ABSTRACT
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?
Subject(s)
Humans , Male , Middle Aged , Retroperitoneal Fibrosis/complications , Retroperitoneal Fibrosis/diagnosis , Retroperitoneal Fibrosis/therapy , Recurrence , Time Factors , Tomography, X-Ray Computed , Diagnosis, Differential , Hypothyroidism , Immunosuppressive Agents/therapeutic useABSTRACT
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.
Subject(s)
Humans , Retroperitoneal Fibrosis/surgery , Retroperitoneal Fibrosis/diagnosis , Aortic Aneurysm, Abdominal/diagnosis , Aorta, Abdominal , Retroperitoneal Fibrosis , Retroperitoneal Fibrosis/pathology , Aortic Aneurysm, Abdominal/surgery , Aortic Aneurysm, Abdominal/pathologyABSTRACT
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.
Subject(s)
Aged , Female , Humans , Male , Middle Aged , Neoplasms/complications , Retroperitoneal Fibrosis/etiology , Fatal Outcome , Retroperitoneal Fibrosis/diagnosis , Tomography, X-Ray ComputedABSTRACT
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.
Subject(s)
Middle Aged , Retroperitoneal Fibrosis/surgery , Retroperitoneal Fibrosis/diagnosis , Diagnosis, Differential , Abdominal Pain/etiology , Retroperitoneal Fibrosis/complications , Ureteral Obstruction/etiology , Photomicrography , Tomography, X-Ray ComputedABSTRACT
Retroperitoneal fibrosis (RPF) is a rare disease with unclear etiology, which is characterized by chronic non-specific inflammation of the retroperitoneum. This study was performed to investigate the clinical characteristics, laboratory findings, radiologic findings, treatment and outcome in Korean patients with RPF. We retrospectively reviewed medical records of 27 RPF patients who were admitted to Yonsei University Medical Center between 1998 and 2009. Twenty-two patients (81%) were male. The mean age at diagnosis was 56 yr. Nine patients had identifiable risk factors of RPF and three patients had combined autoimmune diseases. Acute phase reactants were elevated in most patients. Rheumatoid factor was positive in 3 of 16 patients (19%) and antinuclear antibody in 4 of 17 (24%). Five of 6 patients who were taken positron-emission tomography showed positive uptake. Glucocorticoids were used in 16 patients (59%) and four of them received combination therapy with azathioprine. After immunosuppressive treatment, the levels of acute phase reactants dropped, and the size of mass also decreased in most patients. In conclusion, the clinical characteristics of RPF in Korean patients are similar with other series except for higher proportion of male. Some patients with RPF have autoimmune features. The effect of immunosuppressive treatment on RPF is good.
Subject(s)
Adult , Aged , Female , Humans , Male , Middle Aged , Acute Disease , Antibodies, Antinuclear/immunology , Azathioprine/therapeutic use , Drug Therapy, Combination , Glucocorticoids/therapeutic use , Immunosuppressive Agents/therapeutic use , Positron-Emission Tomography , Republic of Korea , Retroperitoneal Fibrosis/diagnosis , Retrospective Studies , Sex Factors , Tomography, X-Ray ComputedABSTRACT
We present a 13 year old girl from Assam who had been treated as abdominal tuberculosis for 2 years due to the presence of refractory lymphocyte-predominant ascites and multiple small bowel strictures associated with significant anorexia and weight loss. On evaluation she was found to have retroperitoneal fibrosis with hydroureteronephrosis, mediastinal fibrosis and a retro-orbital pseudotumour. Based on these findings the diagnosis of Multifocal Idiopathic Fibrosclerosis (MIFS) was made. Ascites and multiple bowel strictures have been only rarely been described in association with MIFS. The other unique features in this patient were the early age of presentation, the presence of mediastinal fibrosis in association with retroperitoneal fibrosis, extensive soft tissue fibrosis of the neck, axillae and the presence of trismus. In a country like ours where Tuberculosis is commonplace, one would not think twice about treating such a case with antituberculous therapy. However, with a constellation of findings suggestive of a diffuse fibrotic process, MIFS should be an important consideration.©
