Diagnosis and treatment of lymphoedema

The causes of lymphedema are divided into:

1. primary – congenital disorders of the lymphatic system, resulting from abnormalities during embryogenesis (development of organs during fetal life)
2. secondary – accounting for 95% of all cases of lymphedema, resulting from damage to the lymphatic system throughout your life.

surgical treatment of cancers in which there is intervention in the lymphatic ducts or lymph nodes:

• Breast cancer
• Malignant melanoma
• Gynaecological cancers (ovarian cancer, cervical cancer and endometrial cancer)
• Prostate cancer
• Lymphoma
• radiotherapy
• Parasitic infections (filariasis) – mainly in tropical countries
• Inflammatory conditions
• Obesity, inactivity – significantly increases the risk of lymphatic insufficiency
• vascular surgeries
• medications taken
• injuries
• orthopaedic procedures

At the same time, there is a large group of patients in whom oedema appears without any identifiable cause – the so-called oedema of undetermined aetiology. Due to the lack of a clear cause, this group of patients is the most difficult to diagnose and potentially treat.

It is estimated that around 250 million patients worldwide currently suffer from lymphedema. Every year there are about 300,000 new cases of lymphedema worldwide.

This is related to the high incidence of oncological diseases and the constantly increasing number of surgical interventions. Statistics show that after axillary lymphadenectomy (removal of axillary nodes) the risk of lymphedema is respectively:

• 24-49% (amputation),
• 4-28% (BCT).
• 4-10% if the sentinel node is harvested alone
• Radiotherapy alone increases this risk to 15-50%

In Poland, there are about 250-300 thousand people with various stages of lymphoedema, of whom about 100 thousand patients are eligible for surgical treatment.

Analysis of the available data has determined a number of factors that predispose to and increase the risk of lymphoedema in the postoperative period:

• Larger tumour size (T) – the larger the tumour the potentially greater the extent of resection (removal), i.e. the damage caused by the surgical action is also greater
• Tumour in the upper-external quadrant – this location increases the risk of lymphatic drainage damage during tumour resection
• Greater number of lymph nodes removed – more lymph nodes removed always means a higher risk of lymphatic failure, so whenever possible we try to remove only the sentinel node
• Lymph node metastases – the presence of metastases is associated with the presence of cancer cells in the lymphatic pathways, more radical surgery in the lymph nodes of the axillary fossa, and the need for radiotherapy
• Radiotherapy, chemotherapy – cause local (radiotherapy) or systemic (chemotherapy) damage to the lymphatic ducts
• BMI > 30 – obesity itself limits the capacity of the lymphatic ducts. In combination with oncological treatment, it radically increases the risk of lymphoedema
• Sedentary lifestyle

Lymphoedema can occur at any time after completion of oncological treatment.

Changes in the lymphatic system in the initial stages are not visible to the naked eye. A lymphography done in a timely manner will allow assessment of the lymphatic pathways and possible indications for therapeutic/surgical intervention – even when there are no clinical features of lymphoedema yet.

In patients with lymphoedema, lymphography will allow to assess the probability of lymphoedema aggravation as well as to choose the appropriate surgical treatment technique and will precisely indicate the place of the appropriate surgical intervention.

There are a number of diagnostic tests, measuring methods and tools that can provide us with a lot of precise information regarding the lymphoedema; however, most of them do not provide the information necessary to make specific clinical decisions or to be eligible for surgical treatment. Therefore, we will only mention tools and techniques relevant to clinical practice:

• Physical examination – is a fundamental part of the preoperative assessment in any patient with lymphedema. It allows us to assess the circumference of the limb, compare it to a healthy limb, evaluate the skin texture and the clinical features and reversibility of the lymphedema. Thanks to these, we can classify the lymphoedema clinically.
• Indocyanine green (ICG) lymphography – using indocyanine green dye for near-infrared fluorescence labeling in lymphaticovenular anastomoses, helps define lymphatic dysfunction and delineate individualized lymphedema treatment. This study also helps visualize blood vessels and tissue perfusion in real time by observing fluorescence emitted by ICG dye. 

