Weber-Christian disease

In 1948 Ormond reported two cases of unheralded anuria due to idiopathic retroperitoneal fibrosis. Previous documentation of the condition had been scanty, but since then over a hundred similar cases have been reported. The patient, usually an adult, at first has only vague symptoms such as abdominal discomfort, anorexia, loss of weight, or nausea. The diagnosis is made usually only when signs of ureteric obstruction appear later in the illness. At operation there is mechanical obstruction to the ureters by a mass of exuberant retroperitoneal fibrous tissue. Freeing the ureter from the mass almost always results in relief of the obstruction and cure of the symptoms. The purpose of this paper is to review some aspects of the pathology of this and similar conditions, and to draw attention to evidence which suggests a possible pathogenesis.