal tissue biopsy was performed, showing the typical findings of idiopathic RPF. In 6 patients MRPF appeared one to 15 years after cancer diagnosis and in two, it appeared simultaneously. The clinical presentation, course and therapeutic response are described. Six patients were treated with steroids alone and five with steroids associated with colchicine.

Fibrosis retroperitoneal asociada a una hiperostosis corticalgeneralizada: presentación de un caso / Retroperitoneal fibrosis associated with a generalized cortical hyperostosis: presentation of a case

La fibrosis retroperitoneal es una entidad clínica, de presentación poco frecuente,caracterizada por un trastorno fibroso e inflamatorio crónico, que rodea estructurasabdominales, principalmente los aspectos peri vasculares de las mismas, y que esexplicada por diferentes etiologías, siendo las más importantes la fibrosis retroperitonealidiopática y la fibrosis secundaria a trastornos relacionados con la subclase de IgG4. Por suparte, la hiperostosis cortical generalizada pertenece a un grupo de enfermedades raras depatologías metabólicas óseas osteocondensantes. En este caso, se presenta una pacientede 45 años de edad con fibrosis retroperitoneal, de etiología no establecida, asociada ahiperostosis cortical generalizada. La asociación de estas dos patologías, no reportadaanteriormente en la literatura, parece ser una relación espuria, sin embargo, existenalgunos mecanismos patogénicos entrelazados.

Retroperitoneal fibrosis is a rare clinical condition, characterized by a fibro-inflammatory disorder that surrounds abdominal structures, principally at perivascular level, and that is explained by different etiologies like idiopathic fibrosis and IgG4 related disorders. Generalized cortical hyperostosis belongs to a group of rare metabolic bone condensation diseases.The case is presented of a 45 year old female patient with a retroperitoneal fibrosis of unknown origin associated with generalized cortical hyperostosis. The association of these two conditions has not previously reported in the literature. This appears to be a spurious relationship, although some pathogenic mechanisms are intertwined.

Fibrosis retroperitoneal: presentación de un caso y revisión del tema / Retroperitoneal fibrosis: case report and literature review

Introducción: la primera descripción de obstrucción ureteral extrínseca por un proceso de fibrosis retroperitoneal se efectuó en 1905. En poco más de un siglo sólo se han reportado unos 800 casos de esta patología. Caso clínico: se reporta el caso de un paciente femenino de 55 años, que cursa con dolor abdominal difuso de larga evolución, acompañado de náusea, vómito, fiebre de 38°C e hipertensión de 160/100 mmHg. Se realiza tomografía axial computarizada observándose masa retroperitoneal que produce obstrucción de uréteres. Se realiza laparotomía exploradora con toma de biopsia y liberación de uréteres. El análisis histopatológico demuestra la presencia de fibrosis retroperitoneal, una entidad patológica poco frecuente, cuyo diagnóstico requiere un alto índice de sospecha en base a los hallazgos clínicos, de laboratorio y de gabinete. Revisión de la literatura: no se han formulado definiciones claras de los diferentes trastornos que se incluyen en el espectro de la fibrosis retroperitoneal, debido a lo infrecuente de esta enfermedad. Por esta razón hoy se carece de criterios diagnósticos y de una clasificación coherente de las diferentes formas que puede adoptar la enfermedad. Sin embargo, ante la sospecha de una fibrosis retroperitoneal se debe distinguir entre una forma idiopática y una secundaria, por las diferentes implicaciones para el tratamiento. Conclusión: la fibrosis retroperitoneal es un diagnóstico diferencial ante la presencia de dolor abdominal difuso asociado a síntomas de compresión ureteral o de grandes vasos.

Introduction. The first description of extrinsic uretheral obstruction by retroperitoneal fibrosis occurred in 1905. In little more than a century, about 800 cases of this disease have been reported. Case description. We report the case of a female 55 year-old patient who presents with diffuse abdominal pain of long duration, nausea, vomiting, fever of 38°C and hypertension of 160/100 mmHg. A CT scan is performed that shows a retroperitoneal mass that obstructs the urethers. Exploratory laparotomy was performed, urethers were released and biopsy was taken. Pathology analysis showed the presence of retroperitoneal fibrosis, a rare pathological entity whose diagnosis requires a high index of suspicion based on clinical, imaging and laboratory workup. Literature review. There are no clear definitions of the variety of disorders that are included in the spectrum of retroperitoneal fibrosis, due to the rarity of this condition. Consequently, we lack diagnostic criteria and a consistent classification of the different forms that it may adopt. However, when there is suspicion of retroperitoneal fibrosis, the first step is to establish whether it is idiopathic or secondary, as there will be treatment implications. Conclusion. Retroperitoneal fibrosis should be considered in the differential diagnosis whenever diffuse abdominal pain is associated with uretheral or great vessels compression.