Subject(s)
Abdomen , Adolescent , Anti-Inflammatory Agents/therapeutic use , Diagnosis, Differential , Female , Fibrosis/diagnosis , Humans , Orbital Pseudotumor/diagnosis , Peritonitis, Tuberculous/diagnosis , Peritonitis, Tuberculous/drug therapy , Peritonitis, Tuberculous/pathology , Prednisolone/therapeutic use , Retroperitoneal Fibrosis/diagnosis , Retroperitoneal Fibrosis/pathology , Sclerosis/diagnosisABSTRACT
La enfermedad de Ormond's o fibrosis retroperitoneal, es una afección rara de etiología incierta, caracterizada por reemplazo de tejido normal del retroperitoneo central por proliferación de tejido fibroso. Se localiza habitualmente en la zona entre la arteria y el sacro. Puede envolver la aorta, cava inferior, uréteres y otros órganos intraabdominales. Puede ser idiopática (2/3 de los casos) o secundaria (fármacos, neoplasias, traumas, radioterapia). Se describe su asociación a enfermedades autoinmunes.
Subject(s)
Female , Middle Aged , Humans , Retroperitoneal Fibrosis/diagnosis , Retroperitoneal Fibrosis/drug therapy , Adrenal Cortex Hormones/therapeutic use , Abdominal Pain/etiology , Low Back Pain/etiology , Retroperitoneal Fibrosis/complications , Treatment OutcomeABSTRACT
Relata-se o caso de um paciente com ureter retrocava, diagnosticado inicialmente em exame de urografia excretora e confirmado com tomografia computadorizada do abdome. A tomografia computadorizada torna-se necessária não apenas para confirmar a posição do ureter em relação à veiacava inferior, como também para excluir outras doenças que cursam com hidronefrose, pois hidronefrose é o principal achado do ureter retrocava sintomático.
The authors report a case of a patient with retrocaval ureter found out in an excretory urography and confirmed with abdominal computerized tomography. The computerized tomography is necessary to confirm the position of the ureter and to exclude other diseaseswhich are associated with hydronefrosis, the main imaging finding,when there is a symptomatic retrocaval ureter.
Subject(s)
Humans , Male , Adult , Ureteral Diseases/diagnosis , Tomography, X-Ray Computed , Urography , Vena Cava, Inferior/pathology , Diagnosis, Differential , Retroperitoneal Space/pathology , Retroperitoneal Fibrosis/diagnosis , Hydronephrosis/diagnosis , Ureteral Obstruction/diagnosisABSTRACT
Retroperitoneal fibrosis is one of the rare fibrotic processes mainly involving caudal aspect of the retroperitoneum. In up to 70% of patients no causative factor for retroperitoneal fibrosis can be identified, where it resembles auto-immune reaction. A 30-year-old lady presented with progressive oedema of the lower extremities, jaundice and abdominal swelling. Clinical examination revealed doughy abdominal feel and diffuse tenderness. Ultrasonography showed dilated intrahepatic bilary radicles and mild ascites. Contrast CT-scan showed enhancing sheath of soft tissue extending from porta down to aortic bifurcation encasing common bile duct, aorta, inferior vena cava and the ureters. CT-guided fine needle aspiration cytology revealed metastatic adenocarcinoma. Ascitic fluid did not show any malignant cell. No abdominal mass could be detected. The patient died during the course of palliative chemotherapy.
