眼窩偽腫瘍後発生した後腹膜線維化症の1例 - 全身性線維化症の1型として -

Abstract

A 63-year-old woman was admitted to our hospital with the complaint of an abdominal mass on April 16, 1981. She had been treated for a right orbital pseudotumor five years ago. DIP demonstrated a poorly functioning left kidney. RP revealed left hydronephrosis due to ureteral stellosis at the fourth vertebra. CT disclosed a thickened mass in the retroperitoneal space. Laparotomy performed on May 28, 1981, revealed a hard fibrous mass extending from the promontory to the L3 vertebra. The resected tissue histologically proved to be fibrosis with non-specific inflammatory reactions. With these findings taken into consideration, the final diagnosis of retroperitoneal fibrosis was made. Prednisolone was given at the doce of 30 mg/day for the first four days, 15 mg/day for the following five days; the maintenance dose was 10 mg/day. Following the steroid therapy, the patient has been well with marked urographic improvement. In particular, DIP showed slight improvement of left renal function following steroid therapy for three months. The patient was discharged on June 21, 1981. A second case of retroperitoneal fibrosis following orbital pseudotumor, which was probably a type of “multifocal fibrosclerosis " is presented. This case is discussed with reference to previous cases.