PREVENTION OF LYMPHEDEMA

PREVENTION OF LYMPHEDEMA

PRIMARY, SECONDARY AND TERTIARY PREVENTION

Lymphedema prevention includes aspects of primary, secondary, and tertiary prevention. For primary prevention we mean the careful collection of anamnestic data, which is aimed at finding risk factors for the appearance of lymphedema, such as biological factors (sex, constitution, in particular obesity, hormonal status, familiarity), hygienic-environmental and climate, work activity and the most significant events in the remote pathological history, and of protective factors, such as lifestyle and lifestyle habits (diet, hygiene, sports activities), socio-health culture of Preventive Medicine, also in relation to the pathology of lymphatic circulation (similar to what is already practiced in the field of cardiovascular, arterial and venous diseases), as well as epidemiological studies on a regional, national, European and worldwide scale. For most of the risk and protection factors, however, the mechanism of action is not yet known.

Secondary prevention includes the clinical examination of the patient and the execution of a lymphoscintigraphy, which can highlight a condition of lymphatic stasis in the affected limb even before the clinical manifestation of the same. The main causes of secondary lymphedema are surgery, irradiation, infection, infestation (filariasis), trauma, metastatic lymph node involvement, etc. Surgical procedures at greatest risk include radical mastectomy, Wertheim-Meigs surgery, urologic oncological interventions, abdominal oncological surgery, axillary or inguinal lymphadenectomy, and other interventions, such as the removal of lipomas in critical sites (groin, axilla), safenectomy, and inguino-crural hernioplasty.

Tertiary prevention occurs in the more advanced stages of lymphedema, preventing the accumulation of fibro-adipose tissue produced by chronic lymph stasis, which increases the risk of new infections and causes a poor quality of life.

 

ASPECTS OF DIAGNOSIS AND TREATMENT

(with particular regard to secondary lymphedema of the upper limb)

Despite the technical evolution in surgical therapy of breast cancer, the problem of secondary lymphedema of the upper limb homolateral to breast pathology is still very important, both for the high incidence of this complication and for the possibilities of prevention. From the analysis of international literature on epidemiological aspects, it appears that the incidence of secondary limb lymphedema varies from 5 to 25% of women undergoing mastectomy or quadrantectomy and axillary lymphadenectomy, up to 40%, with the association of radiotherapy. A certain degree of lymph stasis can, however, be seen in almost all patients after axillary lymphadenectomy and can be demonstrated by lymphoscintigraphy, even before the clinical appearance of lymphedema. The time of occurrence of the edema remains, however, unpredictable, varying from immediately after surgery, to months or years after, and often triggered by acute erysipeloid lymphangitis.

The advances in the lymphoscintigraphic technique, as already mentioned, have allowed the study the patients even in the absence of clinically manifest edema, providing significant data on the correlations between type and extent of anatomical anomalies of the lymphatic circulation of the limb examined, with the appearance and degree of evolution of lymphedema. Taking into account these considerations, a study on patients undergoing breast cancer therapy was conducted by the Genovese study group in order to determine and verify the incidence of secondary lymphedema and to analyze preventive diagnostic and therapeutic methods that could reduce the possibility of such a complication.

The incidence of secondary lymphedema of the upper limb reported by several groups varies (from 5 to 45% of cases). This high variability depends on different factors, including, in particular, lymphedema diagnosis, which is often made when the disease is already at an advanced stage, breast cancer treatment protocol (whether the sentinel lymph node technique is used, radiation therapy, etc.) and, finally, the level of propensity of the post-mastectomy woman to develop this type of disease (prevention measures taken against bouts of lymphangitis). To this end, a closer interdisciplinary collaboration is desirable, to foster more frequent exchanges of experience and opinions specifically regarding these lymphangiological aspects. Preventive diagnostic and therapeutic procedures employed in this study have allowed a significant reduction in the incidence rate of lymphedema, compared to a control group of patients who had not undergone any prevention protocol. The importance of lymphoscintigraphy in the detection of early lymph stasis of the affected extremity, even before the onset of clinically overt lymphedema, has been pointed out in the international Literature. In the group of patients who have undergone lymphoscintigraphy, those cases with the highest risk of lymphedema development were detected, and were then treated with proper therapeutic procedures aimed at limiting lymph stasis as much as possible, while, at the same time, stimulating other supplementary lymphatic drainage pathways.

While seemingly easy to apply, adopted medical and physical-rehabilitation measures, must be carried out by professionals with a specific and thorough lymphangiologic experience in order to ensure their proper implementation. Patients who fail to react positively to medical-physical treatment and who, in spite of therapy, show a progressive disease, complicated by bouts of lymphangitis, are then selected for derivative lymphatic-venous microsurgery. A specific training and a good clinical experience are necessary to perform Lymphatic Microsurgery. The outcome, however, when the intervention is correctly performed, is excellent, with functioning and patent anastomoses also at long-term follow-up.

Therefore, today, thanks to lymphoscintigraphy, medical-physical, as well as microsurgical treatments can be carried out as early as possible, and therefore it is possible to treat the early stages of lymphedema, and obtain an almost total regression of disease, and even clinical healing. Also, if preclinical alterations are detected in the lymphatic drainage of the limb homolateral to axillary lymphadenectomy, lymphedema onset can be prevented by fashioning a microsurgical anastomosis concurrently with axillary lymph node resection in this seemingly unaffected limb (primary surgical prevention of lymph stasis).

More recently, the favorable and significant results obtained with the complementary procedure of Fibro-Lipo-Lymph Aspiration with “Lymph Vessel Sparing” procedure in the more advanced stages of lymphedema only partially responsive to Microsurgery (MLVA), allow us to realize also a tertiary prevention. This significantly and stably reduces the risk of further lymphangitis complications and ensures a very satisfactory quality of life even in patients in whom the lymphatic pathology is of a very advanced and disabling stage.

 

CURRENT STATUS OF RESEARCH AND FUTURE PROSPECTS

Therefore, in patients undergoing surgery who, due to their medical history, have a higher risk of developing lymphedema, their education is key: they must learn to examine themselves often for signs of swelling, as well as to undergo regular specialist medical checks and lymphoscintigraphies. However, also patients who do not carry anamnestic risks should be taught self-examination and should undergo follow-up visits at regular intervals after their primary tumor surgery.

Secondary Lymphedema prevention requires an interdisciplinary approach, which comprises specialist doctors of different medical and surgical disciplines (general surgeons, urologists, gynecologists, radiotherapists, oncologists, etc.). It is aimed at identifying risk factors of lymphedema development in patients who have undergone certain surgical and/or radiotherapy treatments, while identifying primary and secondary prevention methods and developing specific prevention protocols for the various specialty areas.

The secondary lymphedema prevention protocol, that includes clinical and instrumental (lymphoscintigraphy) tools, can offer a guideline with which each specialist physician (general surgeon, urologist, gynecologist, radiotherapist, oncologist, etc.), in their daily clinical activity, can work out a suitable diagnostic, clinical, and therapeutic approach aimed at preventing the onset of more or less severe lymph stasis in the affected limb/s of patients at risk who have undergone surgery or radiation therapy for a primary disease which, often, is already a serious condition (malignant tumor). The end goal is to ensure patient quality of life, and, at the same time, prevent the onset of frequent and crippling lymphangitis, as well as the vicious but still possible, although rare, growth of lymphangiosarcomatous tissue (Stewart-Treves Syndrome) in the same lymphedematous limb