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Serial lymphoscintigraphic findings in a patient with Gorham's disease with lymphedema.

Identifieur interne : 001192 ( PubMed/Curation ); précédent : 001191; suivant : 001193

Serial lymphoscintigraphic findings in a patient with Gorham's disease with lymphedema.

Auteurs : F. Baulieu ; G. De Pinieux ; A. Maruani ; L. Vaillant ; G. Lorette

Source :

RBID : pubmed:25420304

Descripteurs français

English descriptors

Abstract

Gorham's disease is a rare disorder characterized by vascular, "lymphangio- matous" and/or "hemangiomatous" lesions in bone and surrounding soft tissues. Associated lymphedema has not been reported and clinical evolution is unpredictable. Plain radiographs, CT, MRI, and occasionally bone scintigraphy, are used to detect the bone and soft tissues changes. Biopsy is a major component of the diagnostic process. We report the findings of serial lymphoscintigraphy in a young boy with a polyostotic Gorham's disease associated with lymphangioma of the thigh and lower limb lymphedema. In this patient, lymphoscintigraphy was useful for diagnosis and follow-up of primary lymphedema. It provided valuable information concerning the occurrence, location, and progression of lymphatic lesions in both bone and soft tissues.

PubMed: 25420304

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pubmed:25420304

Le document en format XML

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<name sortKey="Maruani, A" sort="Maruani, A" uniqKey="Maruani A" first="A" last="Maruani">A. Maruani</name>
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<name sortKey="Vaillant, L" sort="Vaillant, L" uniqKey="Vaillant L" first="L" last="Vaillant">L. Vaillant</name>
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<div type="abstract" xml:lang="en">Gorham's disease is a rare disorder characterized by vascular, "lymphangio- matous" and/or "hemangiomatous" lesions in bone and surrounding soft tissues. Associated lymphedema has not been reported and clinical evolution is unpredictable. Plain radiographs, CT, MRI, and occasionally bone scintigraphy, are used to detect the bone and soft tissues changes. Biopsy is a major component of the diagnostic process. We report the findings of serial lymphoscintigraphy in a young boy with a polyostotic Gorham's disease associated with lymphangioma of the thigh and lower limb lymphedema. In this patient, lymphoscintigraphy was useful for diagnosis and follow-up of primary lymphedema. It provided valuable information concerning the occurrence, location, and progression of lymphatic lesions in both bone and soft tissues.</div>
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