A chronic and disabling vascular pathology requiring a thorough diagnostic approach, multidisciplinary therapeutic management and long-term clinical-instrumental follow-up since only a rigorous integration of the various therapeutic options currently available is able to ensure these patients a better quality of life. Lymphedema, commonly classified as primary or secondary, is an edema of high interstitial protein concentration determined by a reduced capacity of lymphatic, congenital or acquired transport.
From the
pathophysiological point of view, the mechanisms involved in the determinism of
this condition are essentially three:
• increased lymph production in the interstitial spaces, in the time unit
considered, with the same "transport capacity" of the lymphatic system;
• production of constant lymph, but reduced "transport capacity" of the
lymphatic system, for congenital reasons (reduced development of part of the
local lymphatic system or impaired functioning of the valve systems, typical of
primary lymphedema), or acquired (destruction or surgical ablation, radiotherapy
or traumatic pathways and / or Iinfo-glandular stations for parasitic
infestation, radicality of surgical treatment and / or oncological radiotherapy,
trauma);
• functional overload of the regional lymphatic system for reasons linked to
other organs or systems (for example, post-phlebo-thrombotic syndrome, chylous
ascites or chylothorax).
• Primary lymphedema: may be present at birth. or manifest itself within the
second decade of life (early) or later (late).
• Secondary lymphedema: can intervene by removal or destruction of lymphatic
pathways and stations following oncological surgery, radiotherapy, parasitic
infestation (especially from Filaria bancrofti in the populations of the Middle
and Far East and in Latin America), traumas, "disuse" (mainly in late age),
iatrogenic lesions or functional overload (postflebotrombotic limb syndrome).
According to data from the World Health Organization (WHO), about 300 million
cases of lymphedema reported annually 150,000,000 are primary, 50,000,000
secondary to surgery, 70,000,000 due to parasitic infestation and about
30,000,000 fall in the so-called functional forms.
According to recent studies, primary lymphedema would not be an autosomal dominant "incomplete penetrance" disorder in which multiple members of the same family may carry the mutation - generally for the FOXC2 and VEGFR3 genes - while not showing anatomical alterations, but rather "to expressivity variabile", a condition in which all the subjects of the same family carrying the mutation also present an anatomical alteration, evidenced by lymphoscintigraphic examination; in some of these subjects the anatomical defect manifests itself clinically in the course of life, while in others it remains clinically silent (figure 3). This aspect is very important for primary prevention purposes. However, there exist forms of sporadic primary lymphedema in which the genetic mutation responsible for the disease is not found in other subjects of the same family; in these cases, in general, other genes are also called into question.
• Stage 0: absence of edema in predisposed subjects (for example, mastectomized
woman with limbs coincident for volume and consistency, consanguineous of
subject with primary lymphedema with positive lymphoscintigraphic examination).
• Stage l: Persistent edema that regresses spontaneously with nighttime rest or
declivity.
• Stage 2: permanent edema that only partially regresses with treatment (with
possible infectious and dystrophic complications).
• Stage 3: elephantiasis with disappearance of bone, tendon disorders and
possible infectious (bacterial and / or fungal) complications, ulcerative and
neoplastic degenerative (Stewart-Treves sarcoma.) Since errors are not
infrequent, it is necessary to proceed with extreme accuracy through each step
of the diagnostic procedure.
It plays a fundamental role: just think, for example, of the significance of
information such as the coexistence of other cases in the family context or the
presence of a previous trauma or another "trigger" factor for primary forms, or
the anamnestic data of an intervention, a trauma, a radiotherapy or an
infestation for the secondary ones.
The progression of the edema along the limb is distal-proximal in the primary forms, proximal-distal in the secondary ones. Often we associate positive thermotact (cutaneous temperature reduction) and increase of tissue consistency (with sign of the fleeting or absent fovea). In the primary lymphedema of the lower limbs, the sign of Stemmer, or the impossibility of "pinching" the skin of the toe with respect to the underlying bone phalanx, is pathognomonic. In case of complications it is possible to observe skin dystrophies, pachidermia, verrucosis and ulcerations.
The gold diagnostic standard for lymphedema is lymphoscintigraphy. The technique of execution of the survey involves the subcutaneous inoculation of radioactive technetium-labeled n-nocolloids in the interdigital spaces of the hands and / or feet, followed by some muscular exercises that facilitate the sliding of the radioactive tracer through the lymphatic canalicular system. A gamma camera then picks up the tracer which, meanwhile (on average 20-30 '), has gone to the root of the limb and beyond. From a morpho-functional examination (times of appearance) of the three segments of the limb it is possible to establish the level and degree of lymphatic transport deficit. One of the typical lymphoscintigraphic signs is the dermal back flow which testifies to the stagnation of tracer at the edematous area. Another survey used is the high-resolution ultrasound of the above and subfascial tissues that allows to highlight the degree of thickening of the epidermis, the increase of the overfascial thickness (skin-muscle thickness) in the affected side, the prevalent water quota or fibrotic tissue and the presence or absence of lymphatic "lacunae" and "lakes"; the exam is also useful in monitoring the treatment.
