Treatment of EARLY‐ONSET Gorham Syndrome with 8‐Year Follow‐Up. Issue 3 (1st March 2012)

• Record Type: Journal Article
• Authors: Maruani, Annabel; Thimon, Sylvie; de Courtivron, Benoît; de Pinieux, Gonzague; Baulieu, Françoise; Machet, Laurent; Lorette, Gérard
• Is Part Of: Pediatric dermatology. Volume 30:Issue 3(2013)
• Page Start: 391
• Page End: 394
• Publication Date: 2012-03-01
• Subjects: Pediatric dermatology -- Periodicals; Children -- Diseases -- Periodicals; 618.925
• DOI: 10.1111/j.1525-1470.2011.01712.x
• Languages: English
• ISSNs: 0736-8046

Abstract

Abstract : 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.