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Imaging and intervention of retroperitoneal fibrosis.

Retroperitoneal fibrosis is a rare condition characterized by the development of fibrous plaques in the retroperitoneal space. The fibrous plaques characteristically arise distal to the bifurcation of the abdominal aorta and progress to encase the iliac vessels distally and are defined by the associated encasement of one or both ureters. Imaging plays an important role in not only establishing the diagnosis, but also in monitoring disease progression. Historically, the radiological diagnosis was made predominantly by intravenous urography and retrograde pyelography. More recently, advances in cross-sectional imaging with ultrasound and contrast-enhanced CT have allowed for a more precise diagnosis as well as helping to accurately define the extent of the disease. At our institution, we have found ultra-fast MRI to also play a useful role in establishing the diagnosis. In particular, magnetic resonance urography using HASTE (half Fourier-acquired single shot turbo spin-echo) sequences allow a safe alternative to intravenous urography, particularly in patients with poor renal function. The purpose of this article is to describe the role of the various imaging methods available to the radiologist and to emphasize the important role that the interventional radiologist now plays, not only in obtaining tissue for diagnosis, but also in providing treatment of the disease by percutaneous nephrostomy drainage and subsequent stent placement in select cases.

Diagnostic Imaging↗

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↗

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↗

Tuberculosis as a trigger of retroperitoneal fibrosis.

Retroperitoneal fibrosis (RPF) is usually idiopathic but sometimes secondary to underlying diseases. In this report, we describe a case of apparently idiopathic RPF associated with a mediastinal tuberculous adenopathy. After a course of antituberculous treatment, a complete resolution of both adenopathy and RPF was observed. We discuss the potential pathogenetic mechanisms linking the 2 diseases.

Antitubercular Agents↗

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↗

[Outcome of immunosuppressive treatment of a patient with renal failure due to retroperitoneal fibrosis].

Retroperitoneal fibrosis (RPF) is an uncommon disease of unclear aetiology. The review of the literature over the past 20 years revealed 160 published cases. Till now, no accepted diagnostic or therapeutic strategy exist. Most of patients diseases progress to end-stage renal failure without pharmacological treatment. In the paper we report a case of a 58-year old man admitted to the department of urology due to body mass loss, lower-back pain, vomiting, development of oliguria and anuria and intermittent claudication. On physical examination arterial hypertension (180/100 mm Hg), peripheral oedema, tenderness of the enlarged kidneys and lower limbs ischemia were found. Creatinine serum level (Pcr) was 232 micromol/l (2.69 mg/dl). On ultrasonography, symmetrical hydronephrosis and the existence of a hypoechogenic mass along of the aorta and below of renal arteries was found. The diagnosis of RPF was confirmed with MRI. Ureteral catheters were inserted with subsequent decompression of both kidneys and the patient was discharged from the hospital. Seven months later he still presented symptoms of lower limbs ischemia, arterial pressure was high and Per decreased to 138 micromol/l (1.55 mg/dl). The patient was admitted to the department of internal diseases. The treatment with prednisone at the dose of 40 mg/d during 6 weeks was introduced, and the dose was decreased gradually to 10 mg/d within 6 months. Simultaneously, the patient received intravenous therapy with cyclophosphamide 600 mg/infusion once monthly during 6 months. Two month after starting immunosuppressive treatment the intermittent claudication disappeared and after six months MRI examination demonstrated the regression of RPF. The ureteral catheters were removed. After 18 months of follow up, no recurrence of RPF is observed and the kidney function is normal.

Anti-Inflammatory Agents↗

Retroperitoneal fibrosis.

Retroperitoneal fibrosis (RPF) is an uncommon collagen vascular disease of unclear aetiology. It is characterised by a chronic non-specific inflammation of the retroperitoneum, which can entrap and obstruct retroperitoneal structures, notably the ureters. Because of the protean manifestations of RPF, awareness of the disease is important. It is still not uncommon to detect RPF only after severe renal failure is present. This comprehensive review deals with the various aspects of RPF and tries to provide a framework for the diagnosis, treatment and follow-up of this intriguing condition. Although it may have various causes, chronic periaortitis appears to be an increasingly encountered form of secondary RPF in patients with advanced atherosclerosis. Irrespective of its cause, most cases of non-malignant RPF - if in the active 'cellular' stage - will respond to treatment with corticosteroids, thereby obviating the need for surgical treatment. The clinical and radiographic improvement seen after starting steroid therapy is often impressive and reassuring as to the diagnosis. Treatment with corticosteroids may also make aneurysmectomy, if indicated, feasible in the patient who presents with perianeurysmal fibrosis and renal failure. Accumulating data suggest alternative treatment strategies for steroid-resistant cases (i.e. intensive immunosuppression) or when steroids are not feasible (i.e. other forms of immunosuppression or hormonal treatment, particularly tamoxifen). Although early diagnosis and treatment provide excellent renal and patient outcome, long-term follow-up is mandatory in all cases.

Adrenal Cortex Hormones↗

Robotic ureterolysis, retroperitoneal biopsy, and omental wrap for the treatment of ureteral obstruction due to idiopathic retroperitoneal fibrosis.

Retroperitoneal fibrosis (RPF) is a rare disorder of unclear etiology characterized by chronic inflammation of the retroperitoneum, which can involve any of the retroperitoneal structures, most notably the ureters, aorta, and vena cava. Historically, open biopsy, ureterolysis, and transpositioning or omental wrapping of the involved ureter(s) have been the preferred surgical treatments of RPF, with success rates greater than 90%. More recently, successful laparoscopic biopsy, ureterolysis, and ureteral omental wrapping and intraperi-tonealization have been described. We report the first case in the literature of idiopathic RPF managed with robotic ureterolysis and laparoscopic omental ureteral wrapping.

Case Reports↗

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↗

[Spinal cord compression: an unusual clinical manifestation of retroperitoneal fibrosis].

INTRODUCTION: Retroperitoneal fibrosis is a rare disorder consisted of a periaortic fibrous mass that compresses retroperitoneal organs. CASE REPORT: We report a case of retroperitoneal fibrosis diagnosed after a spinal cord compression. Magnetic resonance imaging scan showed a prevertebral mass which affected a vertebral body. It extended to the epidural space and compressed the spinal cord. The final diagnosis was established through the mass biopsy by means of mediastinoscopy. After prednisone treatment, both clinical remission and mass reduction in the follow-up MRI were observed. CONCLUSIONS: The spinal cord compression is an extremely unusual initial manifestation of retroperitoneal fibrosis. Nevertheless, it should be considered in the differential diagnosis of spinal cord compression syndrome, specially given the possibility of its medical treatment instead of aggressive surgical treatment.

Aged↗

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↗

Endovascular treatment of iliocaval occlusion due to idiopathic retroperitoneal fibrosis.

Idiopathic retroperitoneal fibrosis is a rare cause of venous occlusion that is mainly treated conservatively or surgically. However, endovascular techniques can be proposed for its treatment. We report here a case of a 64-year-old man who was admitted for bilateral lower limb swelling due to iliocaval occlusion caused by idiopathic retroperitoneal fibrosis. Due to high anesthetic risk, related mainly to congestive heart failure, the patient was treated percutaneously by balloon angioplasty and stenting instead of receiving surgical treatment. Clinical and radiological results were excellent, both early and in midterm, confirming that endovascular techniques constitute an interesting alternative to surgery, especially in fragile patients.

Journal Article↗