Steroid therapy as treatment for idiopathic fibrosis of the retroperitoneum and mediastinum

Multifocal idiopathic fibrosis of both mediastinum and retroperitoneum is rare in childhood.1,2 We describe a patient who presented with biopsyproven mediastinal and retroperitoneal fibrosis associated with progressive pulmonary fibrosis, cardiomyopathy, and bilateral ureteropyelocaliectasis; the patient responded to intravenous pulse and oral steroid therapy with relief of his uretenal obstruction and an improvement in pulmonary and cardiac function. Although surgical ureterolysis is generally the management of choice for ureteral obstruction caused by retroperitioneal fibrosis,3,4 steroid pulse therapy and a regimen of oral prednisone may provide an alternative modality of themapy.5-7 CASE REPORT This 12-year-old black boy was seen in the emergency room with a chief complaint of shortness of breath.