Introduction

    Lymphedema is characterised by a chronic swelling of the tissues, resulting from accumulation of lymph fluid. This is caused by insufficient transport and drainage of lymph fluid. Worldwide millions of people suffer from lymphedema. It is a chronic disease which has a high morbidity. Lymphedema can be primary or secundary.


Causes

    Primary lymphedema is caused by a congenital dysplasia of the lymph tissue. The edema can be present a birth (congenital lymphedema), but regularly begins during puberty (lymphedema praecox). It can either begin spontaneously, or be triggered by an exogenous event (i.e. infection, surgery). Primary lymphedema can be the result of mutations in the VEGFR3 gene or the FOXC2 gene. These are genes, that are involved in the development of the lymphatic system during embryogenesis. Primary lymphedema can be hereditary.

    Secundary lymphedema is caused by damaging, obstruction or overload of the (normally developed) lymph tissue. Damaging of the lymph tissue can be caused by infections (erysipelas, filariasis), surgery, radiotherapy, or complicated bone fractures. Obstruction of lymph tissue can be caused by tumours or metastases. Overload of the lymphatic tissue can be a complication of chronic venous insufficiency, in which the amount of fluids exceeds the transport capacity of the lymphatic system. In western countries surgery of malignancies is the most important cause of lymphedema, especially when additionally lymph nodes are surgically removed and/or radiotherapy is applied. Worldwide filariasis is the most frequent cause of lymphedema. This is a disease that is caused by a tropical nematode worm, which is transmitted by blood sucking insects. Filariasis is endemic in tropical regions of Africa, Asia, Central and South America. Persons with lipedema also have a higher risk to develop lymphedema.


Symptoms

    Patients experience pain, swelling, feeling of heaviness, decreased mobility, disability or psychosocial complaints. There is a higher risk to develop erysipelas. The longer lymphedema is present, the more difficult the treatment is. This is due to the fibrosing of the proteins in the lymph fluid. A rare complication of lymphedema is lymphangiosarcoma, which is an aggressive malignancy.


Treatment

    The first phase of the treatment of lymphedema consists of a combination of manual lymphatic drainage, intermittent pneumatic compression, compressive bandaging, and exercise. The goal of this therapy is reduction of swelling.

    Manual lymphatic drainage (MLD) is a specific massage technique, based on the principles of Vodder and Foldi, and intents to increase in lymphatic contractility and increase lymphatic flow through cutaneous lymphatics.         

    In intermittent pneumatic compression a cuff with multi chambers is inflated in a sequential order from the distal part of the limb towards the proximal part. This causes external compression in order to reduce swelling.

    The second phase of treatment consists of wearing elastic stockings (hosiery) in order to prevent relapse of edema and to sustain the reduced limb circumference. During this maintenance phase additional treatment with manual lymphatic drainage and/or intermittent pneumatic compression can be indicated. Skin care is important in order to prevent infections.

    The positive effect of lymphatic-venous shunts has never been demonstrated by scientific studies of good quality. In a recent prospective study, in which 11 lymphatic venous anastomosis procedures in 10 patients with breast cancer related lymphedema were performed, no effect could be measured. (Damstra 2008).


References

    For more information about lymphedema:


  1. -Novel missense mutations in the FOXC2 gene alter transcriptional activity.

  van Steensel MA, Damstra RJ, Heitink MV, Bladergroen RS, Veraart J, Steijlen PM, van Geel M.

  Hum Mutat 2009;30(12):E1002-9.


  1. -Lymphedema after Greater Saphenous Vein surgery.

  Heitink M.V., Schurink G.W.H., de Pont C.D.J.M., van Kroonenburgh M.J.P.G., Veraart J.C.J.M.

  Eur J Vasc Endovasc Surg. 2009;38:656.


  1. -Lymphedema in Prader-Willi syndrome.

   Heitink MV, Sinnema M, van Steensel MA, Schrander-Stumpel CT, Frank J, Curfs LM.

  Int J Dermatol. 2008 Nov;47 Suppl 1:42-4.


  1. -Lymphedema-distichiasis syndrome: a distinct type of primary lymphedema caused by mutations in the FOXC2 gene.

  Vreeburg M *, Heitink MV *, Damstra RJ, Moog U, van Geel M, van Steensel MA.

  Int J Dermatol. 2008 Nov;47 Suppl 1:52-5.


* Both first author.

 
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