Lymphedema (overview) I89.00

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

lymphedema

Definition
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Insufficient transport capacity of the lymph vessel system caused by a congenital (primary lymphedema) or acquired (secondary lymphedema) lymphangiopathy, which can no longer or only insufficiently transport the normally occurring lymphatic protein load.

Classification
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Due to its etiopathogenesis, lymphedema is divided into primary lymphedema (rare, mostly genetic) and non-genetic (acquired) secondary lymphedema. The group of non-classifiable, idiopathic lymphedema is listed in the group of genetic lymphedema.

Primary lymphedema: mainly in younger patients. Genetic or sporadic, unilateral or bilateral lymphedema manifesting at birth or in early adolescence due to structural lymphatic vessel abnormalities.

Primary lymphedema can be divided into genetic and idiopathic lymphedema.

Etiopathogenesis
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Genetic, inflammatory or caused by organ insufficiencies. Aetiopathogenetically, the disease is based on an imbalance between lymphatic filtration and the transport capacity of the lymphatic vessels. A distinction is made between the relative imbalance due to impairment of the lymphatic flow(afterload impairment) and the absolute imbalance due to increased filtration (preload impairment) (Wollina U et al. 2018). In the lymphostatic congestion area, congested plasma proteins cause a cellular reaction (macrophages, fibroblasts) and connective tissue proliferation with proliferation of blood vessels. Increased susceptibility to infection in the lymphostatic area can lead to recurrent erysipelas, which in turn cause secondary lymphedema.

Localization
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Preferably on the extremities; less frequently on the head, neck and trunk.

Clinical features
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Extent and heaviness of extremities, thickening of dermis (based on lymphostatic fibrosclerosis) and subcutaneous fatty tissue, verruciform epidermal hyperplasia (papillomatosis lymphostatica), post-inflammatory hyperpigmentation, onychodystrophy, onychogrypose, disturbance of nail growth Development of elephantiasis possible, see table 1.

Laboratory
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CBC, protein, kidney function, electrolytes.

Diagnosis
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  • Anamnesis, clinic, (local findings, skin fold test/ Malleese sign), laboratory.
  • In lymphedema of the lower extremity: Doppler or colour duplex to exclude the involvement of the veins.
  • Extended diagnostics: Indirect lymphography, lymph scintigraphy; if necessary X-ray examination of the thorax, sonography of the abdomen, thorax, neck (lymph node enlargements, tumours?), computer tomography of the abdomen and the upper thoracic aperture; in rare, unclear cases magnetic resonance imaging (in lymphoedema: honeycomb pattern in the subcutis).

Complication(s)
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With chronic lymphedema:

Therapy
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  • The focus is on consistently decongestive therapy!
  • Földish 2-phase treatment:
  • Stabilisation measures under outpatient conditions: after maximum decongestion, prescription and consistent wearing of compression stockings (class III). Made to measure. Fitting in the morning in a slim leg condition; if necessary, incorporation of pads pre- or retromalleolar. Continuation of manual lymphatic drainage, if necessary additional intermittent mechanical compression. Coordination of a long-term treatment plan with patient and family, specific sports activities (cycling, hiking, swimming), treatment of obesity.

    Remember!

    Putting 2 pairs of compression stockings of compression stockings class II on top of each other corresponds to pressure class III!

Notice! The sole use of intermittent lymphatic drainage equipment (see below, pneumatic intermittent compression) is not recommended. It is only useful as a supplement to manual lymphatic drainage! Beforehand, apply to your health insurance company for the prescription of an aid for therapy with intermittent compression.

  • Inpatient treatment phase: Complex physical decongestion therapy with at least one manual lymphatic drainage and compression therapy with short-stretch bandages. If necessary, padding with cotton wool bandages to avoid pressure points. If necessary, additional intermittent pneumatic compression therapy. Nocturnal elevation of the extremity. Physiotherapy.
  • Notice! The use of diuretics for lymphedema is viewed with caution, as diuretics are only effective in low-protein oedema. Otherwise, paradoxical effect by accumulation of protein in the interstitium possible!

  • The following should always be avoided on the affected extremity: injections, infusions, cold and heat treatment, blood sampling, acupuncture, blood pressure measurement, kneading massage, invasive procedures, spreading of the oedema.

Internal therapy
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  • Anticoagulation (5,000-10,000 IU heparin s.c. 2 times/day).
  • If necessary, infection prophylaxis: Intermittent antibiotic therapy (Penicillin G 10 Mega IU for 10 days i.v. every 3 months).

Operative therapie
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Elephantiasis surgery: This is reserved for specialist clinics and is limited to the final stage of individual elephantiasis.

Progression/forecast
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Reductions have also been described in primary lymphedema. However, the prognosis is rather unfavourable. The prognosis for secondary lymphedema is more favourable.

Tables
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Development of lymphedema according to Földi

Phase I

Latent lymphedema, regression within 2 weeks.

Phase II

Reversible lymphedema: Discrete evening swelling of the back of the foot and the ankles, spontaneous improvement by bed rest, elevation.

Phase III

Irreversible lymphedema: Hard, pale, non-penetrable edema, no spontaneous improvement.

Phase IV

Elephantiasis: deformation of the leg to the grotesque disfigurement.

Note(s)
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Intermittent lymphatic drainage devices are commercially available, e.g.
  • Lympha-mat of the company Bösl Medizintechnik GmbH
  • Hydroven from HNE Healthcare.

Literature
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  1. Baltaci M et al (2004) Elastolytic granulomas of the legs in familial visceral lymphangiodysplasia with chronic lymphedema. JDDG 2: 766-769
  2. Cohen SR et al (2001) Lymphedema: strategies for management. Cancer 92: 980-987
  3. Földi E (1985) Pathogenesis of lymphedema. In: Holzmann H, Altmeyer P, Hör G, Hahn K (eds.) Dermatology and Nuclear Medicine. Springer, Berlin Heidelberg New York, S. 395-400
  4. Földi M (1996) The current state of the art of drug-based lymphedema treatment. Phlebology 25: 48-52
  5. Gaber Y (2004) Secondary lymphoedema of the lower leg as an unusual side-effect of a liquid silicone injection in the hip and buttocks. Dermatology 208: 342-344
  6. Hesse G et al (2003) Ultrasound diagnostic techniques in dermatologic angiology and phlebology. dermatologist 54: 614-625
  7. Levinson KL et al (2003) Age of onset in hereditary lymphedema. J Pediatr 142: 704-708
  8. Muehlberger T et al (2001) Etiology, clinical aspects and therapy of penoscrotal lymphedema. Surgeon 72: 414-418
  9. Saaristo A et al (2002) Insights into the molecular pathogenesis and targeted treatment of lymphedema. Ann NY Acad Sci 979: 94-110
  10. Vana J et al (2002) Massive localized lymphedema in an extremely obese patient. surgeon 73: 383-386
  11. Tiwari A et al (2003) Differential diagnosis, investigation, and current treatment of lower limb lymphedema. Arch Surgery 138: 152-161

Disclaimer

Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 29.10.2020