Secondary Retroperitoneal Fibrosis Research Articles

Retroperitoneale Fibrose

Retroperitoneal Fibrosis (RPF) is a rare disease with peak incidence in the fifth to seventh decades of life. Its etiology remains to a great extent unclear; however, many characteristics of the disease suggest an autoimmune origin. Distinction is made between primary/idiopathic and secondary RPF. Symptoms may be general/nonspecific or localized (due to replacement or compression of organs). In addition to patient history, laboratory analysis and radiologic examinations, diagnosis often requires biopsy taken from the area affected. After restoring the function of involved (hollow) organs, medical therapy with prednisone, immunosuppressive drugs or tamoxifen is aimed at converting active disease to stable disease. However, some patients require ureteral surgery due to unsuccessful treatment. Life-long follow-up of patients is necessary since RPF can show a chronically relapsing course.

Comparison of Laparoscopic With Open Approach for Ureterolysis in Patients With Retroperitoneal Fibrosis

The surgical management of retroperitoneal fibrosis has traditionally involved open ureterolysis. We compared laparoscopic and open ureterolysis to determine if the minimally invasive approach offered advantages with respect to perioperative morbidity and treatment efficacy. We reviewed our retroperitoneal fibrosis database at a single institution to identify all patients who underwent open or laparoscopic ureterolysis between 1995 and 2005. Clinical, perioperative and outcome data were prospectively collected and compared between the open and laparoscopic ureterolysis cohorts. Subgroup analysis was performed on patients with primary retroperitoneal fibrosis comparing outcomes in open and laparoscopic ureterolysis groups. We identified 36 (51.4%) patients who underwent open ureterolysis and 34 (48.6%) who underwent laparoscopic ureterolysis. Conversion to open surgery was required in 17.6% of the laparoscopic ureterolysis cohort. The etiology of obstruction was primary idiopathic retroperitoneal fibrosis in 35 (50%) patients, whereas the remainder had secondary retroperitoneal fibrosis, largely related to gynecological malignancy. There was no difference between the 2 groups when comparing operative time, estimated blood loss, length of hospital stay, complications, transfusion requirements and postoperative resolution of ureteral obstruction. Subgroup analysis limited to patients with primary idiopathic retroperitoneal fibrosis demonstrated that those who underwent laparoscopic ureterolysis had a shorter hospital stay (3.4 vs 10.8, p <0.001) and were less likely to require transfusion (3.7% vs 13.7%, p = 0.007) compared to patients who underwent open surgery. Laparoscopic ureterolysis is an excellent option for patients with retroperitoneal fibrosis of all causes with morbidity and efficacy comparable to open surgery. In patients with primary idiopathic retroperitoneal fibrosis laparoscopy offers the added advantages of shorter hospital stay and reduced transfusion requirements.

Fibrosis retroperitoneal secundaria a neoplasia gástrica

The retroperitoneal fibrosis is a very little frequent pathology that habitually includes the abdominal aorta and ureters. Two thirds of cases the etiology is idiopathic and in a third it is secondary to other causes as drugs, generalized infections or diseases and tumors which are considered to be responsible from 8 to 10%. The most frequent is colorectal adenocarcinoma, being anecdotal the gastric location with six described cases to date. We present a case of a patient with gastric cancer and secondary retroperitoneal fibrosis of difficult diagnosis and handling.

Retroperitoneal Fibrosis, Today: An Updating of Knowledges on this Subjet

Retroperitoneal fibrosis (RPF) is characterized, at first, by a replacement of the normal retroperitoneal tissue by an active granulomatosis inflammation (cellular phase), and at a later stage by a fibrous scar tissue (established fibrotic phase). The aetiology of secondary RPFs includes several drugs (notably methysergide, ergotamine, pergolide, hydralazine), both chronic atherosclerotic aortitis-periaortitis and inflammatory aortic aneurisms, autoimmune diseases such as different forms of systemic vasculitis and collagen diseases, histiocytosis such as Erdheim-Chester syndrome, desmoplastic reactions to retroperitoneal malignancy carcinoid syndrome, retroperitoneal accidentally and surgically occurred traumas, abdominal radiation therapy. On the contrary, the causes of idiopathic RPF remain uncertain; its pathogenesis is associated to immuno-mediated mechanisms. The inflammatory process can involve retroperitoneal vessels, ureters, peri- and pararenal spaces, mesenteric small intestine, duodenum, psoas muscles, and can spread to mediastinal space. Diagnosis and characterization of the polyphase inflammatory evolution require integrated approaches including laboratory tests, morpho-functional imaging and, sometimes, histopathologic assessment. In the early stages, the management of RPF ranges from the removal of identifiable etiologic agents to the interfering with the inflammatory immuno-mediated process by means of several drugs. Unfortunately, many effective immunosuppressive drugs induce adverse reactions unrelated to their specific immunosuppressive action; this is the reason why the biopharmacology research today is struggling towards the identification of molecular targets having their expression restricted to immune cells and/or cytokines. Moreover, the progression of atheromatous aortitis to RPF could be prevented by statins which are able to interfere with the inflammatory pathway as well as to induce the well-known reduction in the levels of atherogenic lipoproteins. In the late established fibrotic stage, either open surgery or endourologic, laparoscopic procedures are performed; nevertheless neoadiuvant and adiuvant corticosteroid-immunosuppressive treatments are mandatory in order to avoid any relapse of the disease.

