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Changes in clinical management and the evolution of an interdisciplinary therapeutic approach: 640 lymphedema patients over 20 years
(Portuguese PDF version)

José Luis Ciucci¹, Juan Carlos Krapp², Jorge E. Soraccco², Julio Ayguavella², Luis Daniel Marcovecchio², Carmen Salvia², Sandra Gerez², Cristina Felicia², Cleusa Ema Quilici Belczak², Mirta Critelli Isola³, Inés Lamuedra³, Silvina Rosales⁴

1. PhD. Chief of the Service of Phlebology and Lymphology, Hospital Militar Central de Buenos Aires. President of the Escola Argentina de Linfologia (Argentinean Medical Association - AMA). Professor and chief of the Service of Lymphatic and Venous Anatomy, Universidade de Buenos Aires, Buenos Aires, Argentina.
2. Physician and/or Professor, Escola Argentina de Flebologia e Linfologia, AMA, Buenos Aires, Argentina.
3. Psychologist, Hospital Militar Central de Buenos Aires, Buenos Aires, Argentina.
4. Physical therapist, Service of Phlebology and Lymphology, Hospital Militar Central de Buenos Aires, Buenos Aires, Argentina.

Correspondence:
Cleusa Ema Quilici Belczak
Centro Vascular
Av. Tiradentes, 1081
CEP 87013-260 - Maringá, PR
Brazil
E-mail: belczak@wnet.com.br

J Vasc Br 2004;3(1):72-6

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The clinical course of lymphedema is such that a completely successful cure cannot be expected. This represents a challenge to specialists who must be ever conscious that definitive results are unobtainable. Nevertheless, when the correct treatment is given during the initial stages, highly rewarding results can be achieved.^1-4

The chronic nature of lymphedematous patients' lymphatic stasis, with high protein content liquid accumulation results in histopatological changes to the subcutaneous cellular tissues which in turn compromises the skin and the lymph vessels themselves.^5-7

Fibroblasts progressively take the place of normal tissues, forming irreversible collagen with increasing intensity.^6,8,9 There is fibrosis of subcutaneous cellular tissue. Lymph vessels develop morphological and functional abnormalities due to valve malfunction and loss of contractibility.^10-12

Lymphedema, an infirmity that is easy to diagnose and difficult to treat, presents a chronic nature particular to the disease, which involves a range of other concurrent problems. This range of difficulties inspired the formation of an interdisciplinary team to treat all of the patients' problems at a single physical location in a comprehensive manner, thus obtaining better results.^3,13,14

The objective of this paper is to draw attention to current treatments for lymphedema patients and to the way that the approach to treatment has evolved over 20 years at this service.

METHOD

This is a retrospective analysis of the diagnoses of 640 patients and of the evolution of the conduct of their treatment for lymphedema at the Hospital Militar Central in Buenos Aires, during the period between 1980 and 2000. The patients underwent isotope lymphography.

The Fisher test was used for statistical analysis accepting an α a error of 5%, with a significance level of P < 0.05.

Patients were aged from 2 to 89 years; 65% were female, 35% male, which difference is significant (P < 0.0001).

RESULTS

Three hundred and thirty-two (50.3%) of the patients were suffering from upper limb lymphedema and 318 (49.7%) had lymphedema of a lower member. Of this last group, 211 patients had primary lymphedema and 107 secondary (33% and 16.7%, respectively, of the total number of patients), which difference was significant (P < 0.0001).

These patients were treated according to the influence of three schools of thought, during three different periods: the first phase involved the influence of the Brazilian Degni School, Cordeiro and Mayall (1980 to 1989); the second phase was influenced by Földi, Leduc, Casley-Smith (1990 to 1995); the third phase was based on the concept of an interdisciplinary approach (1995 to 2000) developed in-house and it was during this phase that better results were observed.

There was greater prevalence among female patients and upper and lower members were similarly affected across the group.

DISCUSSION

Observe that the experience gained between 1980 and 2000 through the treatment of 640 patients with primary and secondary lymphedema allowed different schools of approach to influence the therapy adopted. During the first period, from the start, up to approximately 1989, influenced by the Brazilian School of Professors Degni, Cordeiro and Mayall, who treated lymphedema with resection surgery, physiological surgery or a combination (as with lymphovenous anastomosis using Cordeiro's techniques, which were current at the time ^8,9), together with medico-pharmaceutical techniques and dietary-hygienic methods, we obtained satisfactory results for the period.

During the second period, the influence was from European schools, with Földi's complex decongestive therapy (manual lymphatic drainage, hygiene and compressive therapy) ^10,15 and Leduc's ^16,17 (manual lymphatic drainage, hygiene, compressive therapy and intermittent pneumatic compression).¹⁸ The therapeutic system was then reviewed and the physiotherapeutic treatments were used together with high doses of pharmaceuticals derived from benzopyrones, as proposed by Casley-Smith.^5,19,20

After approximately 5 years, the third period began, due to dissatisfaction with the results obtained. It was believed that success in combating this disease should be defined in wider terms by the treating doctors. The interdisciplinary approach,¹³ involving specialists who are not traditionally found in the same building, such as lymphologists, dermatologists, nutrionists, pain management specialists, psychologists and physiotherapísts, resulted in a new system for caring for these patients, making better results possible. The patients became conscious of the fact that lymphedema was only a part of their affliction and that the team would meet their needs,²¹ reducing secondary skin complications, achieving appropriate body weight by means of nutritional planning, treating pain ²² effectively and dealing with the psychological aspects ¹⁴ of the disability itself.

