Retroperitoneal fibrosis (ormond’s disease). Clinical case

Abstract

Retroperitoneal fibrosis (Ormond’s disease) is a nonspecific inflammatory process in the retroperitoneal tissue with the formation of fibrous tissue that causes compression of the ureter and other adjacent structures. This is a rare disease, its frequency is about 1 case per 200 thousand people. This explains his little scrutiny, the absence of a real standard of patient management with the determination of the nature of drug therapy and the most effective method of surgical treatment. The prognosis is determined by the activity of the disease with the development of urinary tract obstruction and the occurrence of renal failure and other complications. The article presents a clinical observation of a 40-year-old patient suffering from Ormond’s disease. In this case, the initial treatment to remove retroperitoneal fibrosis was undertaken by surgeons 5 months after the onset of the first symptoms of the disease. Drug therapy was started 10 months later, when the final diagnosis was made using immunohistochemistry and the progression of the disease became apparent (by increasing the amount of retroperitoneal fibrosis). On the background of immunosuppressive therapy, a decrease of the severity of retroperitoneal fibrosis was noted, however, it was not possible to achieve the full effect, most likely due to the late start of treatment and the irreversible fibrosis formed in this connection. Treatment was also hampered by the persistently recurring urinary tract infection. The best method of treatment in this situation (with persistent obstruction of the ureter and the threat of renal damage) can only be surgical treatment aimed at restoring adequate urodynamics. Based on the presented clinical observation, we can make the following conclusions: Ormond's disease (retroperitoneal fibrosis) needs further study and development of standards for the management of patients with this pathology; immunosuppressive therapy should be prescribed as soon as possible, which can prevent the development of irreversible fibrosis; With advanced stages of the disease, treatment should be comprehensive, including both remedies and surgical intervention.

Keywords

• retroperitoneal fibrosis
• ormond's disease
• immunosuppressive therapy