Subject(s)
Adenocarcinoma/pathology , Adult , Fatal Outcome , Female , Humans , Retroperitoneal Fibrosis/diagnosis , Retroperitoneal Neoplasms/pathology , Retroperitoneal Space/pathology , Tomography, X-Ray ComputedABSTRACT
La fibrosis retroperitoneal constituye una afección infrecuente y de causa generalmente no identificable, sin embargo, se ha descrito su asociación con algunas drogas, enfermedades del tejido conectivo y patología neoplásica. Se caracteriza por el depósito de tejido fibroso en el retroperitoneo y manifestaciones secundarias a la compresión ureteral o de estructuras vasculares. Las técnicas de imágenes (principalmente la tomografía axial computarizada) resultan importantes en su sospecha y diagnóstico diferencial, con utilidad en la exclusión de una enfermedad tumoral subyacente. La biopsia abierta se considera el gold standar para establecer el diagnóstico, cobrando especial relevancia en los casos sin una causa evidente. El enfrentamiento terapéutico tradicional se basa en la cirugía (ureterolisis) y suspensión de drogas potencialmente injuriantes, pero diferentes terapias médicas han sido planteadas, incluyendo el uso de corticoides, inmunosupresores y tamoxifeno, ya sea en forma exclusiva o asociados a técnicas intervencionistas o quirúrgicas propiamente tales.
Subject(s)
Humans , Male , Middle Aged , Retroperitoneal Fibrosis/diagnosis , Retroperitoneal Fibrosis/etiology , Retroperitoneal Fibrosis/therapy , Adrenergic beta-Antagonists , Adrenal Cortex Hormones/therapeutic use , Diagnosis, Differential , Abdominal Pain/etiology , Immunosuppression Therapy , Ureteral Obstruction/surgery , Ureteral Obstruction/etiology , Signs and Symptoms , Tamoxifen/therapeutic useABSTRACT
La fibrosis retroperitoneal idiopática es una entidad patológica histológicamente benigna aunque de curso habitualmente maligno, que se presenta en alrededor 1/200.000 habitantes, con predomino en hombres, con edad media de 50 a 60 años, cuya etiología sería inmunológica. Compromete preferentemente tejidos ubicados por debajo de la bifurcación aórtica y tejidos vecinos, siendo lo más frecuente el compromiso del o ambos uréteres. Su sistomatología es inespecífica y su diagnóstico es imagenológico e histopatológico. Su tratamiento es quirúrgico y medicamentoso en base a corticoides e inmunosupresores, aunque también se ha utilizado antiestrógenos como el tamoxifeno. Existe una forma de fibrosis retroperitoneal secundaria en alrededor del 33 por ciento de los pacientes, asociada al uso de algunos medicamentos y que mejora con la suspensión de éstos
Subject(s)
Humans , Ureteral Diseases/diagnosis , Retroperitoneal Fibrosis/diagnosis , Adrenal Cortex Hormones/therapeutic use , Immunosuppressive Agents/therapeutic use , Retroperitoneal Fibrosis/chemically induced , Retroperitoneal Fibrosis/drug therapy , Retroperitoneal Fibrosis/etiology , Tamoxifen/therapeutic useABSTRACT
Se presenta un caso de fibrosis retroperitoneal benigna idiopática (FRB) de localización exclusivamente pelviana, estudiada mediante radiología convencional, ecografía y tomografía axial computada, llegándose al diagnóstico definitivo por biopsia quirúrgica debido a que el aspecto en imágenes fue de "pelvis congelada". La enfermedad remitió completamente con tratamiento médico con progesterona, sin recidiva del cuadro en un año de seguimiento. Se plantean las posibles etiologías y diagnósticos diferenciales (principalmente con la patología tumoral pelviana), de esta forma de presentación inusual
Subject(s)
Humans , Female , Middle Aged , Pelvis , Progesterone/therapeutic use , Retroperitoneal Fibrosis/diagnosis , Fibroblasts/drug effects , Hydronephrosis/drug therapy , Hydronephrosis/etiology , Pelvic Neoplasms/diagnosis , Pelvic Neoplasms/etiology , Pelvis , Pelvis/pathology , Progesterone/therapeutic use , Retroperitoneal Fibrosis/complications , Retroperitoneal Fibrosis/drug therapy , Tomography, X-Ray ComputedABSTRACT
