Lymphedema is an edema of a leg due to lymphatic hypoplasia (primary) or due to relocation or interruption (secondary) lymphatic vessels (overview of the lymphatic system) .. Symptoms are brown, fibrous edema in one or more extremities. The diagnosis is made because of the physical examination. The treatment consists of physical training, clothing for increasing the pressure gradient, massage and sometimes surgery. A cure is unusual, but the treatment can reduce symptoms, slow the progression and prevent complications. The patients have an increased risk of cellulitis, lymphangitis and rarely Lymphangiosarkome.
Lymphedema is an edema of a leg due to lymphatic hypoplasia (primary) or due to relocation or interruption (secondary) lymphatic vessels (overview of the lymphatic system) .. Symptoms are brown, fibrous edema in one or more extremities. The diagnosis is made because of the physical examination. The treatment consists of physical training, clothing for increasing the pressure gradient, massage and sometimes surgery. A cure is unusual, but the treatment can reduce symptoms, slow the progression and prevent complications. The patients have an increased risk of cellulitis, lymphangitis and rarely Lymphangiosarkome. Etiology Lymphedema may be primary: By lymphatic hypoplasia secondary: Due to a relocation or interruption of the lymphatic vessels Primary lymphedema Primary lymphedema are innate and rare. They vary in phenotype and age of the patient at the presentation. A congenital lymphedema occurs before the age of two and is due to a lymphatic aplasia or hypoplasia. The Milroy’s disease is an autosomal dominant familial form of congenital lymphedema, the mutations of the vascular endothelial growth factor receptor-3 (VEGFR-3) gene is assigned and is sometimes associated with cholestatic jaundice, edema and diarrhea, which as a result of protein-losing enteropathy arises due to an intestinal lymphangiectasia. Lymphoedema praecox occurs between 2 and 35 years of age in appearance, typically in women at the beginning of menstrual periods or during pregnancy. The Meige’s disease is an autosomal dominant familial form of lymphedema praecox, the mutations in the gene for the transcription factor (FOXC2) is associated with particular eyelashes (Distichiasis), cleft palate and edema in the leg, arm and sometimes associated on the face is. Lymphoedema tarda occurs from the age of 35. It occur familial and sporadic forms, the genetic cause of both is unknown. Clinical findings are similar to those of lymphedema praecox, but may be less severe. Lymphedema is prominent in some other genetic syndromes, including Turner syndrome yellow nail syndrome is characterized by pleural effusion and yellow nails Hahnenkamm syndrome is a rare congenital syndrome characterized by intestinal and other lymphangiectasia, facial malformations and mental retardation is , Secondary lymphedema Secondary lymphedema is much more common than primary. The most common causes are surgery (especially lymph node dissection, typical of the treatment of breast cancer) Radiation therapy (especially armpit or groin) Trauma lymphatic congestion caused by a tumor lymphatic filariasis (in developing countries) Mild lymphedema can also by a Lymphleck in the interstitial tissue in chronic venous insufficiency arise. Symptoms and discomfort symptoms of secondary lymphedema include painful discomfort and a feeling of heaviness and fullness. The main symptom is the soft tissue, which is divided into three levels of severity. In stage I, the edema is dented and the affected area is often in the morning back to normal. In stage II, the edema is nichtgedellt and chronic soft tissue inflammation leads to an early fibrosis. In stage III, the edema is brownish and irreversibly, mainly due to a Weichteilfibrosierung. The swelling is usually unilateral, it can get worse in warm weather, before menstruation and after a long period with drooping limb. It can affect any part of the extremity concern (isolated proximal or distal) or even the whole extremity; it can limit the movement, if the swelling is periarticular present. Disability and emotional discomfort can be significant, v. a. if the Lymphedema is caused by a medical or surgical treatment. Skin lesions are common and include hyperkeratosis, hyperpigmentation, warts, papillomas and fungal infections. In rare cases, an affected limb is extremely large, and the hyperkeratosis is hard, giving the appearance of an elephant skin (elephantiasis). This phenomenon is more common in filariasis than in other causes of lymphedema. Complications may develop lymphangitis, most often when bacteria through skin cracks between the toes, for. As for fungal infections, or cuts penetrate in hand. Lymphangitis is almost always caused by streptococci that cause erysipelas due; sometimes it is also caused by staphylococci. The affected limb becomes red and feels hot; red stripes may proximally towards the point of entry to expand starting, lymphadenopathy may develop, often also the skin replaces. Rarely longstanding lymphedema lead to Lymphangiosarcoma (Stewart-Treves syndrome), usually in patients after a mastectomy and in patients with worm diseases. Diagnosis Clinical diagnostic CT or MRI if the cause is not apparent Primary lymphedema are clearly evident by characteristic soft tissue edema throughout the body and other information from the history and physical examination in general. The diagnosis of secondary lymphedema is usually obvious from the physical examination. Additional studies are indicated if a secondary lymphedema is suspected, unless the diagnosis and the cause is obvious. CT and MRI can detect the locations of lymphatic obstruction; a radioactive lymphoscintigraphy can demonstrate lymphatic hypoplasia or slugg-like flow. The progression can be monitored by measuring the circumference of the extremities, by the volume of water displaced by the submerged limb, is measured, or by a skin or Weichteiltonometrie; but these test methods are validated. In developing countries, studies should be carried out on worm diseases. If lymphedema much larger than expected seems to occur (eg. As after a lymph node removal) or after a delay in a treated for breast cancer woman should have a recurrence of cancer are considered. Prognosis If lymphedema is once occurred, a cure is unlikely. Meticulous treatment and possibly preventive measures can alleviate symptoms, slow the progression or stop and prevent complications. Treatment If primary lymphedema sometimes surgical reconstruction mobilization of liquid (eg. As by elevating and compression, massage, pressure bandage, intermittent pneumatic compression) The treatment of the primary lymphoedema, the surgical soft tissue reduction include (removal of the subcutaneous fat and connective tissue) and reconstruction if the quality of life is significantly impaired. The treatment of the secondary lymphedema relates v. a. treating the cause. For the lymphedema itself, various measures can be used to mobilize liquid (complex decongestive therapy). These include. Manual lymph drainage in which the extremity slightly raised and in the direction of the heart by kneading ( “milked”) is. Gradientdruckbandagen or cuffs Hüftübungen massage of the extremities, including intermittent pneumatic compression A surgical soft tissue reduction, Reanastomosierungen lymphatic vessels and the formation of Drainagekanälem are sometimes tried, but have so far not been systematically studied. Preventive measures include avoiding heat, strong physical exertion and constrictive clothing (incl. Blood pressure cuffs) to the affected limb around. Skin and nail care require meticulous attention; Vaccination, phlebotomy and intravenous catheterization of the affected limb should be avoided. Tips and risks Avoid vaccination, blood-letting and i.v. Catheterization in the affected limb lymphedema. Cellulitis and lymphangitis are treated with beta Lactamasefesten antibiotics that effectively are (z. B. with dicloxacillin, cephalexin) against gram-positive organisms.