Lymphedema

from Wikipedia, the free encyclopedia
Classification according to ICD-10
I89.0 Lymphedema, not elsewhere classified
I97.2 Lymphedema after mastectomy
elephantiasis
Q82.0 Hereditary lymphedema
ICD-10 online (WHO version 2019)

The lymphedema is a visible and palpable fluid accumulation in the interstitium (intercellular space). It is caused by mechanical insufficiency of the lymphatic system , so that the interstitial fluid can no longer be sufficiently transported away via the lymphatic vessels . This leads to a back pressure and to the accumulation of fluid in the intercellular spaces ( edema ). In addition to the extremities , the face, neck, torso, and genitals can also be affected. Lymphedema is a symptom description, not a diagnosis.

Lymphedema in a woman's right lower leg after hysterectomy.

Stages of lymphedema

  • Stage 0, latency stage (subliminal edema): no symptoms appear yet.
  • Stage 1, reversible stage: protein-rich edema, only a few, small, locally limited fibrosclerotic tissue changes. The edema is still doughy and soft, a “dent” can still be easily made with the finger. Elevating the edema (if possible) reduces the swelling to some extent. The Stemmersche sign is positive (usually).
  • Stage 2, spontaneously irreversible stage: In this stage, fibrosclerotic changes and fat tissue proliferation take place. The edema is hard and no longer reacts to swelling when it is elevated. No or only a very shallow dent can be made in the skin with the finger.
  • Stage 3, elephantiasis : extensive fibrosclerotic changes as well as fatty tissue proliferation have taken place. The affected part of the body may be swollen to the point of shapelessness. The swelling severely restricts mobility. The skin is prone to vesicles and fistulas , eczema and poorly healing wounds .

Etiological classification

The video describes the pathomechanism of lymphedema (English with German subtitles).

Lymphedema is to be differentiated from lipedema and lipohypertrophy in the differential diagnosis . A distinction is made between the primary and the secondary form of lymphedema.

  • In the primary form (rare), the lymph vessels and / or lymph nodes are not or only partially or with a malformation due to a developmental disorder. Entire extremities or body regions can be affected here. The complete lack of lymphatic vessels in an entire region of the body is incompatible with life and leads to death in the womb or shortly after birth. There are also congenital forms (hereditary lymphedema), s. Milroy disease and Meige disease , formerly known as Nun-Milroy-Meige syndrome .

Diagnosis

Stemmer's sign
  • Stemmer's sign : If a fold of skin over the 2nd and 3rd toe cannot be lifted off at all or only with great difficulty, this is a clear sign of lymphedema. The same goes for arms and hands. One speaks of a positive Stemmer's sign. In the further course of the process, the toes will thicken with an almost four-sided appearance.
  • Ultrasound (duplex sonography): The changes in the skin and subcutaneous tissue can be precisely assessed with an ultrasound examination. It provides information about whether it is a disease of the lymph vessels or the veins.
  • Lymphography / lymph scintigraphy

therapy

The therapy of choice is complex physical decongestive therapy (KPE). This includes:

  • Manual lymphatic drainage : Manual lymphatic drainage is a special drainage technique with which the lymphatic tissue is softened and the congested lymphatic fluid is transported towards the abdomen and chest without increasing the blood flow. The skin and the subcutaneous fatty tissue are drained with different pressures. The therapist uses special grips to stimulate the lymphatic vessels to move, thereby promoting the transport of the lymph. With consistent use - depending on the severity once or several times a week - the edema volume is reduced. The therapeutic effect lasts for about 24 hours. Therefore, compression treatment must also be carried out.
  • Mechanical lymph drainage: Special lymph pumps are connected to leg boots or arm cuffs. The cuffs, which have several different air chambers, are then pressurized. The lowest chamber has the highest pressure. A cycle then begins in which the chambers are inflated sequentially.
  • Skin care
  • Compression bandage / compression stockings : Compression bandages are used to wrap the affected arms or legs. The external pressure supports the removal of the lymph fluid and thus the reduction of the lymph congestion. In addition to compression bandages, professional bandaging also includes fleece pads and foam sheets for cushioning. When the tendency to swell decreases, the bandages can be replaced by compression sleeves or stockings. These are specially made gloves, compression sleeves or foot caps and leg stockings made of sturdy flat knit material that are only suitable for the treatment of lymphedema and not for venous diseases.
  • Special movement therapy in compression: regular movement exercises promote the lymph flow and reduce the lymphatic congestion. Compression bandages or stockings are worn to increase the effectiveness of the unblocking exercises. The therapist develops a suitable exercise program for the patient. a. depends on where the lymphedema is.

Furthermore, lymph drainage can be restored within the framework of super microsurgery, thereby permanently reducing lymphatic water retention.

Diuretics (water drugs) are not the only therapeutic option . Treatment with diuretics alone initially and primarily reduces the volume of vascular fluid. The oncotic suction of the plasma is only built up briefly and does not positively change the protein and protein concentration of the edema. So you will see a yo-yo effect. In order to obtain the desired effect of the diuretic, the diuretic will have to be administered continuously and in increasing concentrations. It should also be noted that a diuretic impairs the lymphatic transport mechanism.

Precautions

All activities that prevent the development of lymphedema are understood as precautionary measures. Any injury to the affected arm / leg must be avoided at all costs. This includes avoiding injections, blood draws, mosquito bites, sunburn, etc. Even constricting clothing can trigger lymphedema. The following applies to this disease: "Once lymphedema, always lymphedema". All the more reason to pay attention to the rules of conduct and precautionary measures.

Web links

Wiktionary: Lymphedema  - explanations of meanings, word origins, synonyms, translations

literature

Individual evidence

  1. ↑ Lymphedema: The Disease After Cancer , on spiegel.de from December 17, 2014. Accessed November 21, 2015.
  2. ↑ Erika Mendoza, Tobias Hirsch: "Further education: Lipedema, Lymphedema, Lipohypertrophie", in: The doctor , 69th year, issue 5/2020 of May 5, 2020, pp. 54–59.
  3. ↑ Milroy disease. In: Orphanet (Rare Disease Database).
  4. ^ Meige disease. In: Orphanet (Rare Disease Database).