At specialized centers it is possible to perform surveys such as
computerized tomography and lymphangiorance, whose standardization is still
ongoing. Other methods, such as indirect lymphography, fluorescein
microlymphography, the Houdack-Mc Master lymphoid test, flow and lymphatic
pressure measurements and laser-Doppler can provide useful information on
anatomic and functional conditions, as well as blood microcirculation (
laser-Doppler) also of the initial lymphatics and lymphatic collectors, but
their clinical utility is limited. The lymphography, surpassed for many types of
clinical pictures, remains valid in the optics of demolition interventions or
reserved for deep anatomical, abdominal and thoracic areas.
... and functional evaluation
Depending on the overall detectable disability in the patient affected by limb
lymphedema (including the items of the Classification of Functioning, Disability
and Health - ICF, relating to the psychic, sexual, social spheres, etc.), it is
possible to distinguish five different degrees of functional commitment. .
• Grade 0 - Absence of disability: the patient performs his activities without
restrictions in his choices and / or functions, even if with orthoses.
• Grade 1 - Mild disability: the patient has a value of difficulty qualified as
mild in at least one of the activities / participations coded by the ICF. ''
• Grade 2 - Moderate disability: the patient experiences a value of difficulty
qualified as moderate in at least one of the activities / participations encoded
by the ICF.
• Grade 3 - Serious disability: the patient has a value of difficulty qualified
as severe in at least one of the activities / participations coded by the ICF.
• Grade 4 - Complete disability: the patient presents a difficulty value
qualified as complete in at least one of the activities / participations coded
by the ICF.
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Elastocompressive bandage: begin the bandage from the foot proceeding towards the leg and the thigh, with limb in discharge, better in the morning after a night of rest, with limb detumed. Bandages with zinc cream are used and then on these bandages, the elastic bandage is positioned, eg. type "varimed" |
In general, the diagnosis of lymphedema does not involve particular problems as
this pathology usually has very peculiar anatomical, clinical and objective
characteristics; however
Lymphoedemas make use of the competition of several types of treatment with
respect for individual indications and contraindications.
First phase: involves skin care, manual lymphatic drainage, a series of
gymnastic exercises and elastic compression, normally applied with multi-layer
anelastic bandages. Ultrasound, shock waves and depressotherapy (vacuum therapy)
are also useful in the anatomical areas most affected by tissue fibrosis.
Second phase: it must be started as soon as the first phase is completed, with
the aim of maintaining and optimizing the results obtained; includes skin care,
elastic compression by means of a brace (stocking or "flat knit" bracelet,
standard or made to measure - classes of compression between I and IV according
to the RAL system), gymnastics for functional recovery of the 'limb / s.
Essential conditions for the success of the combined physical protocol are the
patient's availability and his full participation in the therapeutic protocol in
both phases, as well as the preparation of medical personnel (clinical
lymphologists), nursing and physiotherapy. Elastic compression, if not properly
applied, can be not only useless, but also harmful. The daily wear of the
definitive elastic, prescribed and tested with positive results, is the most
important predictive prognostic factor.
The sequential pressure therapy, always preceded by the manual preparation of
the lymphatic stations at the root of the limb, is now reserved for patients
susceptible to prevalent passive physical therapy (allured, hypo-mobiles for
neurological or osteo-arthropathy). Given the rapid growth in volume, this
option should be used with caution in individuals with arterial hypertension and
heart failure.
• Benzopyrones: include coumarin and derivatives (alpha-benzopyrones) and
bioflavonoids and derivatives (gamma-benzopyrones, ie diosmin, rutin, hesperidin,
quercetin, etc.). Alpha-benzopyrones increase capillary tone, resulting in
reduced permeability, increase and activate macrophages, stimulate the
contractility of lymphatic collectors (prolokokinetic action) and inhibit the
synthesis of prostaglandins and leukotrienes. They then promote interstitial
reabsorption and promote partial regression of fibrosis. The best results are
achieved in the early clinical stages. Specifically, the benzopyrones are able
to improve the subjective symptomatology and functional recovery of the
lymphedematous limb. The activities performed by gamma-benzopyrones include the
reduction of endothelium permeability to protein macromolecules and capillary
filtration, as well as an increase in the venular tone; consequently, the
compounds belonging to this therapeutic class exert a stabilizing action on the
in-tertial connective tissue and on the capillary wall and inhibit the
production of prostaglandins and leukotrienes.