La fibrosis retroperitoneal es una enfermedad poco común descrita por primera vez en 1905; desde entonces más de 500 casos han sido reportados en la literatura internacional. Esta enfermedad tiene una gran variedad de causas y formas de presentación donde el proceso fibrótico casi siempre produce obstrucción ureteral. El diagnóstico siempre debe ser confirmado por el estudio histológico y el objetivo del tratamiento es el conservar la función renal. Reportamos un singular caso de fibrosis retroperitoneal en una paciente que acudió a la consulta de Ginecología de la Maternidad Concepción Palacios
Subject(s)
Humans , Female , Pelvis , Retroperitoneal Fibrosis/diagnosis , Retroperitoneal Fibrosis/etiology , Retroperitoneal Fibrosis/therapy , Ultrasonics , Abdomen , Histological TechniquesABSTRACT
Se describe el caso de un paciente de sexo masculino, 44 años con una fibrosis retroperitoneal idiopática derecha. El paciente se presentó con lumbalgia de 10 años evolución, cólico renal derecho reciente y masa presacra palpable por tacto. La UIV mostró hidronefrosis derecha. Se practicó ureterolisis, reimplantante de uréter derecho y bypass de arteria ilíaca común a ilíaca externa. El paciente se mantiene sin tratamiento anticoagulante y al año, la UIV es normal
Subject(s)
Adult , Humans , Male , Retroperitoneal Fibrosis/diagnosis , Retroperitoneal Fibrosis/etiology , Retroperitoneal Fibrosis/surgery , UltrasonographyABSTRACT
Os autores relatam um caso de fibrose retroperitoneal diagnosticada por laparotomia exploradora a qual havia sido indicada por uma hipótese diagnóstica de aneurisma de aorta fissurado. Dada a raridade da doença, fazem o presente relato e revisam a literatura
Subject(s)
Humans , Adult , Male , Aortic Aneurysm/surgery , Aorta, Abdominal/surgery , Retroperitoneal Fibrosis/diagnosis , Aortic Rupture , Laparotomy , Retroperitoneal Fibrosis/pathology , Retroperitoneal Fibrosis/surgery , UltrasonographyABSTRACT
Fibrose retroperitoneal idiopática é um processo inflamatório inespecífico localizado no retroperitônio entre L4 e L5, cuja etiopatogenia é desconhecida. Sua sintomatologia é incaracterística, com mal-estar, dor abdominal ou lombar e desconforto gastrintestinal. A morbidade está relacionada à obstruçäo ureteral, que pode levar à destruiçäo do rim, e, caso seja bilateral, à insuficiência renal. Säo apresentados 6 casos desta doença, sendo que em 4 pacientes havia obstruçäo ureteral unilateral e, em 2, bilateral. Todos apresentavam antecedentes de dor abdominal ou lombar. Dois apresentavam-se com edema de membros inferiores e 2 com insuficiência renal. Os dois pacientes em insuficiência renal foram tratados inicialmente com cateterizaçäo ureteral, sendo que em um deles optou-se pela permanência dos mesmos (cateteres duplo J) a longo prazo, com ótimos resultados. Cinco pacientes foram tratados cirurgicamente pela ureterolise. Dois destes apresentaram recorrência contralateral de obstruçäo ureteral, que regrediu com uso de corticóides
Subject(s)
Adult , Middle Aged , Humans , Male , Female , Retroperitoneal Fibrosis/diagnosis , Retroperitoneal Fibrosis/therapy , Ureteral ObstructionABSTRACT
Se presenta un caso de fibrosis retroperitoneal con obstrucción ureteral e infiltración vesical con remisión completa después del tratamiento con progesterona. La exploración abdominal y retroperitoneal, en una segunda laparatomía por un tumor inflamatorio apendicular, permitió constatar la ausencia de fibrosis. Cuatro años después de la última laparotomía, el paciente se encuentra asintomático y con estudios radiológicos y de laboratorio normales