Treatment of EARLY‐ONSET Gorham Syndrome with 8‐Year Follow‐Up
Identifieur interne : 000051 ( Istex/Checkpoint ); précédent : 000050; suivant : 000052Treatment of EARLY‐ONSET Gorham Syndrome with 8‐Year Follow‐Up
Auteurs : Annabel Maruani [France] ; Sylvie Thimon ; Benoît De Courtivron ; Gonzague De Pinieux ; Françoise Baulieu ; Laurent Machet [France] ; Gérard LoretteSource :
- Pediatric Dermatology [ 0736-8046 ] ; 2013-05.
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.
Url:
DOI: 10.1111/j.1525-1470.2011.01712.x
Affiliations:
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<front><div type="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.</div>
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