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 ca se of severe lower limbs lymphedema occurring in a young woman with polyspl enia, azygous continuation of the inferior vena cava, short pancreas, and p reduodenal portal vein is reported, tower limb lymphedema could represent a new clinical manifestation associated with polysplenia syndrome. Lymphedem a could be the result of a primary anomaly of the lymphatic system or be in duced by high pressure in the venous system or by compression of the lympha tic circulation by the hypertrophic azygous vessels in the posterior medias tinum.