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Retroperitoneal fibrosis is characteristic plaques of dense fibrous tissue, typically extending from the level of the kidney down to the sacral promontory. Involvement of the presacral space is extremely rare in retroperitoneal fibrosis. We report two cases of idiopathic retroperitoneal fibrosis manifested by a presacral mass.
The nephropathology associated with retroperitoneal fibrosis has been commonly related to compression of the ureters and hydronephrosis. This assumption has been made without the use of immunofluorescent microscopy and electron microscopy. The authors studied renal tissue from a patient with retroperitoneal fibrosis by light microscopy, immunofluorescent microscopy and electron microscopy. In addition to the gross and microscopic changes of obstructive nephropathy, histologic findings of glomerular immune-complex deposition were also present. It is postulated that in some cases of retroperitoneal fibrosis, destruction may be mediated by both obstructive and immunologic processes.
Six cases of retroperitoneal fibrosis, each with a different pathogenesis and unusual localization were observed from 1980 to 1996. Four patients had had previous surgery for a neoplasm, one patient had idiopathic retroperitoneal fibrosis, and the last patient was hardly classifiable due to the complexity of the clinical pattern. The mean survival in 3 patients with malignant retroperitoneal fibrosis was 7 months. Two patients treated with medical therapy are still alive and in good clinical condition. The aspecificity of the symptoms makes early diagnosis difficult. CT and NMR are essential procedures for differential diagnosis of abdominal masses. Histology differentiates benign from malignant retroperitoneal fibrosis. The choice between medical or surgical therapy depends on the general condition of each patient.
Retroperitoneal fibrosis may occur secondary to metastatic neoplasm and may simulate the features of the idiopathic variety. A case of retroperitoneal fibrosis due to gastric carcinoma is presented and the historical, physical, cystoscopic, urographic, operative, and pathologic similarities to the idiopathic form discussed. Generous biopsy of retroperitoneal plaques and careful pathologic examination are stressed to avoid missing malignant disease.
Retroperitoneal fibrosis is a rare disease, typically with an insidious clinical course. It is thought that this disease process is perhaps an exaggerated reaction to an inciting inflammatory event. In this study, a case of retroperitoneal fibrosis is reported, in which the patient presented with typical symptoms of retroperitoneal fibrosis, along with some atypical vasculitic symptomatology. Retroperitoneal fibrosis is a disease process with an unknown etiology, which has been observed to be associated with a number of different possible inciting factors. Two factors that have been documented in the literature as being associated with retroperitoneal fibrosis include the use of beta-blocking agents, and connective tissue disease processes such as systemic lupus erythematosus. The patient discussed was using beta-blocker medication and also had signs and symptoms suggestive of a lupus syndrome. There are no reported cases of the combined association of beta-blocker usage, lupus, and retroperitoneal fibrosis.
Despite a thoracoabdominal retroperitoneal approach to an enlarging symptomatic infrarenal aortic aneurysm, proximal aortic dissection was hazardous due to radiation induced retroperitoneal fibrosis. Iliac artery ligation and thoracic aorta to iliac artery bypass has resulted in successful management during 14 months of follow-up.
Retroperitoneal fibrosis (RPF) is an uncommon entity that usually presents as a fibrous plaque-like lesion that encases the aorta and inferior vena cava below the level of the renal arteries. We present an unusual mass-like appearance of this disease simulating a tumour, with foci of calcification, seen in a young girl.
We describe a 70-yr-old female patient in whom both a retroperitoneal fibrosis and 6 years later a Riedel's thyroiditis were diagnosed. Both diseases belong to the group of fibrotic diseases called "multifocal fibrosis". Retroperitoneal fibrosis is now known to be an auto-allergic reaction to lipid components of the atherosclerotic process in the abdominal aorta, resulting in a fibrotic reaction around it. We hypothesize that similarly Riedel's thyroiditis is in fact a periarteritis of the carotid artery, resulting in cervical fibrosis, secondarily involving the thyroid. This hypothesis would account for the occurrence of these two rare diseases in one patient.
BACKGROUND: Idiopathic retroperitoneal fibrosis is characterised by proliferation and fibrosis of retroperitoneal tissue. It is complicated by obstruction and encasement of retroperitoneal structures. CASE REPORT: We describe two female patients with idiopathic retroperitoneal fibrosis. Both had to undergo lateralization of the ureter because of ureteral obstruction. Also both patients developed thrombosis of the inferior vena cava resp. the common iliac vein. Because of the eventful course of the disease a combined tamoxifen and steroid therapy was started. Hereafter there was a marked regression of the retroperitoneal fibrotic masses and the previous inflammatory signs disappeared. CONCLUSION: Tamoxifen seems to be effective in the treatment of idiopathic retroperitoneal fibrosis by inducing a regression of the fibrotic masses. Especially in patients with continuous activity of the disease we recommend an additional steroid therapy to prevent a regeneration of the fibrosis.
