Lymphedema | Definition | Therapy
EPIDEMIOLOGICAL ASPECTS
The data obtained from international literature, corresponding to the official ones of the World Health Organization, report an incidence of lymphedema in the world equal to 300 million cases (approximately one person in 20). Almost half of the lymphedema is of primary origin, characterized by a congenital basis lymphangioadenodysplasic, that is due to a malformation and consequent malfunction of the lymph nodes and / or lymphatic vessels. Another 70 million are of parasitic origin (the most frequent forms are represented by the infestation by Filaria Bancrofti), particularly present in tropical and subtropical areas (India, Brazil, South Africa). Another 50 million are post-surgical and, especially, secondary to the treatment of breast cancer. The other 30 million are essentially caused by functional problems of overload of the lymphatic circulation (particularly, in outcomes of deep phlebothrombosis of the lower limb, hepatic insufficiency, nephrotic syndrome, arteriovenous fistulas).
In particular, with regard to secondary lymphedema, the incidence of secondary lymphedema of the upper limb in women undergoing mastectomy or quadrantectomy with axillary lymphadenectomy is, that is, equal to 20-25%, up to 35-40% with association of radiotherapy. With the sentinel lymph node technique, the incidence of secondary lymphedema ranges from 3% to 22%. As regards lymphedema of the lower limbs secondary to the treatment of tumors of the gynecological and urological sphere, the incidence varies from 5% to 30%. Given the high incidence of secondary lymphedema, it is important to underline the importance of the possibilities of preventing lymphostatic pathology, both in terms of early diagnosis and timely treatment. The sex most affected is female and the most affected age corresponds to the third-fourth decade of life. The incidence of lymphangitis, clinically more or less manifest, as a complication of lymphostasis, was very high (practically in almost all cases), to the point of requiring prolonged antibiotic treatment, both for therapeutic and prophylactic purposes.
PATHOGENESIS
Lymphedema represents a not uncommon clinical picture characterized by slowing or blocking of the lymphatic circulation of the affected limb or limbs, with progressively worsening evolution and the appearance of recurrent acute lymphangitic complications of a mostly erysipeloid nature, responsible for a further and rapid increase in volume and consistency of the edema. Although the pathogenetic details are still an open question, the general principles of the pathophysiology of lymphedema are well known. On the one hand, the central disturbance can be represented by a low output failure of the lymphatic system: that is, there is a general reduction in lymphatic transport. An alteration of this type can be caused by congenital lymphatic dysplasia - primary lymphedema - or by anatomical obliteration, such as that resulting from a radical surgical resection (for example, in the case of axillary, iliac-inguinal or retroperitoneal lymph node dissection ), following repeated lymphangitis with lymphangiosclerosis or as a consequence of severe functional insufficiency (for example, lymphangiospasm, paralysis and valvular insufficiency) - secondary lymphedema.
However, the common denominator is the fact that lymphatic transport falls below the capacity necessary to manage the present load of microvascular filtrate, including plasma proteins and cells, which normally enter the interstitium from the bloodstream. On the other hand, high output failure of the lymphatic circulation occurs when a normal or increased transport capacity is overwhelmed by an excessive load of capillary blood filtrate: for example, liver cirrhosis (ascites), nephrotic syndrome (anasarca) and deep venous insufficiency of the lower limbs (post-thrombophlebitic syndrome). Failure to control lymphedema can lead to repeated infections (dermatolymphangiioadenitis - DLA), to progressive trophic skin alterations of the pachydermitic type and, in rare cases, even to the development of a highly lethal lymphangiosarcoma (Stewart-Treves syndrome).
ANATOMO-PATHOLOGICAL CLASSIFICATION AND STADIATION
Lymphedema are generally divided into primary or congenital and acquired or secondary.
Primary lymphedema are further distinguished in connatal, that is present already at birth, or with early manifestation, if they appear before the age of 35, or late, if they occur after the age of 35. Among the compatriots we distinguish the sporadic forms from the hereditary-familial ones which, for the most part, can be framed in more or less complex malformative syndromes, related or not to specific genetic alterations. For the identification of the type of dysplasia underlying the various forms of congenital lymphedema, the classification of C. Papendieck is followed: LAD I (lymphangiodysplasia - dysplasia of the lymphatic vessels), LAD II (lymphadenodysplasia - dysplasia of the lymph nodes), LAAD (lymphangioadenodysplasia - dysplasia of the lymphatics and lymph nodes). The term dysplasia includes: agenesis, hypoplasia, hyperplasia, fibrosis, lymphangiomatosis, hamartomatosis, valvular insufficiency. Secondary lymphedema can be divided into post-surgical, post-actinic, post-traumatic, post-lymphangitic and parasitic.
