Avigdor Zelikowski

The human lymphatic system has two main functions:
a) Transport of proteins from the tissue to the veins through a large network of vessels and lymph nodes.
b) Immunological.
Both functions are still not fully understood.
Lymphedema is a swelling of soft tissues resulting in increased quantity of lymph due to absence (congenital or acquired) of lymphatic vessels. Usually lower upper limbs are affected.
There is no cure for lymphedema, and thus treatments are aimed at reducing the volume of the limbs, maintaining the results, avoiding infections and affording a better quality of life to the patient.
Until the 1970’s, antibiotics, elevation of the limbs and elastic support were the only therapies that existed.
At the beginning of the 1970’s the first pneumatic device appeared, consisting of one air cell that pressed the leg intermittently. In the beginning of 1980 we invented and introduced a new multichambered sequential pneumatic device. Its new technology created an intermittent milking effect on the limb, which made this device very effective in reducing the lymphedema.
During the last ten years, manual lymph drainage has also become a popular and effective treatment for lymphedema. The purpose of this article is to present our experience with our multichambered sequential pneumatic device during the last twenty years, and its new technology which makes it a very powerful tool in the treatment of lymphedema.

Lymphatic system - anatomy and physiology

Lymphedema is a swelling of soft tissues (usually in the limbs) resulting in increased quantity of lymph due to hypoplasia of the lymphatic system (congenital) or due to trauma to the lymphatic vessels (acquired). The function of the lymphatic system begins with the lymphatic capillaries that collect fluid and proteins from the extra-vascular spaces. This intermediate lymphatic system is necessary because proteins cannot be reabsorbed by the capillary veins. The beginning of the lymphatic system is permeable due to absence of a basement membrane beneath the lymphatic endothelial cells. The lymphatic capillaries are found beneath the epidermis in the superficial dermis. These vessels drain into valved channels in the deep dermis and subdermal tissue, forming larger channels that go to the regional lymph nodes where the contact with the immune system is made.
Efferent lymphatic vessels are drained into larger vessels into the thoracic duct, which is drained into the subclavian vein. Central lymphatic flow is promoted by the lymphatic valves, muscular contractions in larger ducts, respiration and arterial pulsation.

Lymphedema

Insufficiency of the lymphatic system results in an increased quantity of lymph, which causes the swelling of soft tissues known as lymphedema. The main reason for this insufficiency is scarceness of lymphatic vessels due to a congenital aplasia or acquired damage to the lymphatic vessels.
Congenital lymphedema appears in three stages of life: · Hereditary or familial ( Milroy’s disease)
· Lymphedema praecox (age of puberty)
· Lymphedema tarda ( until age of thirty )
Acquired (secondary lymphedema) can result from the following:
· malignant occlusion
· surgical removal of lymph nodes
· Intra-abdominal pressure from tumors
· Radiotherapy
· Recurrent erysipelas (cellulitis)
· Filariasis
· Trauma to soft tissue

Diagnosis of lymphedema:

The presence of bilateral dependent “pitting” edema usually indicates a renal or cardiac etiology. Hypoproteinemias, liver cirrhosis and protein losing enetropathy and allergies can be etiologies for bilateral edema. Venous diseases, mainly chronic venous insufficiency, can be a cause of a unilateral edema. Lymphedema is characteristically firm and rubbery but not pitting. Lymph vesicles may be present in advanced cases.
Stemmer’s sign (holding the skin between the toes and feeling the width of skin compared to the other foot) is very helpful (1).

Lymphatic visualization:
Lymphography is not in use today due to its technical difficulties and danger of causing further damage to the delicate lymphatic system.
Isotopic Lymphography is a useful examination which demonstrates the clearance of human serum albumin labeled with radioactive iodine or technetium 99m colloid from an injected area in the foot and can also demonstrate grossly the lymphatic system and the lymph glands of the groin abdomen or axilla (2).

Treatment of lymphedema

Surgical treatment:
This can be divided into two groups: Excisional procedures and lymphaticovenous anastomosis. The historical excisional operations Kondoleon, Sistrunk and Thompson were abandoned because of the incorrect assumption that the deep fascia acted as a barrier to lymphatic drainage. Charles’ operation, which was based on wide excision of lymphedematous tissue followed by skin grafting, was abandoned due to severe distortion of the leg due to recurrent infections of the transplanted skin area.
Partial debulking of the lymphatic tissue in elephantiasis cases, forming a normal shape to the leg with continuation of the conservative treatment, is the preferable excisional technique today (3). The most logical, albeit technically demanding, approach has been directed to establishing lymphaticovenous anastomosis mode by Nielubowicz by anastomosing divided lymphatic gland to a vein in the groin (4). Direct lympho-venous anastomosis was developed by Cardeiro and Degmi (5,6). Those techniques are suitable for a small group of patients. Immediate results are good but after a relatively short time the lymphatic vessels become fibrotic due to fibrosis of the lymph glands and the lymphatic vessels and lymphedema returns.