We evaluated 4 patients with surgically proved retroperitoneal fibrosis by computerized tomography and ultrasound. Retroperitoneal fibrosis was diagnosed correctly in all 4 patients by computerized tomography but was detected by ultrasound in only 1. On computerized tomography, retroperitoneal fibrosis demonstrated a characteristic soft tissue mass enveloping the abdominal aorta, inferior vena cava and ureters. Computerized tomography was capable of detecting the abnormality with greater reliability than ultrasound, and was far superior in delineating the extent of the process and its relation to adjacent abdominal structures. Computerized tomography is the method of choice in the preoperative evaluation evaluation of retroperitoneal fibrosis. It also is anticipated that computerized tomography will prove equally useful in the postoperative followup of this disease.
Retroperitoneal fibrosis is an inflammatory disease, which is either idiopathic or secondary to infection, neoplasm, hemorrhage, aortic aneurysm or drugs. This is a rare disease usually presenting constitutional symptoms, abdominal, back or flank pain and urinary frequency. Treatment includes surgical relief of urethral obstruction and corticosteroids. There is no clear evidence of the beneficial effect of corticosteroids treatment on the course of retroperitoneal fibrosis. We report a patient diagnosed with retroperitoneal fibrosis with an unusual presentation--uncontrolled HTN and renal failure due to renal arteries obstruction, without any abdominal symptoms. This patient responded to steroids and tamoxifen. A review of the literature is also presented.
A case of retroperitoneal fibrosis involving the testicle is presented. The widespread extent of the retroperitoneal fibrosis syndrome is reviewed briefly. The literature contains little information concerning the aetiology of testicular involvement in the disease process, which generally affects the para-aortic tissues. The physical signs mimic testicular neoplasm. A common embryological derivation of the tissues affected is postulated.
OBJECTIVE: A case of idiopathic retroperitoneal fibrosis in a 34-year-old man with anemia and elevated erythrocyte sedimentation rate is presented. METHODS/RESULTS: Ultrasound, CT and MRI evaluation and pathological study were performed. These diagnostic imaging techniques demonstrated an infiltrating mass in the upper portion of the abdomen which was resected. Histological and immunohistochemical studies of the surgical specimen demonstrated sclerosing retroperitonitis. CONCLUSIONS: Idiopathic retroperitoneal fibrosis (Ormond's disease) is a rare disease entity whose etiology is obscure and is characterized by retroperitoneal fibrosis with inflammatory infiltrates composed of plasma cells, lymphocytes and eosinophils. This case is of interest due to its atypical location, and because the patient was young with no remarkable clinical history and only mild anemia and an elevated erythrocyte sedimentation rate.
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Retroperitoneal fibrosis is a rare inflammatory disease, in which the fibrosis plate leads to the compression of the affected structures. There are several causes of retroperitoneal fibrosis like specific and unspecific inflammatory diseases. In the specific group of retroperitoneal fibrosis we find the one due to pancreatitis. We do present a rare case of retroperitoneal fibrosis caused by a pancreatitis in a HIV+ patient.
Four cases of idiopathic retroperitoneal fibrosis are reported. Magnetic resonance and computerized tomographic images were obtained for diagnosis in each case, and followup scans were obtained at various intervals after therapy. In all 4 cases magnetic resonance imaging was equal or superior to computerized tomography in the diagnosis of retroperitoneal fibrosis, outlining the extent of involvement, preoperative planning and assessing response to medical or surgical therapy.
A patient presented with retroperitoneal fibrosis but without any ureteric obstruction. The diagnosis was made by an abdominal CT scan and also at laparotomy. Post-operatively, she developed hypertensive encephalopathy. An isotope renogram with captopril was abnormal but not diagnostic of renal artery stenosis. The patient's condition improved with steroid and antihypertensive treatment. A follow-up CT scan showed complete resolution of peri-aortic thickening. A causative link is postulated between retroperitoneal fibrosis, trauma during laparotomy, and onset of acute hypertension.
Idiopathic retroperitoneal fibrosis may rarely invade the ureteral wall, thereby rendering the attempt to perform classical ureterolysis futile. Possibilities for surgical correction include resection with end-to-end anastomosis, utilization of a spiral strip of ureter, small bowel substitution, iatrogenic nephroptosis or longitudinal incision of the involved ureter down to the mucosa. Two patients treated using the latter technique have been followed for 7 months and 132 months without recurrence of the obstructive process. The surgeon, when treating idiopathic retroperitoneal fibrosis, must be prepared to treat intrinsic ureteral involvement using the most appropriate surgical technique.