SERIAL LYMPHOSCINTIGRAPHIC FINDINGS IN A PATIENT WITH GORHAM’S DISEASE WITH LYMPHEDEMA

Abstract

Gorham’s disease is a rare disordercharacterized by vascular, “lymphangiomatous”and/or “hemangiomatous” lesions inbone and surrounding soft tissues. Associatedlymphedema has not been reported andclinical evolution is unpredictable. Plainradiographs, CT, MRI, and occasionally bonescintigraphy, are used to detect the bone andsoft tissues changes. Biopsy is a majorcomponent of the diagnostic process. Wereport the findings of serial lymphoscintigraphyin a young boy with a polyostotic Gorham’sdisease associated with lymphangioma of thethigh and lower limb lymphedema. In thispatient, lymphoscintigraphy was useful fordiagnosis and follow-up of primarylymphedema. It provided valuable informationconcerning the occurrence, location, andprogression of lymphatic lesions in both boneand soft tissues.