  It is a simple minimally invasive surgery. The patient does not need to be fasting. The dye is administered intradermally, so it is associated with slight pain, analogous to the pain during local anaesthesia. In most cases we inject both limbs for comparison – in total it is 6-8 pricks. The patient is evaluated immediately after the injection in order to assess the dynamics of the ICG flow, and then after about 2 hours – in order to assess the final state of the lymphatic system in the limb. 

  It is the primary test used to determine eligibility for surgical treatment of lymphedema. It allows to select patients eligible for surgical treatment, to choose the appropriate surgical technique and to choose the location of surgical incisions. 

  Lymphoscintigraphy is a special type of nuclear medicine imaging that provides special pictures of the lymphatic system, which transports fluid throughout your immune system. It allows us to assess the capacity of the lymphatic system. In debatable cases it allows us to decide on the choice of a particular surgical technique. The patient does not need to be fasting, with only minor pain. We inject both limbs for comparison. The examination is performed in the nuclear medicine department.

• Computed tomography angiography (CTA) uses an injection of contrast material into your blood vessels and CT scanning to help diagnose and locate blood vessels – that is places which will be anastomosed to lymphatic vessels (LVA) or place of collection and lymph nodes transfer (LNT). The examination is painless.
• A Doppler ultrasound is a noninvasive test that can be used to estimate the blood flow through your blood vessels by bouncing high-frequency sound waves (ultrasound) off circulating red blood cells. It is useful in both preoperative planning of the surgery and intraoperative verification.

The treatment of lymphoedema is a complex process. Many books have been written on the subject, although the level of knowledge is still inexhaustible. It should be mentioned that inherent elements of lymphedema treatment are:

• working with a physiotherapist (lymphatic drainage, CDPT),
• working with the limb on your own,
• wearing compression underwear
• surgical treatment.

Our experience to date shows that traditional conservative treatment is in many cases unsatisfactory, and the more advanced and persistent the oedema, the less benefit patients derive from conservative treatment.

At the same time, an inseparable element of modern lymphedema treatment is surgical treatment, which in many cases can help patients in whom conservative therapies have failed.

In Western European countries, the USA or Japan, surgical treatment of lymphoedema has been widely publicised and has been performed since the 1990s. A spectacular boom in surgical techniques began in 2006 when ICG lymphangiography imaging devices appeared on the market. They allow precise localisation of the lymphatic ducts, and thus have significantly improved the effectiveness of the procedures performed. It is estimated that currently 500 surgeons deal with lymphedema surgery worldwide and in Japan alone about 800 patients with lymphedema are operated on annually. This technique has become a standard worldwide, in Poland it is still in its infancy due to the necessity to purchase appropriate equipment and use super microsurgery techniques. The most commonly used techniques for surgical treatment of lymphedema are:

• lympho-venous anastomosis (LVA)
• lymph node transfer (LNT).

Lymphoedema is a multifactorial disorder with multiple mechanisms of lymphatic dysfunction. Surgical treatment is able to decompress (LVA) the inefficient lymphatic system or partially restore (LNT) it in the most inefficient area.

With lymphovascular anastomoses and lymph node transfer, we are potentially able to reconstruct the function of the lymphatic system. Thus, most patients achieve:

• clinical improvement of the skin and soft tissue
• reduced incidence of rosacea/inflammation
• improved functionality of the limb
• reduced limb circumference – from 10-90% in the long term
• reduced risk of lymphoedema exacerbation in the future

The final results of surgical treatment vary widely. Virtually every patient has a clinical benefit, although we are not able to preoperatively assess precisely how much a patient will benefit.

In patients eligible for lymph node removal, it is necessary to perform: ARM (axillary reverse mapping) should be performed to reduce the risk of lymphoedema.