title: 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 D2-40. At 8 years old, lymphedema of the right inferior leg had increased, leg lengths still differed, but other clinical signs were absent.
authors.affiliation: Departments of Dermatology Pediatric Orthopaedic Surgery Pathology, and Nuclear Medicine, University François Rabelais Tours CHRU Tours Inserm U930, Tours, France.
authors.forename: Annabel
authors.initials: A
authors.lastname: Maruani
authors.forename: Sylvie
authors.initials: S
authors.lastname: Thimon
authors.forename: Benoît
authors.initials: B
authors.lastname: de Courtivron
authors.forename: Gonzague
authors.initials: G
authors.lastname: de Pinieux
authors.forename: Françoise
authors.initials: F
authors.lastname: Baulieu
authors.forename: Laurent
authors.initials: L
authors.lastname: Machet
authors.forename: Gérard
authors.initials: G
authors.lastname: Lorette
mesh_terms: D017668:Age of Onset; D002648:Child; D002675:Child, Preschool; D005264:Femoral Fractures / Q000000981:diagnostic imaging / Q000473:pathology* / Q000601:surgery; D005500:Follow-Up Studies; D006214:Hallux / Q000000981:diagnostic imaging / Q000473:pathology*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008202:Lymphangioma / Q000000981:diagnostic imaging / Q000473:pathology*; D008209:Lymphedema / Q000473:pathology*; D008297:Male; D010015:Osteolysis, Essential / Q000000981:diagnostic imaging / Q000473:pathology* / Q000601:surgery; D011859:Radiography