• Mesoglycan: thanks, above all, to the presence of heparansulphate and dermatan
sulfate, fundamental constituents of the vessel wall, shows its pharmacological
activity at endothelial and subendothelial, antithrombotic (activation of
antithrombin III and heparinic cofactor II) and profibrinolytic (stimulation of
tissue plasminogen activator). Specifically, on the venous side of the
circulatory system, in addition to intervening in the antithrombotic sense, it
is able to restore the physiological properties of the selective barrier exerted
by the capillary endothelial cells, thus performing an effective anti-edema
activity. In a study by Lillo et al, which aimed to evaluate the effect of
mesoglycan in the treatment of lower limb lymphedema, in a group of patients at
the 2nd / 3rd stage and followed up for a follow-up of 12 months, the main
results were: reduction of venous pressure of 12.4% in orthostatism, an
improvement in the consistency of edema, and a satisfactory functional and
aesthetic recovery, a volumetric reduction of the limb of 23.94% 15.
• Antibiotics: in the acute phase for the treatment of
dermato-lymphangien-adenitis (therapy for beta-hemolytic strep-tococco) and for
preventive purposes for the prophylaxis of episodes of acute lymphangitis (penicillin
with protracted action).
• Antifungal: for fungal infections of the extremities (fluconazole, etc.).
• Diethylcarbamazine: to eliminate microfilaria from the bloodstream in patients
with parasitic-based lymphedema and in healthy carriers.
• Diuretics: they are usually used at low dosage and for short periods of
treatment, especially in pictures of lymphedema associated with flebedema or in
other diseases, such as heart disease, kidney disease, ascites, pathologies of
chiliferous vessels, etc. Not removing the interstitial protein component of the
edema, they are exclusively symptomatic
• Protease: able to reduce interstitial macromolecules micromolecules, more
easily absorbed and transportable by the lymphatic system
• Vaccines against the germs commonly responsible for infections of the skin and
respiratory: used by various schools, reduce the frequency and intensity of new
episodes, reducing, among other things, the consumption of antibiotics.
• Diet: in obese patients, the restriction of caloric intake, in association
with a suitable program of physical activity, has its specific effectiveness in
reducing the volume of the lymphedematous limb. The validity of a limited intake
of fluids has not been demonstrated. In syndromes with chylous reflux, a diet
with a low lipid content that includes the intake of only medium-chain
triglycerides (medium chain triglycerides or MCT), absorbed through the portal
circle and therefore without overloading the system of chiliferous vessels.
The surgical techniques used in the past for the treatment of lymphedema aimed
at the volumetric reduction of the limbs by means of de-molitive-resective
interventions (cutolipofascectomy, total superficial lymphangectomy, Thompson's
intervention, debulking, etc.). The advent of microsurgery has instead allowed
to study and implement functional and causal therapeutic solutions aimed at
draining the lymphatic flow or reconstructing the obstructed or missing
lymphatic pathways. The derivative techniques aim at restoring the lymphatic
flow in the obstruction site through the realization of a lympho-venous drainage,
with the use of lymph nodes or directly of the lymphatics. The most recently and
commonly used are the multiple lymphatic-venous anastomoses. These
reconstructive techniques allow to restore a continuity of flow of the lymphatic
circulation, overcoming the site of the block by direct anastomosis of the
afferent and efferent lymphatic vessels or by the implantation of autologous
lymphatic or venous segments between the downstream and upstream collectors. The
autologous lymph node transplantation, recently introduced, finds particular
indications in the secondary (but also primary) forms and in the anatomical
regions with particular fibrosis (for example, after radiotherapy). The
super-microsurgery, even more innovative, includes peripheral interventions that
can be adopted in both primary and secondary lymphedema and can be performed on
pots of 0.3 to 0.8 mm19. The indications to the various techniques of lymphatic
microsurgery are based on the presence of a valid lymphatic-venous pressure
gradient in the affected limb. In cases in which the lymphatic pathology is
associated with venous insufficiency (a prevalent finding in the lower limbs:
varices, venous hypertension, valvular incontinence), the derivative methods are
contraindicated, while the reconstructive ones must be used. When the edema does
not respond to physical treatments (prevalence of the neo-lipogenic component,
present above all in some sequillary forms) and to the objective examination
the sign of the fovea is absent, liposuction can be successfully performed.
Psychosocial support accompanied by a program of assessment and improvement of
the quality of life of patients suffering from lymphedema, is a fundamental
integral component of any type of treatment. The patient often feels the disease
as a serious alteration of his body image which, associated with the functional
deficit and chronicity typical of this pathology, as well as to the diurnal
observance of the definitive elasticity, helps to disturb his psychophysical
balance with important negative repercussions in the psycho-social sphere.
indice argomenti di chirurgia vascolare