Lymphoedema is an accumulation of lymph in the interstitial spaces caused by a defect in the lymphatic drainage system. Reduced lymph drainage can result from either congenital or acquired anomalies of the lymphatic system. Although lymphoedema usually affects one or more extremities, is can also manifest itself in the organs. Regardless of the pathogenesis, it is often a chronic, intractable condition that becomes a long-term physical and psychological problem for patients and a complex therapeutic challenge for doctors.
In many cases of advanced lymphoedema, we find a typical medical history with a characteristic clinical presentation that enables almost certain diagnosis using basic diagnostics comprising a preliminary case history, inspection and palpation, in which a positive Stemmer’s sign is evidence of the disease. Robert Stemmer showed that the dorsal skin fold at the base of the second toe in healthy patients can be lifted from the bone while this is not possible in patients with distal lymphoedema. A positive Stemmer’s sign is a definite indication of lymphoedema, however if Stemmer’s sign is negative, the patient may still have a proximal lymphoedema. In this case, further examination is necessary. Diagnosis is more difficult in the less severe stages of oedema. Available tests include isotope lymphoscintigraphy, direct and indirect lymphography, fluorescence micro lymphography, magnetic resonance tomography, computed tomography and ultrasound.
We differentiate between three forms of primary lymphoedema:
-Congenital lymphoedema occurs at birth or within the first two years of life.
-Lymphoedema praecox is the most common form of primary lymphoedema and occurs around puberty or in young adults up to age 35.
-Lymphoedema tarda occurs in patients aged 35 or older and requires extreme care in diagnosis to ensure that a secondary lymphoedema caused by a tumour-related obstruction is not overlooked.
Secondary lymphoedema develops as a result of damage to the lymphatic pathways and vessels or lymph nodes or from external compression, surgery or various disease processes.
Chronic lymphoedema often entails complications. In the case of a lymphoedema that has persisted for decades, a variety of neoplastic complications can also arise.
Lymphoedema is a chronic disease that requires lifelong treatment. That does not mean that lymphoedema is incurable. It can only be managed. A great number of treatment options are now available that have efficiently reduced oedemas and prevented fluid accumulation. On the other hand, when treatment is discontinued swelling inevitably increases and the patient’s condition worsens considerably following recurring infection as a result of the massive oedema. In addition, patients may experience severely restricted mobility of their extremities accompanied by physiological effects on their everyday life and even life-threatening infections such as erysipelas or malignant complications.
Complex physical decongestion (CPD) therapy
Complex physical therapy is done in 2 phases:
In phase 1 of decongestion, meticulous hygiene is necessary and particular care must be given to digital mycosis, small wounds and cracks in the skin. In addition, phase 1 includes whole-body manual lymph drainage (MLD) up to 5 times per week in keeping with the principles described by Winiwarter, Vodder und Földi followed by compression bandaging and physiotherapy movement exercises and terrain training.
In phase 2 of decongestion, the frequency of manual lymph drainage can be reduced to 2 times per week in an outpatient setting. The patient is then provided with a compression sleeve that includes the shoulder or a compression pantyhose. Sometimes, outpatient manual lymph drainage may be followed by compression bandaging to further promote decongestion.
The maintenance phase seeks to maintain the results achieved through decongestion.
Low-stretch bandages are used to reduce oedema during the decongestion phase. Low-stretch bandages provide high working pressure and low resting pressure to ensure drainage.
Compression garments make treatment easier for the patient during the maintenance phase since low-stretch bandages are difficult to apply and tend to slip during daily use. Manufacturers have recently made considerable improvements in flat-knit products. Because compression garments lose their compression capacity after 3 to 6 months, they must be replaced accordingly. Lower-grade compression garments provide compression of up to 40 mm Hg while higher-compression garments provide 59 mm Hg or more.
Coumarin has proven to slowly reduce various forms of lymphoedema in several studies conducted on both humans and animals. In some cases, the administration of selenium is being considered as it is thought to have a positive effect in reducing the frequency of recurrent erysipelas. There is no specific diet for lymphoedema. However, patients should reduce consumption of items that increase capillary pressure such as coffee, red wine and sparkling wine. Cutting back on proteins does not improve lymphoedema but rather adds to the problem by causing hypoproteinaemic oedema.
Surgery is done when other customary medical therapies have failed. There are two main surgical interventions, 1) reduction plastic surgery and 2) microsurgical lympholymphatic, lymphovenous or lymph node-venous anastomosis.
using infrared light