Ureteral-Arterial-Enteric Fistula As A Complication Of Indwelling Ureteral Stents

Since the first double-J stent by Finney in 1978, ureteral stents have been a safe and effective way to maintain ureteral patency in various benign and malignant conditions.1 Common early complications include stent discomfort, irritative bladder symptoms, hematuria, urinary tract infections and flank pain. Late complications include encrustation, breakage, migration and hydronephrosis. A more serious and life threatening complication, although much less common, is erosion into a major vessel resulting in life threatening hemorrhage. There have been several reported cases of ureteral-enteric and ureteral-arterial fistulas and its management. We report a ureteral-arterial-enteric fistula as a complication of chronic indwelling double-J ureteral stents. CASE REPORT A 59-year-old female with a history of cervical carcinoma underwent a radical hysterectomy followed by external beam radiation to the pelvis. She did well for two years but then developed bowel obstruction secondary to metastasis to the colon and required a left hemicolectomy and Hartman's pouch colostomy. Six months later, she presented in acute renal failure with an elevated creatinine of 2.5 mg/dl (normal 0.6-1.2 mg/dl) and bilateral hydronephrosis believed to be secondary retroperitoneal fibrosis from pelvic radiation or recurrent disease. A right ureteral stent was placed retrograde and a left stent was placed antegrade by interventional radiology with normalization of renal functions. Stent changes at three monthly intervals were performed by interventional radiology for one year. During this time, she maintained a stable creatinine of 1.2-1.4 mg/dl but had reported periodic episodes of painless gross hematuria following stent changes, which was attributed to instrumentation. Subsequently, she developed acute onset of painless gross hematuria and bleeding from her Hartman's pouch. Upon admission, she had a hematocrit of 15% (normal 42-50%) and a creatinine of 1.3mg/dl. Following multiple transfusions, urgent cystoscopic and colonoscopic evaluation was performed. Cystoscopy revealed no active bleeding from the ureteral orifices or stents. Endoscopic evaluation of the pouch revealed a segment of the ureteral stent extruding into the lumen of the pouch. A Hartman's pouchogram

Behandlungsergebnisse der primären und sekundären retroperitonealen Fibrose

Retroperitoneal fibrosis (RPF) is an uncommon inflammatory disease of the retroperitoneum leading to extensive fibrosis with consecutive obstruction of adjacent organs, namely the ureters. Since no consensus on the standard therapy exists, aim of the current study was to evaluate the outcome of 39 patients with RPF. Between 1986 and 1997 39 cases of RPF were diagnosed: 21 cases had primary RPF and 18 patients had secondary RPF after aortofemoral graft (n = 13), radiation (n = 2), or prior retroperitoneal surgery (n = 2). 21 patients demonstrated unilateral and 16 cases had bilateral hydronephrosis, in 2 patients no dilatation was observed. In 28 cases (n = 12 primary RPF, n = 16 secondary RPF) initial management consisted of DJ-stent placement, whereas in 11 cases (n = 9 primary RPF, n = 2 secondary RPF) percutaneous nephrostomy had to be placed. All patients received oral immunosuppressive agents (prednisolone 1 mg/kg, azathioprine 1 mg/kg/day) for 3 months before reevaluation was performed. In case of complete remission, immunosuppressive medication was continued for another 3 months, in case of stable disease or progression surgery was performed. In 26 cases (n = 15 primary RPF, n = 11 secondary RPF) ureterolysis with intraperitoneal displacement and omental wrapping was performed. 3 patients demonstrated complete remission after oral prednisolone/azathioprine; in 2 cases RPF presented as pelvic mass and was resected followed by immunosuppressive therapy, in another 2 cases bilateral ileal replacement of the ureters had to be performed and 4 cases remained on DJ-stents and nephrostomy, resp. Postoperatively, all patients with primary RPF were continued on immunsuppressive medication for another 3 months. After a follow-up of 6 to 120 months only 3 patients developed a retroperitoneal recurrence and were treated by unilateral nephrectomy or DJ stent placement (n = 2). Our data suggest that the combination of both immunosuppressive medication and surgical management results in an excellent longterm outcome in idiopathic retroperitoneal fibrosis with a recurrence rate of only 8%. Combination therapy should be considered as therapeutic option early in the course of the disease. Primary reconstructive surgery appears to be the most promising approach in secondary retroperitoneal fibrosis with a recurrence rate of only 5%; short external compression of the ureter might be managed by endoluminal balloon dilatation.

La fibrosi retroperitoneale idiopatica. Terapia medica: Idiopathic retroperitoneal fibrosis. Medical treatment

Medicai treatment of both idiopathic and secondary retroperitoneal fibrosis (RPF) aims at stopping the inflammatory process in the early stage of the disease. Glucocorticoids and non-steroidal antiestrogen drugs are used. Methylprednisolone, the most commonly used glucocorticoid, is successfully employed with different therapeutic protocols. Although based on a few cases, results of treatment with tamoxifen are also satisfactory. The authors report 25 cases of RPF, idiopathic in 14 and secondary in 11. Twelve patients underwent medical therapy, corticosteroids being used in 10 and tamoxifen in 2. All patients recovered from the fibrotic disease. Only 1 patient stopped steroid therapy due to worsening diabetes. Easy administration and the lack of side effects make tamoxifen a valid alternative to corticosteroids.

Computed tomography in the diagnosis of retroperitoneal fibrosis.

We report the findings on computed tomography (CT) in idiopathic and secondary retroperitoneal fibrosis in 7 patients. In all cases CT showed the fibrotic mass, accurately defining its extent and the involvement of adjacent anatomic structures. The densitometric and anatomopathologic aspects were correlated; contrast uptake was less in areas thought to be dense in collagen than it was in areas with more vascular regions of inflammatory change. CT assessed the extent and nature of the fibrotic plaque.