Metisergida como profilaxia de migrânea e cefaleia em salvas, e a possível ocorrência de fibrose retroperitoneal: relato de casos / Methysergide as prophylaxis of migraine and cluster headache, and the possible occurrence of retroperitoneal fibrosis: a case report

JUSTIFICATIVA E OBJETIVOS: Metisergida é fármaco de eficácia comprovada na profilaxia tanto da migrânea quanto da cefaleia em salvas, embora possa predispor a fibrose (< 1%). O objetivo deste estudo foi relatar dois casos de cefaleia primária de difícil controle, satisfatoriamente conduzidos com metisergida, que precisou ser interrompida por suspeita de fibrose retroperitoneal (FR). RELATO DOS CASOS: A metisergida foi utilizada, com sucesso, como profilático de migrânea e cefaleia em salvas em paciente de 69 anos, sexo feminino, e de 58 anos, sexo masculino, respectivamente, ambos refratários aos fármacos de primeira e segunda linha. Após 24 meses, no primeiro caso e 30 meses, no segundo, de uso contínuo de metisergida, foram observados sinais e sintomas sugestivos de FR como edema assimétrico de membros inferiores, sem dor, na paciente com migrânea; dor abdominal, disfunção sexual e edema de membros inferiores, no paciente com cefaleia em salvas. Apesar do rastreio inicial negativo para fibrose retroperitoneal feito com ultrassonografia e tomografia computadorizada de abdômen normais, no segundo, como os sinais e sintomas estavam progressivos, optou-se pela suspensão e redução, respectivamente, da metisergida. Houve resolução completa do quadro cerca de uma semana após essa conduta. CONCLUSÃO: A metisergida é boa opção nos casos refratários, mas deve ser utilizada com cautela. Estratégia de descontinuidade do fármaco a cada seis meses, por cerca de 4 a 8 semanas, reduz o risco de ocorrência de FR, assim como observação clínica de sinais e sintomas que sugiram esse efeito colateral.

BACKGROUND AND OBJECTIVES: Methysergide is a drug with proven efficacy to prevent both migraine and cluster headache, although it may predispose to fibrosis (< 1%). This study aimed at reporting two cases of primary and difficult to control headache, satisfactorily treated with methysergide, which had to be withdrawn due to suspicion of retroperitoneal fibrosis (RF). CASE REPORTS: Methysergide was successfully used to prevent migraine and cluster headache in a 69-year old male and in a 58-year old female, respectively, both refractory to first and second line drugs. After 24 months for the first case, and 30 months for the second case, of continuous methysergide, signs and symptoms suggesting RF were observed, such as asymmetric painless lower limbs edema in the migraine patient, and abdominal pain, sexual dysfunction and lower limbs edema in the cluster headache patient. In spite of the early negative screening for retroperitoneal edema made with normal abdominal ultrasound and CT, in the second, since signs and symptoms were progressing, we decided for methysergide withdrawal and decrease, respectively. There has been total resolution of symptoms approximately one week after such approach. CONCLUSION: Methysergide is a good option for refractory cases, but should be used with caution. Withdrawing the drug every six months for approximately 4 to 8 weeks decreases the incidence of RF, in addition to clinical observation of signs and symptoms suggesting this side-effect.

Fibrosis retroperitoneal asociada a uso crónico de ergotamina. Tratamiento con colchicina y esteroides: Caso clínico / Retroperitoneal fibrosis associated with chronic use of ergotamine: Report of one case

Retroperitoneal fibrosis (RPF) associated with chronic use of ergotamine is a very rare disorder. We report a 45-year-old woman who presented with a RPf after using, almost daily for 23 years, ergotamine tartrate for migraine relief. FRP presented as a chronicinflammatory state, anemia, abdominal and lumbosacral pain and a hypogastric mass. A CT-Scan showed a periaortic mass and left hydronephrosis. A percutaneous biopsy was obtained and the patient was subjected to a surgical ureterolysis and tissue resection. The biopsy confirmed the presence of RPf. Due to persistent symptoms and increase in the volume of periaortic tissue, treatment with colchicine 1 mg/day and defazacort 30 mg/day was started, resulting in a rapid di-sappearance of symptoms, disappearance ofinflammation and a significant reduction in the volume of the periaortic tissue. The patient remains in complete remission after 29 months of follow up.