Classification of lymphedema
Primary or congenital
Connatali (present from birth)
Sporadic
Eredo-familiar
Early (appear before the age of 35)
Late (appear after age 35)
Primary or congenital
Post-surgical
Post-actinics
Post-traumatic
Post-lymphatic
Parasitic
CLINICAL PRESENTATION AND NOTES OF PHYSIOPATHOLOGY
In most patients, on the basis of the history and physical examination, the diagnosis of lymphedema can be easily made: edema generally of increased consistency, depending on the greater or lesser fibrosclerotic tissue component, absence of the sign of the fovea, even in the stages earlier than the disease, presence of Stemmer's sign (non-plicability of the skin at the base of the 2nd toe), skin dystrophic lesions (post-lymphangitic sequelae, hyperkeratosis, lymphostatic verrucosis, lymphorrhea, chylorrhea), frequent dermato-lymphangio- adenitic (DLA). The evaluation of the lymph node stations is also useful, to highlight the association or otherwise of acute or chronic lymphadenopathy. In the more complex forms of angiodysplasia, characterized by a condition of arteriovenous hyperstomy (Mayall's syndrome) or by congenital arteriovenous macro and microfistulas (Klippel-Trénaunay or Klippel-Trénaunay-Servelle disease), the clinical picture may be characterized by: gigantism with lengthening of the limb, more or less marked dysmorphism of the foot, “Port wine” colored angiomas, flat and geographical maps, hyperhidrosis of the sole of the foot. There are, however, spurious forms, even more difficult to diagnose due to the prevalent lymphedematous component. In some cases, moreover, the presence of overlapping conditions, such as morbid obesity, venous insufficiency, more or less evident trauma and recurrent infections , can complicate the clinical picture. Furthermore, when considering the origin of uni or bilateral lymphedema of the extremities, especially in adults, it is also necessary to consider the possibility of a tumor cause. For all these reasons, before embarking on the treatment of lymphedema, a complete and integrated diagnostic evaluation is absolutely essential. The association of other pathological conditions, such as congestive heart failure, arterial hypertension and cerebro-vascular diseases, including stroke, can in turn influence the therapeutic process. If the diagnosis of lymphedema is not clear or there is a need, even for prognostic considerations, for a better diagnostic definition of the clinical picture, a specialist lymphological consultation is advisable, referring the patient to a specialized lymphology center.
DIAGNOSIS
Lymphoscintigraphy is the first choice exam for the diagnostic definition of edema, to confirm its lymphostatic nature, to identify the cause (from obstacle or reflux), to assess the extent of the disease (dermal back flow), greater or lesser impairment of the deep lymphatic circulation compared to the superficial one, drainage through the lymph node stations. Therefore, the study of both superficial and deep lymphatic circulation is useful, by means of the appropriate injection of the tracer in the specific drainage sites of the two systems. The examination is non-invasive, easily repeatable, and can also be performed in neonatal age. It also allows to identify the stage IA of the lymphostasis, still clinically not manifest, thus playing a fundamental role in the prevention of secondary lymphedema. Finally, useful for the study in the follow-up of the various therapeutic methods of lymphedema and, in particular, of lymphatic microsurgery techniques.
Even today, Lymphography represents an extremely useful investigation for the study of complex congenital or acquired pathologies of the chiliferous vessels, the cisterna chyli and the thoracic duct. It is more modernly performed in the operating room, under local anesthesia and with preparation of the lymphatic vessels using a microsurgical technique. Ultrasound, CT and MRI represent diagnostic tools useful for the definition of complex syndromes in which angiodysplasia and lymphedema are associated, as well as for the study of the possible obstructive organic nature of lymphedema secondary to tumor disease. Lymphangio-MRI, in particular, performed with the digital subtraction method of adipose tissue, can provide important information in advanced obstructive pictures, in which the lymphatic ducts are dilated and filled with lymph. Indispensable is the study of venous circulation using Eco-Color-Doppler (survey constantly used in the instrumental evaluation of an edematous limb), Phleboscintigraphy and Phlebography (if necessary on the basis of the Eco-Doppler examination). indispensable in cases of panangiodysplasia with associated lymphedema. In these cases, in addition to the Eco-Color-Doppler examination, the digital arteriographic study may be useful. The Indocyanine Green Fluorescein Microlymphography (PDE Test) and the Houdack-Mc Master's lymphochromic test at BPV are now able to provide very useful information for the most correct surgical approach to lymphedema.
THERAPY
The therapy of peripheral lymphedema is divided into medical-physical-rehabilitative treatment and surgical methods.
Lymphedema therapy
Physical treatment
Physiotherapy
Combined physical therapy
Mechanical / manual lymphatic drainage
Pharmacological therapy
Benzopyrones
Antibiotics
Antifungals
Diethylcarbamazine
Diuretics
Diet
Surgical treatment
Microsurgery
Directive
Rebuilding
'Selective' and minimally invasive liposuction