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.

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.

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

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.

Tratamento associado (clínico-cirúrgico) da fibrose retroperitoneal idiopática / Associated treatment (medical and surgical) of idiopathic retroperitoneal fibrosis

Os autores descrevem caso de paciente com fibrose retroperitoneal idiopática e revisão da patologia. Esta rara doença de etiologia incerta caracteriza-se por inflamação crônica do retroperitôneo, ocasionalmente aprisionando e obstruindo estruturas, notavelmente os ureteres. Trata-se de um paciente do sexo masculino, 45 anos, com dor em flanco esquerdo irradiada para testículo, em cólica, com piora progressiva. Feito o diagnóstico de fibrose retroperitoneal com compressão ureteral à esquerda (tomografia computadorizada do abdome), o paciente foi submetido a desobstrução ureteral com colocação de cateter duplo “J” e, posteriormente, a tratamento clínico com prednisona. Não houve regressão da massa fibrótica, mas melhora clínica quanto à dor e à ausência de novo episódio de obstrução ureteral. Os autores ressaltam a necessidade de suspeição da patologia descrita, mesmo sendo rara, pela possibilidade de complicações graves, se seu diagnóstico não for realizado e o tratamento não for instituído no tempo ideal

A case report of a patient with idiopathic retroperitoneal fibrosis (RPF) is described and the condition is reviewed as well. RPF is a rare disease of unclear etiology characterized by chronic retroperitoneal inflammation, which can entrap and obstruct retroperitoneal structures, notably ureters. A 45-year-old male patient presented with colic-likeleft flank pain irradiating to the testicle and progressively worsening. Retroperitoneal fibrosis with left ureteral compression was diagnosed in a abdominal computed tomography scan and the patient was submitted to reteral unblocking with insertion of a double-J catheter and then to clinical treatment with prednisone. Although there was no regression of the fibrotic mass, pain clinically relieved and no new ureteral obstruction episodes were seen. This case illustrates the importance of suspecting the above described condition. Although rare, RPF can potentially cause severe complications if it is not diagnosed and treated timely

Idiopathic Retroperitoneal Fibrosis Mimicking a Pelvic Tumor: a Case of Pericystitis Plastica

Retroperitoneal fibrosis was first described in 1905 by Albarran, a French urologist, who performed ureterolysis for ureteral compression produced by the disease. However, this disease became an established clinical entity by Ormond's account in the English literature in 1948. Pericystitis plastica has been used the define an extremely rare type of Idiopathic retroperitoneal fibrosis (IRF) constricting the bladder. In this study, we discussed the recovery of 29-year-old woman with pericystitis plastica who was misdiagnosed as pelvic malignancy or a chronic/subacut pelvic inflammation at the first evaluation.