Cause Of Retroperitoneal Fibrosis Research Articles

OA17 An unusual cause of unilateral retroperitoneal fibrosis

Abstract Background/Aims Retroperitoneal fibrosis (RPF) is a rare condition, with an incidence of around 1 per 200,000 to 500,000 per year. It is distinguished by fibro-inflammatory changes in the retroperitoneum, which typically surrounds the abdominal aorta. Idiopathic RPF is common, whilst secondary causes can result from drug therapies, infection, malignancy, or surgery. Early symptoms include dull, poorly-localised back or flank pain, and lower limb swelling. The classical focal mass of primary retroperitoneal fibrosis is typically situated over the distal aorta, anterior to the L4 and L5 vertebra. External compression of the ureter can result in hydronephrosis due to prevention of normal ureteric urinary peristalsis. Management options are limited, generally involving immunosuppression and urological surgery, often necessitating cross-specialty, multidisciplinary input. Methods We report the case of a young gentleman with no past medical or family history who presented with abdominal pain, vomiting, septic shock and metabolic acidosis. To exclude acute abdominal pathology, computed tomography (CT) abdomen and pelvis was performed. Incidentally, this found chronic inflammatory changes in the descending colon and right retroperitoneal soft tissue thickening causing severe right hydroureteronephrosis. This incidental finding of retroperitoneal fibrosis on CT scan prompted an exploration into infective, inflammatory, and malignant aetiological causes of retroperitoneal fibrosis. Results Following initial stabilisation, urological opinion was sought, which concluded that surgical intervention for ureteric stenting was unnecessary. This decision was supported by the absence of obstruction from fibrosis and the lack of signs indicating infection. No medical explanation was found for the initial presentation in extremis. Rheumatological advice was sought for consideration of biopsy and immunosuppressive therapy for this suspected primary retroperitoneal fibrosis. Upon inquiry, the patient divulged a historical occurrence of retroperitoneal fluid accumulation secondary to a paravertebral mass that mandated laparoscopic drainage four years ago overseas. Histopathological analysis of the mass confirmed the presence of tuberculosis (TB), which was treated. His recovery was complicated at that time by right-sided hydronephrosis and fibrosis necessitating decompression. This sequence of events was deemed by a radiologist to also account for the observed bowel findings in the right flank. Conclusion This case report is the first of its kind to document TB, or any infective aetiology, as being the primary cause of unilateral retroperitoneal fibrosis. Unilateral retroperitoneal fibrosis is incredibly rare, and there are only four English language case reports in the literature. Idiopathic RPF typically presents as bilateral concentric tissue around the abdominal aorta, whilst secondary causes can lead to unconventional imaging patterns, as seen in this case. It was only upon direct inquiry that the patient disclosed a prior diagnosis of TB affecting the lumbar spine. This case highlights the importance of meticulous history taking and systematic exclusion of secondary causes of RPF, prior to contemplating invasive biopsies and steroid therapy. Disclosure D. Li: None. M. Renju: None.

Retroperitoneal fibrosis as a postoperative complication following renal transplantation in cats.

