Idiopathic Retroperitoneal Fibrosis. A Retrospective Analysis of 60 Cases

Abstract

Sixty patients with idiopathic retroperitoneal fibrosis presenting between 1965 and 1984 are reviewed. Their mean age at presentation was 56 years and the male:female ratio was 3:1. The commonest presenting symptoms were flank and abdominal pain, weight loss, nausea and polyuria. Physical examination was usually normal, expect for the presence of hypertension. Anaemia and elevation of erythrocyte sedimentation rate were usually present. Proteinuria was found in less than a third of patients at presentation and significant bacteriuria was uncommon. The correct diagnosis was made or suspected in very few patients before referral. The cumulative actuarial survival rate was 86% at 1 year and 78% at 2 years. Seventeen patients died; they were significantly older and more uraemic at the time of referral than those who survived. A few patients did well with either corticosteroid therapy or ureterolysis alone. In the majority, both operation and steroid treatment were necessary. In bilateral obstruction with residual function in both kidneys, bilateral ureterolysis proved superior to unilateral operation (each followed by steroid therapy) in conserving renal function. Operation alone or steroid therapy alone should be considered in cases where steroids or surgery respectively present particular hazards. The less traumatic unilateral operation should be considered in poor risk patients and in those whose renal function is absent on one side. In many survivors, disease activity has persisted for many years. Life-long follow-up is recommended.

Simultaneous bilateral subcutaneous pyelovesical bypass as a salvage procedure in refractory retroperitoneal fibrosis

“…2 Ureteral obstruction with secondary acute or chronic renal failure is the most common and severe complication associated with RPF, with ureteral involvement reported in 80% to 100% of cases. 4,5 Generally, the goals of therapy are to relieve obstruction and to slow or prevent the progression of fibrosis. Initial treatment of urinary tract obstruction involves steroid therapy together with the placement of indwelling double pigtail stents or percutaneous nephrostomy tubes.…”

Retroperitoneal Fibrosis in a Chinese Woman with Systemic Lupus Erythematosus: A Case Report

“…Some patients also showed symptoms of urinary system such as oliguria, dysuria and hematuria. Hypertention may be found in many patients due to renal artery impingement .The ureters was occasionally pressed by the mass when the disease develops into the ureter, 4which may lead to ureteral stricture [10 ] that causes acute renal dysfunction or chronic renal failure. Since the clinical symptoms of retroperitoneal fibrosis are nonspecific, the diagnosis of RPF depends on laboratory and imaging examination.…”

Retroperitoneal fibrosis – an unsolved mystery

“…It also causes a reduction of retroperitoneal mass and a resolution of obstructive uropathy (29). However, some authors are reluctant to use steroids as primary therapy before performing an open or laparoscopic biopsy to exclude malignant RPF (23). This is usually combined with a surgical procedure at the same time, such as ureterolysis or omental wrap.…”

“…Therefore some authorities consider retroperitoneal biopsy as a mandatory diagnosis confirming step before treating this disease (19,23). Even on biopsy the malignant areas may be missed.…”