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Treatment of EARLY-ONSET Gorham Syndrome with 8-Year Follow-Up


Address correspondence to Annabel Maruani, M.D., Ph.D., Department of Dermatology, Trousseau Hospital, CHRU Tours, Avenue de la République, 37044 Tours Cedex 9, France, or e-mail:


Abstract:  A case of Gorham disease with several years of follow-up is reported. At birth he had a mass in the thigh which was had pathology demonstrating a lymphangioma. By age 3 years, he had lymphedema of the ipsilateral foot and discrepant leg lengths. Radiography revealed heterogenous dystrophy of the bones and osteolysis of the hallux. At age 6, a spontaneous fracture of the right tibia was treated with surgery. Histopathology of a bone sample demonstrated bone remodelling, fibrous tissue, and large vascular lacunas within bone tissue, bordered by cells expressing the lymphaticmarker D2–40. At 8 years old, lymphedema of the right inferior leg had increased, leg lengths still differed, but other clinical signs were absent.