Treatment of early-onset Gorham syndrome with 8-year follow-up.
Identifieur interne : 004B29 ( Ncbi/Curation ); précédent : 004B28; suivant : 004B30Treatment 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 ; Gérard LoretteSource :
- Pediatric dermatology [ 1525-1470 ]
Descripteurs français
- KwdFr :
- Enfant, Enfant d'âge préscolaire, Fractures du fémur (), Fractures du fémur (anatomopathologie), Fractures du fémur (imagerie diagnostique), Hallux (anatomopathologie), Hallux (imagerie diagnostique), Humains, Lymphangiome (anatomopathologie), Lymphangiome (imagerie diagnostique), Lymphoedème (anatomopathologie), Mâle, Nourrisson, Nouveau-né, Ostéolyse essentielle (), Ostéolyse essentielle (anatomopathologie), Ostéolyse essentielle (imagerie diagnostique), Radiographie, Âge de début, Études de suivi.
- MESH :
- anatomopathologie : Fractures du fémur, Hallux, Lymphangiome, Lymphoedème, Ostéolyse essentielle.
- imagerie diagnostique : Fractures du fémur, Hallux, Lymphangiome, Ostéolyse essentielle.
- Enfant, Enfant d'âge préscolaire, Fractures du fémur, Humains, Mâle, Nourrisson, Nouveau-né, Ostéolyse essentielle, Radiographie, Âge de début, Études de suivi.
English descriptors
- KwdEn :
- Age of Onset, Child, Child, Preschool, Femoral Fractures (diagnostic imaging), Femoral Fractures (pathology), Femoral Fractures (surgery), Follow-Up Studies, Hallux (diagnostic imaging), Hallux (pathology), Humans, Infant, Infant, Newborn, Lymphangioma (diagnostic imaging), Lymphangioma (pathology), Lymphedema (pathology), Male, Osteolysis, Essential (diagnostic imaging), Osteolysis, Essential (pathology), Osteolysis, Essential (surgery), Radiography.
- MESH :
- diagnostic imaging : Femoral Fractures, Hallux, Lymphangioma, Osteolysis, Essential.
- pathology : Femoral Fractures, Hallux, Lymphangioma, Lymphedema, Osteolysis, Essential.
- surgery : Femoral Fractures, Osteolysis, Essential.
- Age of Onset, Child, Child, Preschool, Follow-Up Studies, Humans, Infant, Infant, Newborn, Male, Radiography.
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.
DOI: 10.1111/j.1525-1470.2011.01712.x
PubMed: 22380698
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pubmed:22380698Le document en format XML
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<profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Age of Onset</term>
<term>Child</term>
<term>Child, Preschool</term>
<term>Femoral Fractures (diagnostic imaging)</term>
<term>Femoral Fractures (pathology)</term>
<term>Femoral Fractures (surgery)</term>
<term>Follow-Up Studies</term>
<term>Hallux (diagnostic imaging)</term>
<term>Hallux (pathology)</term>
<term>Humans</term>
<term>Infant</term>
<term>Infant, Newborn</term>
<term>Lymphangioma (diagnostic imaging)</term>
<term>Lymphangioma (pathology)</term>
<term>Lymphedema (pathology)</term>
<term>Male</term>
<term>Osteolysis, Essential (diagnostic imaging)</term>
<term>Osteolysis, Essential (pathology)</term>
<term>Osteolysis, Essential (surgery)</term>
<term>Radiography</term>
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<keywords scheme="KwdFr" xml:lang="fr"><term>Enfant</term>
<term>Enfant d'âge préscolaire</term>
<term>Fractures du fémur ()</term>
<term>Fractures du fémur (anatomopathologie)</term>
<term>Fractures du fémur (imagerie diagnostique)</term>
<term>Hallux (anatomopathologie)</term>
<term>Hallux (imagerie diagnostique)</term>
<term>Humains</term>
<term>Lymphangiome (anatomopathologie)</term>
<term>Lymphangiome (imagerie diagnostique)</term>
<term>Lymphoedème (anatomopathologie)</term>
<term>Mâle</term>
<term>Nourrisson</term>
<term>Nouveau-né</term>
<term>Ostéolyse essentielle ()</term>
<term>Ostéolyse essentielle (anatomopathologie)</term>
<term>Ostéolyse essentielle (imagerie diagnostique)</term>
<term>Radiographie</term>
<term>Âge de début</term>
<term>Études de suivi</term>
</keywords>
<keywords scheme="MESH" qualifier="anatomopathologie" xml:lang="fr"><term>Fractures du fémur</term>
<term>Hallux</term>
<term>Lymphangiome</term>
<term>Lymphoedème</term>
<term>Ostéolyse essentielle</term>
</keywords>
<keywords scheme="MESH" qualifier="diagnostic imaging" xml:lang="en"><term>Femoral Fractures</term>
<term>Hallux</term>
<term>Lymphangioma</term>
<term>Osteolysis, Essential</term>
</keywords>
<keywords scheme="MESH" qualifier="imagerie diagnostique" xml:lang="fr"><term>Fractures du fémur</term>
<term>Hallux</term>
<term>Lymphangiome</term>
<term>Ostéolyse essentielle</term>
</keywords>
<keywords scheme="MESH" qualifier="pathology" xml:lang="en"><term>Femoral Fractures</term>
<term>Hallux</term>
<term>Lymphangioma</term>
<term>Lymphedema</term>
<term>Osteolysis, Essential</term>
</keywords>
<keywords scheme="MESH" qualifier="surgery" xml:lang="en"><term>Femoral Fractures</term>
<term>Osteolysis, Essential</term>
</keywords>
<keywords scheme="MESH" xml:lang="en"><term>Age of Onset</term>
<term>Child</term>
<term>Child, Preschool</term>
<term>Follow-Up Studies</term>
<term>Humans</term>
<term>Infant</term>
<term>Infant, Newborn</term>
<term>Male</term>
<term>Radiography</term>
</keywords>
<keywords scheme="MESH" xml:lang="fr"><term>Enfant</term>
<term>Enfant d'âge préscolaire</term>
<term>Fractures du fémur</term>
<term>Humains</term>
<term>Mâle</term>
<term>Nourrisson</term>
<term>Nouveau-né</term>
<term>Ostéolyse essentielle</term>
<term>Radiographie</term>
<term>Âge de début</term>
<term>Études de suivi</term>
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<front><div type="abstract" xml:lang="en">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>
</front>
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