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

Identifieur interne : 000180 ( France/Analysis ); précédent : 000179; suivant : 000181

Treatment 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 Lorette

Source :

RBID : ISTEX:F0D1740F52188D686E72072236E3A07D884D7BCC

Descripteurs français

English descriptors

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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ISTEX:F0D1740F52188D686E72072236E3A07D884D7BCC

Le document en format XML

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<term>Femoral Fractures (pathology)</term>
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<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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