Correctly establishing a diagnosis of etiology (due to the complexity of the disease), made it possible to better guide the interdisciplinary team, who focused, case by case, on the consequences of the edema.

Hygienic and dietary measures maintain cutaneous trophism and help avoid infectious and/or inflammatory complications . It is also necessary to avoid traumas or microtraumas which could go unnoticed and give entry to pathogenic germs. A balanced diet, tailored to the patient's needs, allows the nutritional problems that most frequently accompany this infirmity to be corrected: obesity, lipodystrophy, diabetes mellitus and hyperlipoproteinemia. It is important to highlight that lymphedema is commonly associated with obesity, which, in many cases, is morbid and imposes serious difficulties on treatment. Here the psychotherapist's role is key.^14,21

The physical therapy used with these patients is inspired by the manual lymph drainage (MLD) techniques of Földi ^10,15 and Leduc.^16,17 They consist in moving liquid from the interstitial space to drainage centers using specialized kinetic maneuvers known as "manual lymph drainage", stimulating currents from the affected areas.^2,23,24-27 This procedure requires a profound knowledge of the complex lymphatic anatomy, with its lymph ducts and nodes,^11,23-25 and that the diagnosis is correct.⁷ Physiotherapies such as sequential pneumatic compression therapy (SPT) ^17,18 are also used, in this case equipment with 12 segmented chambers is used. Multi-layered bandages are applied promptly afterwards and personalized exercises designed to have a myokinetic effect on the lymph are also indicated.^3,20,26,27 As soon as the bandages are removed, the patient is given elastic medical stockings or sleeves for maintenance.

Lymphedema patients suffer, in addition to their physical deformities, from frustration from multiple previous treatment failures, from depressive crises, from indifference complexes and lack of confidence in their doctors. The work of the psychotherapist ^14,21 is vital to help the patient interpret and accept their condition and, consequentially, adhere to the medical treatment stipulated, which will be of long duration and of which complete resolution cannot be expected.

It is very common, especially with patients with lymphedema of the upper extremities,^2,27 to come across brachial plexus injuries, inflicted by other treatments and/or by the traction exerted by the weight of the affected limb, producing strong neuropathic pain. Specialist anesthetists treat this problem.²² With advanced cases, pain should be dealt with first and then the remainder of the treatment plan followed. Pain may be chronic, caused by the pathologic process within, or due to permanent dysfunction of the peripheral or central nervous systems, or both. It can even be provoked by psychological or and/or environmental factors. The confluence of pain (from the disease, from treatments or from other symptoms), hate (from lack of medical communication, late diagnosis, etc.), anxiety (fear of the hospital, of pain, of death) and depression (from loss of family and employment role, bodily changes, desperation, etc.) gives rise to what is known as "total pain".^14,21 In cases of somatic pain which has developed profoundly and continuously from the member that is affected by the tension to which lymphedema subjects the tissues the WHO's Pain Ladder is indicated: NSAID (mild opioid) + NSAID (strong opioid) + NSAID, at any strength, with or without adjuncts When pain is due to a plexopathy from traction (neuropathic or neurogenic), the affected areas generate spontaneous or stimulated nervous impulses which results in an abnormal painful sensation (dysesthesia), accompanied by "pins and needles or electric shocks". Initial treatment is with antidepressants (amitriptyline, fluoxetine, imipramine ) and anticonvulsants (carbamazepine, valproic acid , diphenylhydantoin), and it may be necessary to apply local anesthetics (intravenous lidocaine drops, mexiletine, plexus blockade) or sympathetic blockades (stellate ganglion).²²

An exhaustive dermatological examination6 allows the specialist to diagnose and treat cutaneous conditions, such as eczema, dermatosis, vesicles and lymphocytic verruca with or without lymphorrhea, which compromise the success of the treatment of lymphedematous patients.

Basic pharmaceutical treatment is indicated from the outset, using gamma benzopyrones or flavonoids ^19,20 at the therapeutic dosages indicated. These compounds present an action on lymphatic flow, increasing output, easing lymph transport and mobilizing proteins from the interstitium.²⁰ In all cases the initial dose is 1,500 mg of micronized diosmin and control observations are made every 8 to 10 days, assessing objective signs such as the volume of the member (checked by girth and volume), turgor and consistency and texture of the skin, in addition to symptoms reported by the patient, such as reduced pain and weight and increased comfort. If the patient does not exhibit improvement, the dose is increased 500 mg every 10 days, until adequate titers are achieved, which, in general, is never more than 3,000 mg. Laboratory tests are performed to monitor liver function before and during treatment. To date no signs of hepatotoxicity have been encountered.

In cases of genital lymphedema, treatment is always surgical. In all other types, when conservative treatments have not been successful, there is a chance that, in selected cases, surgery will be introduced.^1,28-31 This procedure, when performed at the opportune moment and when ell indicated, can optimize the results obtained with interdisciplinary therapy. Surgery should be performed immediately after the conservative treatment on redundant tissue, or when, despite significant volume reduction having been obtained with clinical treatment, a fibrous blockage persists which is irreversible. Surgical resection may also be indicated for severe primary lymphedema of lower members because of the large initial volume. In such cases it becomes necessary to first reduce the size of the limb in order to continue with conservative treatments. One further indication for surgery is when, despite intensive decongestive treatment, clinical status does not improve and lymphoscintigraphs reveal deterioration.^32-34

CONCLUSIONS

Lymphedema treatment has evolved from the more aggressive approach (surgery) initially conceived, passed through a more conservative phase and reached a stage at which an interdisciplinary team is involved and where surgical solutions are limited to certain selected cases and patients are cared for holistically in a single locations.

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