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Idiopathic retroperitoneal fibrosis is a disease of unknown etiology characterized by a dense rubbery retroperitoneal plaque that encases 1 or both ureters, causing hydronephrosis and impairing renal function. Primary or recurrent disease may result in extensive ureteral damage, leaving the urologist with limited surgical options. We herein report the fourth case managed successfully by renal autotransplantation.
Idiopathic retroperitoneal fibrosis is a rare disease and a delay in diagnosis may cause renal failure. Medical treatment i.e. corticotherapy or more recently, tamoxifene has been used successfully. This approach is recommended in patients either with moderate obstruction of the upper urinary, risk of major surgery or in cases of recurrence after surgical treatment. Ureterolysis using conventional surgery or laparoscopy remains the treatment of choice. This procedure should be considered in patients with neoplasic fibrosis, corticoresistant fibrosis or in cases of peri-aneurysm fibrosis.
Idiopathic retroperitoneal fibrosis (RF) is a histologically benign, nonspecific inflammatory process of the fibroadipose tissue in the retroperitoneum with an unknown etiology. It may be complicated due to encasement and obstruction of the retroperitoneal structures. Tamoxifen has been known to be an effective agent in regression of desmoid tumors which include similar mesenchymal elements to those seen in RF. Herein, we reported a case of RF presented with ureteral obstruction and acute renal failure. The patient was successfully treated with tamoxifen.
A case is presented of retroperitoneal fibrosis secondary to post-traumatic retroperitoneal hematoma, resulting in duodenal obstruction six years after the traumatic event. The fibrotic encasement of the duodenum and the head of pancreas made it necessary to perform an "en bloc" resection, with excellent results both immediately and after three years of follow-up.
Idiopathic retroperitoneal fibrosis is a rare collagen vascular disease of still unclear, immunological etiology. One of the first descriptions of this pathology was derived from Ormond and the Ormond's disease became its second name. It is characterized by a chronic, non-specific inflammation of the retroperitoneum, which obstructs retroperitoneal structures. The most frequent complication is the ureteric obstruction with hydronephrosis and renal failure. The fibrotic process may develop in different, even remote sites, including ophthalmic system. We have presented a 50-year-old woman with Ormond's disease and anterior uveitis. We have also described the other ocular complications of this disease.
Idiopathic retroperitoneal fibrosis (RPF) is one of the causes of obstructive uropathy. This disease, ever since its original description in 1948, has undergone much trial and error in its management. We present a case of a rare type of idiopathic RPF causing obstructive uropathy, which is perirenal and different from the classical RPF seen around the great vessels. To our knowledge, such a type of RPF has not been described. We treated the patient by a combined surgical approach in the form of ureterolysis and omentoplasty, followed by medical management with steroids and tamoxifen. The combined regimen yielded excellent results, and the patient is doing well at 9 months of follow up. The rarity of the presentation, with predominant bilateral perirenal fibrosis, and response of perirenal RPF to steroids and tamoxifen, is highlighted in this case presentation.
Idiopathic retroperitoneal fibrosis (IRF) is a rare condition of unknown etiology. An unusual case of IRF exclusively located at the peripancreatic area without extension below the lower renal poles is reported.
Idiopathic retroperitoneal fibrosis (RPF) is characterized by the development of a fibrotic mass surrounding the abdominal aorta and its branches, of unknown aetiology. Several immunological mechanisms can be operative in the pathogenesis of RPF. Based on this assumption we treated a patient affected by idiopathic RPF with low-dose methotrexate (MTX) therapy. To our knowledge this is the first example of the effectiveness and safety of a long-term low-dose MTX treatment in the post-surgical management of RPF.
Idiopathic retroperitoneal fibrosis (IRF) and primary biliary cirrhosis (PBC) are distinct clinical disorders which rarely occur in the same patient. We report a 79-year-old man with the coexistence of both conditions. The patient had antibodies to both centromere and mitochondria, as indicated by indirect immunofluorescence. Diagnoses of IRF and PBC were confirmed histologically. Although the association between IRF and PBC is obscure, IRF may be involved in many autoimmune diseases associated with PBC.
Idiopathic retroperitoneal fibrosis (IRF) is an inflammatory and fibrosing process that can be complicated by periureteral encasement, ureteral obstruction, and subsequent renal failure if left untreated. Unfortunately, treatment is often delayed due to the nonspecific nature of the presenting signs and symptoms. Clinical, radiologic, and microscopic findings in IRF, if examined independently, are all nonspecific for its diagnosis. Rendering a diagnosis of IRF by fine-needle aspiration (FNA) requires supportive clinical and radiologic data and systematic evaluation of entities in the differential diagnosis. Herein we report 2 cases of IRF diagnosed by FNA with subsequent histologic confirmation. Smears prepared from the aspirates revealed a combination of inflammatory cells and fibrous tissue. The inflammatory component was comprised of a mixture of lymphocytes, plasma cells, and rare eosinophils and mast cells. These 2 cases represent, to our knowledge, only the second report of IRF diagnosed by FNA.
