Primary and secondary prevention

Lymphedema prevention includes both primary and secondary prevention aspects. Primary prevention means an accurate collection of information about the patient’s medical history, aimed at identifying risk factors for lymphedema onset, namely biological factors (gender, body frame – in particular obesity – hormonal status, family history of lymphatic diseases), hygienic environmental and climate factors, occupation, and the most significant elements in the patient’s remote medical history; as well protective factors, such as life style (diet, sanitary hygiene measures, sports), a social-health culture of Preventive Medicine, also with reference to lymphatic circulation diseases. In other words, something similar to what is already being done for cardiovascular (both arterial and venous) diseases. Information about epidemiological studies on a regional, national, European and worldwide level is also important. However, the mechanisms of action of the majority of risk and protection factors are still not exactly known.

Secondary prevention includes the patient’s clinical examination and a lymphoscintigraphy, which can highlight lymphatic circulation stasis in the affected extremity even before any clinical manifestation. The main causes of secondary lymphedema are surgeries, radiation therapy, infections, infestations (filariasis), traumas, metastatic lymph node involvement, etc. Surgeries that have the highest risk are radical mastectomy, Wertheim-Meigs operation, urological surgeries for oncological reasons, abdominal oncological surgeries, lymphadenectomies in the axillary or inguinal regions, as well as other types of surgery, such as the resection of lipomas in critical sites (armpits, groin), saphenectomies, and inguinal-crural hernioplasty.

Diagnosis and treatment (with particular focus on secondary lymphedema of the upper limbs

In spite of technical evolutions in breast cancer surgical therapy, secondary lymphedema of the homolateral extremity in breast cancer is still a very important problem, for the high incidence of this complication, as well as for the chances to prevent it.

From a review of the international literature on epidemiological aspects, secondary lymphedema of the upper extremities has an incidence from 5 to 25% of women who have undergone mastectomy or quadrantectomy and axillary lymphadenectomy, which increases to 40%, when associated with radiation therapy. Further, a certain degree of lymph stasis is observed in almost all of these patients after axillary lymphadenectomy – as demonstrated by lymphoscintigraphy – even before any clinical onset of lymphedema. The time of onset is still unpredictable, and it may range from an appearance immediately after surgery or within a few weeks, to a later onset, after months or years, often triggered by acute erysipeloid lymphangitis.

Following recent progresses in lymphoscintigraphy, as already mentioned, patients can be examined even when the edema is not yet clinically overt. Significant information is provided, by correlations between type and extension of anatomic lymphatic circulation anomalies in the examined limb, and the onset and degree of subsequent evolution of the lymphedema. Based on these considerations, our Group in Genoa has conducted a study on patients who have undergone breast cancer therapy, in order to determine and verify the incidence of secondary lymphedema, as well as to analyze preventive diagnostic and therapeutic methods that would allow the risk of this complication to be reduced.

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, owing 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).

State of the art 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.