PEDIATRICS Vol. 78 No. 5 November 1986, pp. 936-938
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Steroid Therapy as Treatment for Idiopathic Fibrosis of the Retroperitoneum and Mediastinum

CHARLES FEILD MD1, WATSON ARNOLD MD1, ELIZABETH S. GLOSTER MD1, GREG SHARP MD1, and JOHN REDMAN MD1

1 Departments of Pediatrics, Urology, and Pathology, University of Arkansas College of Medicine, Arkansas Children's Hospital, Little Rock

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.

Submitted on December 27, 1985
Accepted on March 4, 1986