Retroperitoneal fibrosis. Demographic, clinical and pathological findings

To describe the clinical characteristics of patients with retroperitoneal fibrosis including the etiopathogenesis, clinical features, diagnostic criteria and effective treatment of the disease.

This study was carried out at Princess Basma Teaching Hospital, Irbid, Jordan, during the year 2001. Records of 15 patients with retroperitoneal fibrosis were retrospectively reviewed. Demographic, clinical and pathological criteria was evaluated and analyzed.

From 1990 to 2000, there were 15 patients seen with retroperitoneal fibrosis (10 men and 5 women; the mean age was 55.8 years, ranging between 39 years to 75 years). Nine cases were idiopathic and 6 were secondary. Risk factors of the idiopathic group were cigarette smoking (6 patients) and arterial hypertension (4 patients). Etiologic factors of the secondary group were aortic abdominal aneurysm (3 cases including one following abdominal aortic surgery), radiation therapy for seminoma (one case), ergot-derivative treatment (one case), retroperitoneal non-Hodgkin lymphoma (one case). Main presentations were abdominal or back pain, or both. Treatment performed was medical in 5 cases, medical and endourological in 3 cases and 7 patients received surgical treatment. Medical treatment induced remission in 3 patients, while endourological and surgical treatment resulted in complete recovery in 9 patients (including the 2 patients who failed medical treatment). In the remaining 3 patients treatment failed and the disease relapsed.

Awareness of this rare disorder is important to prevent misdiagnosis. Retroperitoneal fibrosis remains a difficult therapeutic challenge. Aggressive medical, endourological or surgical treatment, or both are equally good modalities for its treatment.