Retroperitoneal fibrosis

Sonographic image of dilatation of the calicopelvic system of the kidney

Retroperitoneal fibrosis is an inflammatory proliferation of the connective tissue of the retroperitoneum, which by its action affects or oppresses the surrounding organs, most often the ureters.

Etiology

We distinguish between primary and secondary fibrosis. The etiology of primary fibrosis (also Ormond disease) is unclear, an autoimmune reaction is assumed. In contrast, the causes of secondary fibrosis are many, for example drugs (methyldopa, phenacetin, beta blockers, LSD), tumors, infections and others.

The disease occurs between the ages of 40 and 60 with a preference for men (2.9:1).

Manifestations and diagnosis

The most common manifestation is external compression of the ureter with a decline in renal function to the point of renal failure. Branches of large vessels or nerves can also be obturated. At the same time, we can observe non-specific symptoms such as nausea, weight loss or subfebrile.

In diagnosis, we use imaging methods (USG, CT and excretory urography) to evaluate the condition of the urinary tract, dilatation of the calicopelvic system and the general condition of the retroperitoneum. We examine renal function and autoimmune antibodies. In case of uncertainty, a biopsy will confirm the diagnosis.

Treatment

The basis of the treatment is the restoration of urine outflow from the kidney, which leads to an improvement in its function. We use ureteral stents or nephrostomy for derivation. The causal treatment of the primary form is immunosuppressants. Another option is surgical release of the ureter.

Links

Related Articles

• Diagnostic imaging methods in retroperitoneum examination

Literature

• SOBOTKA, Roman – HANUŠ, Tomáš. Primární retroperitoneální fibróza. Urologie pro praxi [online]. 2006, vol. 4, p. 156-159, Available from <https://www.urologiepropraxi.cz/pdfs/uro/2006/04/02.pdf>. ISSN 1213-1768. 

• KAŠÍK, Josef. , et al. Primární retroperitoneální fibróza - Morbus Ormond. Urologie pro praxi [online]. 2008, y. 9, vol. 3, p. 128-132, Available from <https://www.urologiepropraxi.cz/pdfs/uro/2008/03/06.pdf>. ISSN 1213-1768.