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

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 D240. At 8 years old, lymphedema of the right inferior leg had increased, leg lengths still differed, but other clinical signs were absent.
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Submitted on : Friday, May 29, 2020 - 1:46:07 AM
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Annabel Maruani, Sylvie Thimon, Benoit de Courtivron, Gonzague de Pinieux, Francoise Baulieu, et al.. Treatment of EARLY-ONSET Gorham Syndrome with 8-Year Follow-Up. Pediatric Dermatology, Wiley, 2013, 30 (3), pp.391 - 394. ⟨10.1111/j.1525-1470.2011.01712.x⟩. ⟨hal-02645465⟩



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