Severe Lower Limbs Lymphedema of Late Onset Revealing Polysplenia Syndrome: A Case Report

Polysplenia syndrome includes a group of congenital abnormalities involving the spleen, gastrointestinal tract, liver, and cardiovascular system. A case of severe lower limbs lymphedema occurring in a young woman with polysplenia, azygous continuation of the inferior vena cava, short pancreas, and p...

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Bibliographic Details
Published in:Angiology 2001-06, Vol.52 (6), p.421-423
Main Authors: Granel, Brigitte, Serratrice, Jacques, Juhan, Valérie, Champsaur, Pierre, Weiller-Merli, Claude, Pache, Xavier, Swiader, Laure, Disdier, Patrick, Weiller, Pierre-Jean
Format: Article
Language:English
Subjects:
Abnormalities, Multiple - diagnosis
Adult
Biological and medical sciences
Blood and lymphatic vessels
Cardiology. Vascular system
Cardiovascular System - physiopathology
Chronic Disease
Diagnosis, Differential
Digestive System Abnormalities
Diseases of the lymphatic vessels
Female
Humans
Leg
Liver - abnormalities
Lymphedema - etiology
Mediastinum - abnormalities
Medical sciences
Portal Vein - abnormalities
Severity of Illness Index
Spleen - abnormalities
Syndrome
Tomography, X-Ray Computed
Vena Cava, Inferior - abnormalities
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Summary:Polysplenia syndrome includes a group of congenital abnormalities involving the spleen, gastrointestinal tract, liver, and cardiovascular system. A case of severe lower limbs lymphedema occurring in a young woman with polysplenia, azygous continuation of the inferior vena cava, short pancreas, and preduodenal portal vein is reported. Lower limb lymphedema could represent a new clinical manifestation associated with polysplenia syndrome. Lymphedema could be the result of a primary anomaly of the lymphatic system or be induced by high pressure in the venous system or by compression of the lymphatic circulation by the hypertrophic azygous vessels in the posterior mediastinum.
ISSN:0003-3197
1940-1574
DOI:10.1177/000331970105200608