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At least 19 recordsLinked to original sources
PubMed1984

Retroperitoneal fibrosis.

Retroperitoneal fibrosis is a rare disease but is important surgically because it can produce ureteral obstruction and renal failure. A wide variety of inflammatory, infective and neoplastic conditions can result in secondary retroperitoneal fibrosis and it is essential to look carefully for an underlying neoplastic condition in all cases of retroperitoneal fibrosis. Idiopathic retroperitoneal fibrosis occurs predominantly in men and results in some form of renal failure in 75% of patients. The classic triad of medial deviation of ureters, ureteral narrowing at L4-5 and proximal hydronephrosis is usually seen in high-dose intravenous pyelograms and the ease of retrograde ureteral catheterization is often diagnostic. Management depends on the degree of renal impairment, with laparotomy, multiple biopsies and ureterolysis being the treatment of choice. Steroids are beneficial, if used early, and the prognosis is excellent if there is no underlying malignant condition.

Humans↗
PubMed1996

Retroperitoneal fibrosis.

Retroperitoneal fibrosis is an uncommon inflammatory disease that leads to extensive fibrosis throughout the retroperitoneum. The majority of cases are idiopathic. The characteristic perivascular distribution of the idiopathic form supports the theory that the disease is an immune-mediated response to severe atherosclerosis. Evidence suggests that idiopathic retroperitoneal fibrosis may be part of a systemic fibrosing disease. Other less common forms include methysergide-related and malignant retroperitoneal fibrosis. The signs and symptoms of the disease are vague and nonspecific, and therefore the diagnosis relies heavily on radiologic findings. Once the diagnosis is suggested, the distinction must be made between malignant and nonmalignant retroperitoneal fibrosis, because the prognosis is dismal for malignant retroperitoneal fibrosis, but very good for other forms of the disease. Surgical biopsy remains the only way to definitively establish this diagnosis. Treatment may be surgical or medical, with the best outcome observed in patients receiving both.

Diagnosis, Differential↗
PubMed1990

Spinal cord compression secondary to idiopathic retroperitoneal fibrosis.

Retroperitoneal fibrosis is a rare disorder in which the abnormal fibrotic tissue compresses retroperitoneal organs. In the majority of patients no obvious cause can be found, hence the name idiopathic retroperitoneal fibrosis. The process can also arise from other anatomic areas, suggesting a multifocal origin. We report a case of dorsal epidural compression in a 63-year-old patient harboring idiopathic retroperitoneal fibrosis. A magnetic resonance imaging scan revealed the precise location of the lesion. It is suggested that retroperitoneal fibrosis should be considered in the differential diagnosis of epidural spinal cord compression.

Female↗
PubMed1996

Laparoscopic diagnosis of malignant retroperitoneal fibrosis.

Retroperitoneal fibrosis may be caused by a number of disease processes, both benign and malignant. Traditionally, open exploration and biopsy has been performed to exclude a malignant etiology prior to ureterolysis. We report a patient in whom a diagnosis of malignant retroperitoneal fibrosis was confirmed using laparoscopy. In patients in whom a metastatic etiology for retroperitoneal fibrosis is suspected, laparoscopy should be performed and representative biopsies taken. Laparoscopic techniques offer the urologist the ability to obtain an adequate tissue biopsy with a reduction in postoperative morbidity and convalescence associated with traditional laparotomy.

Biopsy↗
PubMed1975

[Problem of intestinal compression due to retroperitoneal fibrosis].

Retroperitoneal fibrosis causes almost without exception ureteral stenosis, frequently vascular constriction but rarely intestinal obstruction. Of 17 cases reported in the literature, 14 showed a colon sigma stenosis and 3 an obstruction of the duodenum. The appearance of intestinal stenosis neither correlates with the duration of symptoms nor with the extent of retroperitoneal fibrosis. Of our own cases, 4 out of 48 patients with retroperitoneal fibrosis presented a colon sigma constriction; of 17 patients with retroperitoneal fibro-carcinomatosis 2 had a stenosis of the sigma and one an obstruction of the duodenum. Discussed in reference to the literature and to two typical cases are difficulties in surgical intervention, the emergency situation of intestinal occlusion and the success and limitations of cortisone therapy for moderate intestinal constriction.

Colonic Diseases↗
PubMed1987

Familial retroperitoneal fibrosis.

Retroperitoneal fibrosis is an unusual cause of obstructive uropathy in the pediatric population. The etiology is unknown although there are laboratory and clinical associations with various autoimmune diseases. Familial associations have not been reported in children. A family is reported where two siblings have idiopathic retroperitoneal fibrosis. In addition, these sisters and the father manifest clinical laboratory evidence for systemic immunologic diseases. These young girls represent the first patients to illustrate both autoimmune and familial characteristics of this disease. These cases support the concept that retroperitoneal fibrosis is a local manifestation of a systemic immune disease.

Child↗
PubMed2000

[Treatment outcomes in primary and secondary retroperitoneal fibrosis].

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.

Adult↗
PubMed1990

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.

Humans↗
PubMed1988

Childhood retroperitoneal fibrosis.

