| The case of a 52-year-old man with retroperitoneal fibrosis and ankylosing spondylitis is described. Inflammatory low back pain and acute renal insufficiency prompted a computed tomography scan of the abdomen with contrast agent injection. A fibrous sheath surrounding the aorta and attracting the ureters toward the midline was seen, strongly suggesting retroperitoneal fibrosis. The diagnosis of ankylosing spondylitis was based on the presence of inflammatory low back pain responsive to nonsteroidal anti-inflammatory drugs, syndesmophytes at the lumbar and cervical spine, bilateral sacroiliitis, and presence of the hla-b27 antigen. prednisone therapy in a daily dosage of 1 mg/kg induced a marked improvement. Only nine cases of concomitant retroperitoneal fibrosis and ankylosing spondylitis have been reported. These two conditions share similarities in some of the etiologic factors and anatomic localizations, suggesting that both may stem from a predisposition to fibrotic diseases. ( info) |
| retroperitoneal fibrosis is a rare disease of uncertain pathogenesis. However, its possible association with several immunopathologic conditions, the possibility of systemic involvement by the fibrous process, the presence of various autoantibodies, and the frequent response to immunosuppressive treatment all support an autoimmune pathogenesis. Riedel's thyroiditis is a rare disease the pathogenesis of which is also thought to be immune-mediated based on its optimal response to steroids; Riedel's thyroiditis is also frequently reported in association with retroperitoneal fibrosis. We describe here two cases of autoimmune thyroid disease associated with retroperitoneal fibrosis, the first with features of primary myxedema, the second of primary thyrotoxicosis. histology of retroperitoneal fibrosis is documented and it is compatible with an immunopathologic condition. Thus, these two cases add further support to the hypothesis of an autoimmune pathogenesis of retroperitoneal fibrosis and indicate the importance of carefully monitoring for the development of other autoimmune disorders, i.e., of the thyroid gland, in patients with retroperitoneal fibrosis. ( info) |
63/359. Clinical and radiological evolution of a case of idiopathic retroperitoneal fibrosis treated with tamoxifen. The authors report a case of retroperitoneal fibrosis conservatively treated with tamoxifen. The computerized tomography (TC) control at ten months showed the persistence of a slight residual of peri-aortic fibrotic tissue. This result allowed removal of the double J catheter. ( info) |
| We present 3 cases of a rare syndrome of extrinsic ureteral obstruction produced by retroperitoneal fat necrosis and compare the medical records and histopathology to 19 cases of classic idiopathic retroperitoneal fibrosis. Clinical similarities include average age of onset in the fourth and fifth decades, male preponderance, abdominal or back pain as the presenting symptom, elevation of the blood urea concentration prior to diagnosis and tendency toward bilateral involvement. Histologically, the 2 conditions were different. Retroperitoneal fat necrosis is characterized by coalescence of fat cells into fat cysts bordered by foreign body giant cell granulomas, foam cells, light chronic inflammatory infiltrate and unimpressive fibrosis. The microscopic pathology of idiopathic retroperitoneal fibrosis is dominated by densely collagenized fibrous tissue of varying cellularity without evidence of fat necrosis. Of the 3 cases of retroperitoneal fat necrosis 2 featured prior ischiorectal abscesses and this is considered in a discussion of the etiology of this unusual cause of ureteral compression. ( info) |
65/359. Localized ureteral fibrosis: an unrecognized complication of radiation therapy for prostatic carcinoma. Complications of radiation therapy for prostatic carcinoma are frequent but usually minor. A patient is described in whom localized ureteral fibrosis developed following curative radiation therapy, which subsequently required a reconstructive operation. An analogous situation regarding patients with carcinoma of the cervix is examined and possible etiologies are discussed. This complication should be recognized so that proper management can be instituted. ( info) |
| We report a rare autopsy case of secondary gastrointestinal amyloid A (AA) amyloidosis associated with idiopathic retroperitoneal fibrosis (IRF) in a 67-year-old woman. Masses were identified around the aorta and inferior vena cava in her abdomen. Histologically, plasma cell infiltration was observed within fibrotic areas. Because no specific cause for the inflammatory mass was apparent, we diagnosed it as IRF. Steroid therapy, which usually reduces IRF masses, proved ineffective, and malabsorption syndrome developed 4 years later. On autopsy, amyloid protein was present systemically in the vascular walls of several organs, and deposition was highest in the gastrointestinal mucosa. Amyloid protein was identified as AA type, strongly suggesting that the amyloidosis was secondarily induced by IRF. To our knowledge, only 2 other cases of IRF-associated amyloidosis have been reported. Two of the 3 patients of these cases were women who showed resistance to steroid therapy. ( info) |
67/359. Haemoperitoneum secondary to ureteric stenting. We report an unusual but catastrophic complication of 'haemo peritoneum' noted following an uneventual insertion of ureteric stent. Its management was primarily affected by the presence of a significant coexisting pathology. This case highlights two important points: (1) recognizing the most rare but real possibility of major intra peritoneal haemorrhage following simple stenting of the ureter (2) the importance of checking that the guide wire has been correctly preloaded in the spool before its placement up the ureter as inserting the wrong (nonfloppy) end may have serious implications. ( info) |
68/359. Closure of a ureterocutaneous fistula with a covered stent-graft. A 74-year-old woman developed retroperitoneal fibrosis after aortic surgery for a left common iliac artery aneurysm. On the 5th day after repair, a left groin ureterocutaneous fistula developed. Because of the presence of a hostile surgical bed, the fistula was treated with percutaneous nephrotomy and double J stent insertion. Despite proximal control for more than 1 year, the fistula persisted. She was referred to the interventional radiology department for exchange to a nephrostomy with ureteral embolization. Rather than perform embolization of the ureter, we successfully repaired the fistula with a covered stent-graft. The patient was seen again 1 year after stent-graft placement. Output continues to fluctuate but never exceeds 5 mL per day. ( info) |
69/359. 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. ( info) |
70/359. Idiopathic presacral retroperitoneal fibrosis: report of two cases. 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. ( info) |