Gorham's disease and diffuse lymphangiomatosis in children and adolescents

Abstract

Gorham's disease is a rare disorder of unknown etiology and variable clinical presentation that is characterized by proliferation of thin-walled vascular channels resulting in destruction and resorption of osseous matrix. The condition is frequently under recognized or misdiagnosed. There is no standard treatment defined for this disease. Here we report on eight children diagnosed with Gorham's disease at our institution over a ten-year period. Soft tissue lymphangioma was present in seven and six children had splenic involvement. Disease stabilization and improvement was observed on treatment with interferon alpha-2b and bisphosphonate therapy.