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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

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

[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

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

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

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

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

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

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

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

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

Idiopathic retroperitoneal fibrosis (sclerosing retroperitonitis).

Three cases of idiopathic retroperitoneal fibrosis, one of which was localized to the perirenal area, are presented. The predominance of plasma cells, which may be difficult to recognize because of distortion unless methyl green-pyronine staining is done, and the character of the fibrous tissue indicated the non-neoplastic nature of the processes. This diagnosis was confirmed by immunoperoxidase studies that demonstrated polyclonality of the lymphoplasmacytic component. Immunologic studies, which may be performed on paraffin-embedded tissue, are helpful in differentiating this lesion from the sclerosing lymphomas that also occur in the retroperitoneal area.

Adult

Idiopathic peritesticular fibrosis associated with retroperitoneal fibrosis.

A 32-year-old male who presented with idiopathic retroperitoneal fibrosis developed peritesticular fibrosis 4 years later. The lesion appeared as a diffuse, uniform thickening without a tendency to form nodules. Histologic examination revealed a densely collagenized tissue with scattered cells, consisting mainly of fibroblasts and plasma cells. Small blood and lymphatic vessels of the tumor showed luminal dilation and perivascular edema with inflammatory infiltrates. No other associated pathology was found in the patient. We suggest that peritesticular fibrosis in this patient may be due to lymphatic drainage obstruction caused by retroperitoneal fibrosis.

Adult

Iliocaval complications of retroperitoneal fibrosis.

BACKGROUND: Retroperitoneal fibrosis can compress ureters, nerves, and blood vessels in the abdomen. However, clinically significant large-vein obstruction secondary to this process is rare. METHODS: Three hundred forty patients with retroperitoneal fibrosis were treated at our institution between 1976 and 1993. The clinical data from seven of these patients, who were treated for iliocaval complications of retroperitoneal fibrosis, were reviewed. RESULTS: Six patients had signs and symptoms of chronic obstruction, and one patient presented with acute iliocaval thrombosis and underwent attempt at venous thrombectomy in the referring hospital. All patients exhibited extremity edema and three had venous claudication. Iliocaval occlusion was confirmed in all patients by venography, computed tomography, or magnetic resonance imaging. The obstructive process involved the iliocaval tree (four patients), the inferior vena cava alone (two patients), and the iliac vein alone (one patient). Five patients were managed conservatively with leg elevation, compression stockings, and anticoagulation. Two patients received prednisone. One patient underwent an iliocaval bypass from the external iliac vein to the juxtarenal cava using a ringed polytetrafluoroethylene graft with a femoral arteriovenous fistula. A second patient with an isolated left common iliac vein obstruction underwent a left-to-right femorofemoral saphenous vein bypass. Four patients treated conservatively continued to have extremity edema. The two patients managed surgically remain asymptomatic from venous insufficiency, with patent grafts at 25 and 12 months after surgery, respectively. CONCLUSION: Iliocaval obstruction is an unusual complication of retroperitoneal fibrosis. Although most cases can be managed conservatively, reconstruction is an option for patients who have failed medical treatment and are symptomatic secondary to chronic venous obstruction. Lifelong anticoagulation should be considered for all patients with progressive iliocaval obstruction secondary to retroperitoneal fibrosis.

Acute Disease

Variable symptomatology in idiopathic retroperitoneal fibrosis.

Idiopathic retroperitoneal fibrosis is uncommon enough to be placed low down on a list of differential diagnoses of any problem facing a general surgeon in a district hospital. In this paper, 4 cases of retroperitoneal fibrosis are described, all of whom presented within a 5-year period to the same surgeon; each described a different symptomatology. This paper serves to reiterate the diversity of presenting features in retroperitoneal fibrosis and to demonstrate that this condition may not be as rare as is widely believed.

Adult

Innovative surgical management of idiopathic retroperitoneal fibrosis.

Idiopathic retroperitoneal fibrosis is a rare entity usually treated with exploratory laparotomy, deep biopsies of the fibrotic process and uretrolysis. Innovative surgical management occasionally is required for ureteral obstruction. We report the use of dismembered pyeloplasty, autorenal transplantation and bilateral psoas hitch ureteral reimplantation for the management of ureteral obstruction associated with idiopathic retroperitoneal fibrosis.

Adult

Maternal death from severe retroperitoneal fibrosis.

Idiopathic retroperitoneal fibrosis is uncommon in pregnancy. We have described the case of a pregnant patient with a benign retroperitoneal spindle cell tumor involving the distal duodenum, right ureter, and right iliac artery and vein. She was treated with a left ureteral stent and a right percutaneous nephrostomy. Ultimate cause of death was electrolyte imbalance due to bowel obstruction.

Adult