The aim of this report was to describe the clinical signs, diagnostic imaging findings, surgical management, histopathological findings, outcome and possible risk factors for cats that developed retroperitoneal fibrosis (RPF) following renal transplantation. Medical records of cats that underwent renal transplantation and developed clinically significant RPF between 1995 and 2019 were reviewed. Eighty-one cats underwent 83 renal transplantations. Of these 81 cats, six developed clinically significant RPF. For all six cats, renal transplantation was performed using cold organ preservation solution and ureteral papilla implantation. Immunosuppression protocol included ciclosporin and prednisolone. All cats had at least one subtherapeutic trough ciclosporin level (<250 ng/ml) in the postoperative period. Cats presented with moderate-to-severe azotemia 39-210 days following renal transplantation. Abdominal ultrasonography and contrast pyelography revealed various degrees of hydroureter and hydronephrosis of the transplanted kidney. Surgical examination revealed a layer of dense fibrous tissue surrounding the transplanted kidney, ureter and bladder resulting in ureteral obstruction. Ureteral obstruction was managed by reimplantation of the proximal ureter or renal pelvis to the bladder. Histopathologic examination of the fibrous tissue and affected portion of the distal ureter revealed fibrous connective tissue with lymphoplasmacytic infiltration and perivascular inflammation suggestive of an autoimmune type reaction. Of the six cats, two died within 5 days after revision surgery, two developed signs consistent with recurrent partial ureteral obstruction (40 and 41 days after revision), one was euthanized 6 years later for an unrelated disease and one was lost to follow-up. The incidence of RPF in this population of cats was relatively low (7%), but still represents a significant cause of morbidity and mortality. The cause of RPF remains unknown, although investigation into suboptimal immunosuppression as a potential cause for local rejection reaction is warranted.

The Role of PET Scanning in the Evaluation of Patients With Kidney Disease.

Patients with underlying kidney disease are often required to undergo imaging for a variety of purposes including diagnosis and prognosis. A test that is being increasingly used with for this group of patients is the positron emission test (PET) scan. In addition, combining the nuclear medicine technique (PET) with computed tomography scan allows additional imaging advantages over either alone. These imaging modalities are commonly used for a number of extrarenal indications (ie, cancer, coronary artery disease, central nervous system disease, infectious diseases, and others). They have also been used for diagnosis of acute tubulointerstitial nephritis, evaluation and management of retroperitoneal fibrosis, identifying infection within kidney and liver cysts, and distinguishing complex kidney cysts from kidney cancer in patients with underlying CKD. We will review PET scan utility in patients with kidney disease.

An atypical cause of retroperitoneal fibrosis: Case report and literature review

IntroductionRetroperitoneal fibrosis (RPF) is a rare inflammatory disease resulting in the development of a retroperitoneal mass, which may encase the aorta and its branches, as well as the ureters. ObservationThe systemic inflammatory response causes constitutional symptoms. The mass itself leads to symptoms of non-specific back pain and abdominal pain, as well as ureteric obstruction and subsequent renal insufficiency. Immunoglobulin 4 (IgG4) has recently been found to be involved in the pathogenesis of multiple autoimmune and inflammatory disorders, including RPF. Blood work-up, imaging and biopsy of the mass remain the mainstay of diagnosis. ConclusionRPF remains a diagnosis of exclusion. Treatment hinges on corticosteroids, but other immunosuppressants and disease-modifying agents are also being used more commonly. Surgical intervention is only carried out when conservative measures have failed or are contraindicated, and in order to preserve renal function.

Laparoscopic Ureterolysis and Omental Wrapping in Patients with Retroperitoneal Fibrosis and Obstructive Uropathy: A Single-Center Experience

To analyze the outcomes with mid- to long-term follow-up of laparoscopic ureterolysis (LU) and omental wrapping in retroperitoneal fibrosis (RPF) with obstructive uropathy. Records of 9 patients with RPF who had obstructive uropathy at presentation and had undergone LU and omental wrapping at our center during January 2004 to June 2012 were collected and analyzed. Six females and three males underwent LU for RPF. Underlying causes of RPF could not be found in 8 (89%) cases. Two patients underwent bilateral LU. Mean operative time and estimated blood loss were 213 minutes (range, 180-280 minutes) and 119 mL (range, 70-200 mL), respectively. No case required conversion to open surgery. The only significant intraoperative complication (1/9 [11%]) was ureteral injury, which was easily repaired intraoperatively. The postoperative complication rate was 44% (4/9). Most complications (75% [3/4]) were minor and did not need specific treatment. The mean follow-up period was 46 months (range, 4-72 months). The success rate at last follow-up was 89%. Treatment of RPF is still controversial. Any future prospective randomized comparative trials seem unlikely in view of the low incidence of RPF. LU and omental wrapping in the setting of obstructive uropathy are safe and an effective alternative with a high success rate at mid- to long-term follow-up.