Idiopathic retroperitoneal fibrosis is generally held to be uncommon. Its etiology is unknown. The disease continues to present with early bilateral ureteric involvement. Nowadays imaging techniques permit so a timely diagnosis to preserve and reduce renal damage. The optimum method of management is still controversial. Controversies on pharmacological, endourological and surgical treatment are debated. The advantages of various types of surgery are reviewed. In the urological department of the Civic Hospital in Brescia from February 1984 to June 1992, 87 patients (6 females and 2 males) with IRP were observed. Surgical treatment was combined with corticosteroids in 6 patients. Ureterolysis was performed with omental wrapping in 5 patients, with ureteric intraperitonealisation in 2 other ones. In the last case an ileal loop replacement was performed. In 5 out of 8 patients the ureteral stricture was resected and a termino-terminal anastomosis was necessary. Long-term follow-up is satisfactory. The authors conclude that omental wrapping is the safest method of choice.
Idiopathic retroperitoneal fibrosis (IRF) is a rare urological disease, for which many pathogenic theories have been proposed. The authors report a series of 13 cases of IRF in order to evaluate the clinical, diagnostic, laboratory, therapeutic and prognostic aspects. They also report a rare case (the ninth case reported in the literature) of multifocal fibrosclerosis. A possible genetic predisposition was studied by testing for the presence of immunophenotype HLA-B27; this test was positive in 44% of cases. A study of the immunological profile and lymphocyte populations revealed the typical features of chronic immune disease. Experience with medical and surgical treatment is reported, comparing various procedures: ureterolysis followed by application of a vascularized omental flap over the ureter (without subsequent corticosteroid therapy) gave the best results, with complete resolution of the symptoms and long-term successful alleviation of ureteric obstruction in 100% of patients, with a mean follow-up of 58 months.
BACKGROUND: Retroperitoneal fibrosis (RPF) is an uncommon disease with unknown causation in most cases. The pathognomonic finding is a fibrous mass covering the abdominal aorta and the ureters. Our aim was to clarify the possible role of asbestos exposure in the development of RPF. The hypothesis was based on the ability of asbestos to cause fibrosis in pulmonary and pleural tissue. METHODS: We undertook a case-control study of 43 patients with the disease (86% of eligible cases) treated in three university hospital districts of Finland in 1990-2001. For every patient, five population-based controls were selected, matched by age, sex, and central hospital district. We assessed asbestos exposure and medical history using a postal questionnaire and a personal interview. Of the 215 eligible controls, 179 (83%) participated in the study. FINDINGS: The age-standardised incidence of RPF was 0.10 (95% CI 0.07-0.14) per 100?000 person-years. The disease was strongly associated with asbestos exposure. The odds ratio (OR) was 5.54 (1.64-18.65) for less than 10 fibre-years of asbestos exposure and 8.84 (2.03-38.50) for 10 or more fibre-years, the attributable fraction being 82% and 89%, respectively. Other risk factors were previous use of ergot derivates (OR 9.92 [1.63-60.26]), abdominal aortic aneurysm (OR 6.73 [0.81-56.08]), and smoking for more than 20 pack-years (OR 4.73 [1.28-17.41]). INTERPRETATION: Our results show that occupational asbestos exposure is an important causal factor for RPF. For patients with work-related asbestos exposure, RPF should be considered an occupational disease.
We describe a previously unreported association of retroperitoneal fibrosis with biopsy proven periarticular inflammatory fibrosis. Histologic features were strikingly similar at the 2 sites. Plasma levels of a platelet derived growth factor, connective tissue activating peptide III, were found to be elevated and may play a role in pathogenesis. A dramatic response of the periarticular process to corticosteroids correlated with improvement of other variables of disease activity.
A patient is discussed who originally presented with retroperitoneal fibrosis, and subsequently developed both pituitary fibrosis and testicular fibrosis. Replacement of testicular tissue by fibrosis has not previously been described in this condition, while we have only been able to find a single previous report of pituitary fibrosis. Fibrous replacement of testis and pituitary should be considered as a further manifestation of the midline fibrosing syndrome.
The management of 2 cases of a rare condition previously labelled idiopathic pelvic fibrosis is described. Two cases reported previously are reviewed. The unusual histologic and radiologic features are emphasized, and the differences between this condition and idiopathic retroperitoneal fibrosis are summarized. Etiologic features, including the possible role of previous pelvic infection, drug-induced hypersensitivity and autoimmunity, are discussed. We believe that disease originating in the vas may be the source of this unusual fibrotic process.
Retroperitoneal fibrosis is a disease with replacement of normal retroperitoneal tissue with isolated fibrotic plaque placed around aorta and entrapping ureters. In 15% of patients an fibrotic process can be found outside the retroperitoneum. The most common localisation outside retroperitoneum is mediastinum, however, it can occur in every organ. The patient usually is diagnosed due to symptoms connected to compression of the ureters. We report the case of 41-year-old woman with chronic obstructive pulmonary disease complaining of nonspecific, dull, noncolicky pain localized to the back and abdomen. Finally we recognised retroperitoneal fibrosis. The diagnosis was proven by lumbar mass biopsy.
Retroperitoneal fibrosis was induced in 30 rats by placing a paraffin pellet containing 5 mg of phenol-mandelic acid behind the left ureter. In an additional 15 rats, the left urinary tract was transposed into a silicone sheath before the pellet of phenol-mandelic acid was placed behind the ureter in an attempt to protect the urinary tract from retroperitoneal fibrosis. In all 30 animals without the sheath, hydronephrosis of the left side occurred, and in the 15 animals with the sheath, lateral and anterior displacement of the left tract occurred, but function was not impaired. It is concluded that the silicone-sheath technique may represent a new therapeutic approach to retroperitoneal fibrosis.