1/359. Multifocal fibrosclerosis presenting as Grave's orbitopathy. Bilateral exophthalmos associated with retroperitoneal and sellar fibrosis. BACKGROUND: Multifocal fibrosclerosis (MF) is a rare disease that may be misdiagnosed as Graves' orbitopathy. The combination of localisations of MF presented here has not been reported before. CASE REPORT: A 44-year-old man was referred with progressive bilateral exophthalmos. CT of his chest and abdomen revealed an intrathoracic and retroperitoneal peri-aortal soft-tissue process with hydronephrosis. Histological examination of orbital masses showed a fibrous process with low inflammatory activity. Later the sella was found to be involved as well. A diagnosis of MF was made. CONCLUSION: MF should be considered in the differential diagnosis of bilateral exophthalmos. ( info) |
| A case of rare combination of retroperitoneal fibrosis and Riedel's thyroiditis is presented. The term multifocal fibrosclerosis is used in the literature to denote such combination and others which include idiopathic retroperitoneal fibrosis, mediastinal fibrosis, sclerosing cholangitis, invasive (Riedel's) thyroiditis and pseudotumor of the orbit. ( info) |
| retroperitoneal fibrosis in a 12-year-old boy is reported. This was associated with a fibrotic mass in the neck which resolved spontaneously. Right-sided ureteric obstruction responded to treatment with steroids. ( info) |
4/359. Systemic multifocal fibrosclerosis. We describe a case of hydronephrosis as a result of retroperitoneal fibrosis in a patient who had previous sclerosing lobulitis of the breast. To the best of our knowledge this is the first reported association between these two conditions in the english literature. We presume these conditions are linked and unify them under the general heading of systemic multifocal fibrosclerosis. ( info) |
5/359. Management of ureteric obstruction in the solitary kidney by a segmental suspended ureteric prosthesis. Ureteric obstruction of a single kidney, secondary to an aorto-iliac bypass graft, was treated with a suspended segmentary ureteric prosthesis. No urinary stasis was observed during 1 year and the urodynamic implication are discussed. ( info) |
| A case of retroperitoneal fibrosis with an unusual perirenal involvement diagnosed at MR imaging is reported. Other conditions, such as metastatic disease or lymphoma, may be considered especially when the initial presentation is not typical. Imaging modalities in this condition are discussed. ( info) |
7/359. Conservative treatment of acute Ormond's disease. Ormond's disease is a chronic inflammatory process of the retroperitoneum, which sometimes takes a very acute course. We report the case of a man with acute bilateral hydronephrosis. diagnosis was based on typical criteria seen by magnetic resonance imaging, which made biopsy unnecessary. ( info) |
8/359. A case of ureteric obstruction, retroperitoneal fibrosis, and carcinoid tumour. We report the incidental finding at surgery for retroperitoneal fibrosis of a carcinoid tumour causing complete right ureteric obstruction. retroperitoneal fibrosis is an uncommon inflammatory disease that leads to extensive fibrosis throughout the retroperitoneum. It can occur at any age, peak incidence being in patients between 40 and 60 years of age. Carcinoid tumours arise from enterochromaffin or amine precursor uptake and decarboxylation cells that occur in gastrointestinal tract. Carcinoid tumours are an uncommon clinical entity and incidence varies with gender and age. No association between retroperitoneal fibrosis and carcinoid tumour has been previously reported in the English literature, although one case has been reported in a French journal. ( info) |
9/359. Fine-needle aspiration findings in idiopathic retroperitoneal fibrosis. 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. ( info) |
| In a 46-year-old man endoscopic retrograde cholangiopancreatography and computed tomography scan showed a stenosis of the common bile duct by a hypodense mass highly suggestive of a Klatskin tumor. Histologic examination of the resected tumor revealed only non-specific inflammatory, fibrotic tissue without any evidence of malignancy. Three months later, the patient presented with hydronephrosis of the left kidney. Computed tomography scan showed a retroperitoneal mass with encasement of the left ureter. A percutaneous nephrostomy was performed and immunosuppressive therapy with prednisolone and azathioprine was initiated. Under this medication, almost complete regression of the pelvic mass and reopening of the ureter were observed within 3 weeks. Eight months later, azathioprine was withdrawn and prednisolone was tapered continuously to a dose less than 10 mg/day. After a follow-up of 2 years, the patient is still well. Although the histologic findings were non-specific, further evaluation of this case suggests that Ormond's disease was responsible for the tumor that had to be resected. ( info) |