Evidence for clonal fibroblast proliferation and autoimmune process in idiopathic retroperitoneal fibrosis

Idiopathic retroperitoneal fibrosis is an uncommon disease characterized by encasement of retroperitoneal structures by fibrosis and chronic inflammation. Multiple etiologies have been proposed. First, we investigated if idiopathic retroperitoneal fibrosis is a clonal fibroblast proliferation by performing X-chromosome inactivation analyses. Second, we sought to determine if idiopathic retroperitoneal fibrosis is an autoimmune or immunoglobulin G4-driven process. Thirty cases of idiopathic retroperitoneal fibrosis, in whom known causes of retroperitoneal fibrosis were excluded and those for which paraffin blocks were available, were included in this study. We performed clonality analysis in 16 female patients. Genomic DNA samples were prepared from formalin-fixed, paraffin-embedded tissue sections using laser capture microdissection. Of the 16 cases, 15 were informative. Of 15 informative cases, 8 (53%) showed nonrandom X-chromosome inactivation or a clonal process. Of the 26 patients for which immunoglobulin G4 analysis was performed, 14 (54%) were positive for immunoglobulin G4-positive plasma cells, and all were negative for anaplastic lymphoma kinase. Of cases positive for immunoglobulin G4, the immunoglobulin G4:immunoglobulin G ratio ranged from 0.30 to 1.00 (mean, 0.80). Of the 12 patients for which both clonality analysis and immunoglobulin G4 analysis were performed, 4 (33%) were clonal and immunoglobulin G4 negative; 2 (17%), clonal and immunoglobulin G4 positive; 2 (17%), nonclonal and immunoglobulin G4 positive; and 4 (33%), nonclonal and immunoglobulin G4 negative. Our data indicate that a significant proportion (53%) of idiopathic retroperitoneal fibrosis cases in women is associated with a clonal expansion of fibroblasts. In addition, a subset of idiopathic retroperitoneal fibrosis cases could be classified in the immunoglobulin G4-related sclerosing disease spectrum.

Metastatic retroperitoneal fibrosis as first sign of breast cancer

Introduction: Malignancy is an uncommon cause of retroperitoneal fibrosis. Case Report: We discuss the case of a 66­year­old female who presented with anuric acute renal failure due to obstructive uropathy from retroperitoneal soft tissue infiltration and entrapment of both ureters. Fine needle aspiration and special staining of the retroperitoneal mass confirmed the malignant retroperitoneal fibrosis with breast as the primary site. Renal failure improved temporarily with bilateral ureteral stent placement but patient died during the course of palliative chemotherapy. Conclusion: Patients with retroperitoneal fibrosis often presents a diagnostic challenge. It is important to distinguish malignant retroperitoneal fibrosis from non­malignant causes due to its poor prognosis.

日本住血吸虫症による後腹膜線維症の１例

We report a case of retroperitoneal fibrosis due to Schistosoma Japonicum in patient with urothelial carcinoma. Retroperitoneal fibrosis was observed in a-83-year-old man during examinations for postrenal renal failure. The symptoms were improved by percutaneous nephrostomy and pulse therapy using corticosteroids, however, urothelial carcinoma was detected during follow-up examinations. The biopsy of retroperitoneal tissue was performed during the surgery for urothelial carcinoma. The histopathological examination revealed scattered calcified eggs of Schistosoma Japonicum in retroperitoneal fibrosis tissue. Therefore, in this case, we assumed Schistosoma Japonicum was the cause of retroperitoneal fibrosis. In our knowledge, there is no report about retroperitoneal fibrosis due to Schistosoma Japonicum. We hypothesize the pathway that the eggs penetrate into retroperitoneal space is extravasation from intestinal wall and peritoneal cavity, although the detail of this mechanism is not obvious. It is well known about the relationship between Schistosomasis and malignant tumors. However, it seemed no evidence regarding the relationship between Schistosoma Japonicum and urothelial carcinoma, since there is no report about it and the eggs were not found in specimen of urothelial carcinoma.