Conservative treatment:
Until the 1960’s, elevation of the limb and firm elastic bandaging of the affected limb was the only treatment for reducing the limb volume. Van Der Molen introduced the use of a rubber tube that was pressed around the limb (7). At this time the first pneumatic device appeared. This device consisted of an air compressor connected to a single air cell boot, which was inflated and deflated every 3 minutes (8).
At the end of the 1970’s, we developed a new multichambered sequential pneumatic device (MSPD) (Fig. 1).
This device contains twelve independent, overlapping air cells per limb that are inflated sequentially, creating a milking pressure wave on the limb. The inflation deflation cycle was short - 25 seconds - and high pressures of 80 mmHg to 120mmHg were used (9,10,11). Results were achieved very rapidly.
There was an enormous initial reduction in the lymphedema, which has as yet not been described. The results were maintained by firm elastic support for the lower limbs and by permanent daily use of the MSPD for the upper limbs (since a high-pressure support cannot be used on the upper limb).
Many articles were written about this method of treatment, including its use on upper and lower limb lymphedema and it use in reducing huge lymphedema before surgery (9,10,11,12,13,14,15). Over the years new technical modifications were made, and a new electronic multichambered sequential pneumatic device was developed.
Characteristics of the “Computerized multichambered sequential pneumatic device” ( CMSPD): The CMSPD was developed as a new concept in lymphedema treatment, and is specifically designed for professional use in hospitals and clinics (Fig.2).
Its computer technology provides the physician or therapist with a fully programmable treatment system that allows selection from a variety of treatment cycles. All treatment cycles are preceded by an innovative pre-therapy cycle that works in accordance with the principles of manual lymph drainage in order to prepare the proximal areas to accept the excess lymphatic fluid that will be mobilized during the regular treatment cycles.
This pre-therapy encourages the lymphatic fluid to flow from the limb towards the torso. The CMSPD treatment also includes the regular sequential cycle of the MSPD, as well as two different types of peristaltic compression which have been especially designed for treatment of venous disorders and lymphedema with focal discomfort, as treatment pressure is never applied to the entire limb at one time. The device is operated independently or by means of a PC, which provides data management features to assist the practitioner in storage and retrieval of patient data.

Mode of treatment with the MSPD:
Upper limb lymphedema:
First treatment is usually to eliminate lymphedema from the arm completely, and to follow the rate of its recurrence after 4-8 hours of treatment with the MSPD, with pressure set between 60 to 80 mmHg (depending on the volume of the arm). Elastic support is essential between treatments.
The need for maintenance treatments with the MSPD depends on the recurrence rate of the lymphedema. Upper limb lymphedema needs repeated treatments because high-pressure elastic support cannot be used on the arm (Figs. 3a, 3b).
Lower limb lymphedema: Usually one treatment with the MSPD is needed, which continues until the lymphedema disappears. The length of the treatment depends on the leg diameter. It can take from several hours to several days (the device should work continuously). Recommended pressures are between 80-120 mmHg. If lymphedema is huge it is possible to reduce the surplus skin and subcutaneous tissue by surgery, forming a normal shape of the leg, and maintaining these results by means of firm elastic support (11) (Figs. 4a, 4b, 4c).
In cases with mild to large lymph edematous legs, no surgery is needed and after the treatment with the MSPD, results are maintained by means of firm elastic support and walking exercises (Figs. 5a, 5b).

Manual Lymph Drainage:
During the last ten years, manual lymph drainage has become popular as a method for reducing Lymphedema. This method reduces the Lymphedema by manual massage performed by physiotherapists, involving several treatments, which can be repeated, and maintaining the results by means of firm elastic support (16). This method is a good one but obligates patients receiving treatments to be dependent on their therapists, and in cases of huge Lymphedema it takes a long time to reduce the volume of limb.

In summary:
Lymphedema is still today a major therapeutic problem, and no cure has been found for this pathology. Treatments exist to reduce volume of the limb and to avoid infection, and by this giving patients a better quality of life. The best two methods existing are the MSPD and manual lymph drainage.
Combining both techniques will most probably give even better results. In both methods, trained people in the lymphological field should be the ones to administer treatment.

Avigdor Zelikowsky
Primario, Reparto di Chirurgia Vascolare,
Scuola di Medicina Rabin Medical Center,
Beilinson Campus
Università di Tel Aviv - Israele