Retroperitoneal fibrosis is rarely considered in the differential diagnosis of ureteral obstruction in children even when clinical presentation and radiologic findings are typical. In a 12-year-old boy admitted with a 2-week history of flank pain computed tomography showed an enhancing mass obstructing the left ureter. Pathologic examination of the mass and adjacent segment of ureter revealed retroperitoneal fibrosis.

Child↗
PubMed1997

Tamoxifen for retroperitoneal fibrosis.

Retroperitoneal fibrosis, either idiopathic or associated with aortic inflammatory aneurysm, is a chronic fibrotic disease that causes progressive obstruction of ureters and vessels around the lower abdominal aorta. Treatment is often difficult (surgery) or hazardous (steroids). We report a case of a woman with retroperitoneal fibrosis associated with aortic inflammatory aneurysm, who was successfully treated with oral tamoxifen.

Aortic Aneurysm, Abdominal↗
PubMed1990

Retroperitoneal fibrosis.

Retroperitoneal fibrosis, an uncommon and ill-defined condition, has a variety of causes and presenting features. The fibrotic process often produces ureteral obstruction and compression of surrounding structures. Pain in the flank, lower abdomen or lumbosacral region is the most common presenting symptom. Certain drugs, neoplasms, retroperitoneal injury or infections may all initiate the fibrotic process. This article describes a case of retroperitoneal fibrosis in a 34-year-old man who had been receiving a beta-adrenergic blocking agent.

Adult↗
PubMed1984

The urographic appearances in acute and chronic retroperitoneal fibrosis.

Retroperitoneal fibrosis is a disease which causes renal failure by obstruction of the ureters. The fibrosis may be benign or secondary to malignant disease. The clinical and radiological features of 30 cases have been reviewed. The symptoms are non-specific and diagnosis is often difficult. The most important features are back pain associated with a high ESR. Urography is diagnostic; the well known features are obvious dilatation of the pelvicalyceal system and ureter above the level of the obstruction. However, there is a spectrum of appearances corresponding to the duration of the disease and there may be only minimal dilatation of the pelvicalyceal system or no pelvicalyceal opacification at all. The progress of the disease is variable. Slow progress leads to chronic obstruction and chronic ill health. With more rapid progression, the patient may present with acute obstruction in anuria and, in such cases, urgent ureterolysis is necessary to conserve nephrons. Benign and malignant retroperitoneal fibrosis are often indistinguishable, clinically and radiologically, and laparotomy with biopsy is essential for diagnosis.

Acute Disease↗
PubMed1991

Retroperitoneal fibrosis.

Retroperitoneal fibrosis is idiopathic in two thirds of cases and is found most commonly as an isolated fibrotic plaque centered over the lower lumbar spine and entrapping one or both ureters. It has been postulated that the fibrosis in the idiopathic cases results from a hypersensitivity reaction to antigens leaking into the retroperitoneum from atheromatous plaques in the aorta or common iliac arteries. In the remaining one third of cases, causes include ergot-derivative drugs, retroperitoneal hemorrhage or urine extravasation, and a desmoplastic response to a variety of tumors. In up to 15% of cases, associated fibrotic processes are found elsewhere in the body. Although the disease is imaged best with CT or MR, neither of these techniques can be used to differentiate benign from malignant plaques. Histologically, the disease is characterized in its early stages by inflammatory cells and edema in a loose collagen network. The mature plaque is composed of dense fibrous tissue with minimal cellular infiltration. This progression is important as it influences MR signal characteristics.

Humans↗
PubMed1980

Retroperitoneal fibrosis.

Retroperitoneal fibrosis may be classified as either primary or idiopathic, probably of autoimmune origin or secondary to a malignant process. Both classes are usually presented identically, and it is the responsibility of the physician to exclude a malignant process. The disease should be especially suspected in patients with signs and symptoms of irritation of the retroperitoneal space, such as pain in the lower part of the back or in the flank, a distribution of pain down the legs and, at times, a positive Patrick's sign. Gray scale ultrasonography and computerized axial tomography may now be used to help in the diagnosis of this entity and also in the follow-up management. Operation is recommended for diagnosis and correction of any ureteral impairment. Steroids may be an invaluable aid for the treatment of this disease.

Adult↗
PubMedDate not supplied

Pergolide-induced retroperitoneal fibrosis.

Retroperitoneal fibrosis is a rare complication of pergolide therapy. This complication can be easily missed, so it is essential to have a high index of suspicion. We describe a case of well controlled Parkinson's disease who presented with shortness of breath and oedema.

Antiparkinson Agents↗
PubMed1982

Diagnosis and management of retroperitoneal fibrosis.

Retroperitoneal fibrosis (RPF) is an uncommon and ill-understood condition with a variety of causes and presenting features. An auto-immune vasculitis is thought to be responsible for the nonspecific inflammatory reaction, which is situated in relation to the aorta and major vessels, mainly in the lumbar region. Radiology plays a major role in its diagnosis, which can be confirmed by laparotomy and biopsy. Ureteric obstruction frequently occurs and must be relieved, and steroids may be required in its treatment. Seventeen patients diagnosed as having RPF are reviewed, and current views on its diagnosis and treatment are discussed.

Adolescent↗