The combined surgical and medical treatment of retroperitoneal fibrosis.

Abstract While the cause of retroperitoneal fibrosis remains obscure, it is clear that a systemic disturbance often occurs at the onset. The treatment of choice remains ureterolysis combined with extraperitoneal lateral displacement of the ureter. Steroid therapy plays a part in dealing with the acute systemic phase, acts as a valuable adjuvant to surgery, and occasionally may obviate the need for operation in aged and debilitated subjects.

Fibrosis retroperitoneal por pancreatitis en paciente vih+

Retroperitoneal Fibrosis Due To Pancreatitis In Hiv+ PattentRetroperitoneal 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.

Retroperitoneal fibrosis

Retroperitoneal fibrosis is the formation of a fibrotic plaque in the prelumbar or presacral area which becomes clinically significant when it produces ureteral obstruction. It can occur as a result of taking certain drugs, retroperitoneal fluid collections, inflammatory disease of various intraabdominal organs, previous surgery or radiation therapy, and various metastatic neoplasms. The majority of the causes of retroperitoneal fibrosis are considered idiopathic.

Ureteral changes in gardner's syndrome simulating retroperitoneal fibrosis

The development of desmoid tumors in patients with Gardner's syndrome may produce ureteral findings on excretory urogram which, by themselves, are indistinguishable from other causes of retroperitoneal fibrosis.

Problem areas in uroradiology

Summary The representative roentgenologic studies in patients with retroperitoneal fibrosis of various causes have been presented. Retroperitoneal fibrosis may occur from presently unknown causes, and this type has been called idiopathic retroperitoneal fibrosis. It has now been shown that some patients have developed retroperitoneal fibrosis as a consequence of prolonged therapy with ergot derivatives, particularly methysergide. Withdrawing the methysergide from such patients can reverse the obstructive changes in the urinary tract, suggesting that the retroperitoneal fibrosis in these particular patients is preceded by some type of reversible exudative and/or cellular infiltrative process before actual fibrosis occurs. Recent reports note the occurrence of retroperitoneal fibrosis with carcinoid tumors of the small bowel. Retroperitoneal fibrosclerotic changes secondary to retroperitoneal malignancy occur, and the exuberance of this desmoplastic fibrotic response can hide the potentially fatal primary underlying etiology. This type of retroperitoneal fibrosis should be suspected in any patients with known past or present malignancy elsewhere in whom “retroperitoneal fibrosis” is found at surgery. There are other causes of retroperitoneal fibrosis, including heavy therapeutic irradiation, postoperative and post-traumatic sequelae and inflammatory changes about synthetic grafts. When the process is confined to the abdomen the ureters are frequently involved, resulting in symptoms and signs of progressive obstruction of the upper urinary tract(s). Because the fibrosis can encase and encroach upon other structures, the presenting symptoms may suggest primary disease in nonurologic organs. Urographic studies are presently the best method for demonstrating underlying pathophysiology. These and other roentgenologic findings, however, are more often suggestive than specifically diagnostic and the changes can be simulated by other nonfibrotic lesions that produce upper urinary tract obstruction, particularly primary or metastatic malignancy without fibrosis.

Retroperitoneal fibrosis associated with malignant disease.

The clinical features of 19 patients with malignant infiltration of the retroperitoneal tissues are described. These patients usually presented with unexplained uraemia and nonspecific symptoms; only a few had other evidence of malignancy. The diagnosis was resolved only by histological examination of multiple biopsy specimens. Since the prognosis is usually very poor it is essential to distinguish this condition from non-malignant causes of retroperitoneal fibrosis.