Unfortunately, breast cancer is a relatively common condition. According to the American Cancer Society, more than 300,000 new cases of breast cancer will be diagnosed in the United States in the year 2021. Advances in medicine and surgery currently allow cure of breast cancer for many individuals. As such, at present time, there are more than 3.5 million breast cancer survivors in North America. However, many breast cancer survivors suffer the adverse side effects of their treatments, and lymphedema can be one of these. Although breast cancer treatment is the most common cause of lymphedema, other conditions may cause it as well. Lymphedema has been estimated to affect between 90 and 250 million people worldwide.
“There are more than 3.5 million breast cancer survivors in North America“
Lymphedema leads to abnormal accumulation of liquids and the formation of thick scar tissue, most commonly in the limbs. Patients with lymphedema are recommended to be extremely careful in protecting their upper extremity from cuts and even needle-sticks, for fear of lymphedema worsening or infection. In addition, the affected limb oftentimes feels heavy and aches. Some patients with upper extremity lymphedema also develop bad shoulder arthritis and may need to consider shoulder replacement surgery. Not many centers around the world have experience with shoulder replacement in limbs with lymphedema. The question then becomes: how dangerous is it?
What is lymphedema?
Most people know about arteries, veins and their role in blood circulation through the body. Arteries carry blood with high content of oxygen, cells and multiple nutrients and other molecules to our organs and limbs. Veins return blood to the heart and lungs. However, some fluid accumulates in our tissues with normal blood circulation. This fluid is recovered by the lymphatic system, a separate network of small vessels intimately associated with our immune system.
When the lymphatic vessels are obstructed or damaged, lymphedema may occur. Lymphedema literally means “swollen with lymph”. Protein-rich fluid accumulates in tissues. This fluid leads to inflammation, formation of scar tissue and deposition of fat. As a consequence, the affected area grows in size, feels heavy, sometimes tender, and it becomes prone to worrisome infection after even the most minor cut.
The lymphatic system, 101!
The very small vessels of the lymphatic system are called lymph capillaries. These capillaries represent the entry point for collection of fluid. Lymph capillaries coalesce in larger and larger lymph vessels or collectors, similar to ravines and torrents coalescing into larger rivers. The walls of lymphatic collectors have muscle cells that can help push fluid forward. Lymph collectors terminate in superficial lymph nodes that then filter lymph into deep lymph nodes. Superficial and deep lymph nodes filter lymph in order to identify harmful bacteria and other infectious organisms. Ultimately, all recovered lymph is drained into the venous system. It is easy to understand two main consequences of failure of the lymphatic system: lack of fluid collection leads to swelling, whereas compromised lymph node function increases the risk of infection.
Why does lymphedema happen?
Lymphedema is the consequence of damage or obstruction of the lymph system. Although breast cancer is the most common reason for lymphedema in individuals presenting with the need to undergo shoulder replacement surgery, lymphedema can occur as a consequence of multiple conditions.
In patients treated for breast cancer, surgical removal of lymph nodes as part of treatment may lead to lymphedema. Radiation therapy for breast or other cancers may contribute as well. The lymphatic system may also get blocked as a consequence of infection (especially parasite infections such as filariasis, where adult worms live in the lymphatic system of humans), severe injuries, chronic venous insufficiency, and even obesity. In fact, the obesity pandemic has produced a rapidly expanding subgroup of patients with lymphedema in the setting of obesity who lack other sources of lymphatic compromise.
Very rarely, lymphedema occurs because individuals are born with either no lymphatic conducts or the lymphatic conducts are too small or too large. The names of doctors that first identified these conditions, or Latin descriptors of the disease, are used to label them (Noone-Miloy syndrome when it starts at birth, Lymphedema Praecox Meige when it starts in adolescence or early adulthood, and Lymphedema Tarda when it starts later in life).
What are the usual symptoms of lymphedema?
The most common complaints of patients with lymphedema include heaviness, tightness, achiness and swelling. Initially, swelling may be minimal or absent. Next swelling develops but the affected part of the body can be restored to normal volume and contour by compression or elevation. Eventually, excessive swelling cannot be reversed due to permanent inflammatory changes. In advanced lymphedema, the skin itself becomes thicker with formation of papillomas and verrucous bodies; the affected limb looks like an elephant leg, large, cylindrical and with rough, thick skin. As such, the term elephantiasis is used to describe advanced stages of lymphedema.
|Stage 0||No swelling|
|Stage I||Reversible swelling|
|Stage II||Permanent swelling|
|Stage III||Abnormal skin changes, elephantiasis|
How is lymphedema managed?
The management protocol used to treat lymphedema is called complex decongestive therapy and includes two phases: reduction (phase 1) and maintenance (phase 2). Ideally, phase 1 measures should be performed twice daily; they include (1) manual lymphatic drainage through specific massage techniques, (2) repetitive limb movements for muscle contraction to pump lymph, (3) short periods of compression dressing application, and (4) skin care with lotions and ointments. Within 2 weeks of initiation of phase 1, most patients experience swelling reduction of 50% or more, and can be transitioned to phase 2. Phase 2 consists of the permanent use of compression garments worn during waking hours. Off-the-shelf lymphedema garments may work for patients in Stage I, but patients in Stage II or III require custom-made garments.
Surgery can be considered for stage III lymphedema as well as for stages I and II that do not improve after 6 to 12 months of complex decongestive therapy. In early stages, microsurgical techniques may be used to restore lymph flow with bypass procedures or transfer of lymph nodes from non-affected areas. In late stages, surgery involves liposuction or removal of lymphedematous tissue.
What if you have lymphedema and need shoulder replacement surgery?
When patients with lymphedema require any type of procedure, several concerns arise. First, any procedure may further compromise lymph flow and lead to worsening lymphedema, especially if the limb cannot be moved or garments cannot be applied during recovery. Second, the risk of infection is thought to be increased when procedures are performed in a limb with lymphedema. Third, if surgery must be performed through diseased tissues that are thicker and stiff, the procedure may be more difficult to perform. Finally, the much heavier limb could lead to incomplete recovery of function. These questions are very relevant for patients who have lymphedema in the same upper extremity that needs shoulder replacement surgery. Due to the nature of our practice, in our Service we have developed a number of strategies to minimize complications and provide a good patient experience for patients with lymphedema requiring shoulder replacement.
- Optimize limb condition prior to surgery
- Close collaboration with personnel lymphedema clinic
- May require reinstitution of phase I treatment modalities
- Cephalic vein preservation
- Meticulous attention to detail so that all regional vessels are preserved, and in particular the cephalic vein
- Prevention of infection
- Identification and management of other risk factors
- Skin cleaning and preparation
- 3 doses of IV antibiotics and possible continuation of oral antibiotics for 2 weeks or more
- Antibiotics (vancomycin) sprinkled at the surgical site before closing the incision
- Meticulous skin closure and sealing
- Postoperative lymphedema garment application
- Fabrication of adequate garment for the postoperative period
- Close collaboration with personnel from the lymphedema clinic
- Immediate limb motion
- Physical therapy starts in the hospital
- Instructions on safe use of a shoulder immobilizer only when needed
- Reverse arthroplasty may be selected to facilitate early motion without increasing risk of prosthetic instability
If you have lymphedema and need a shoulder replacement, we will be sure to collaborate closely with members of our lymphedema clinic before surgery, during the short hospital stay, and during the recovery period. Every effort will be made to prevent infection and preserve the veins in the arm. Emphasis will be placed in motion of the limb and limited use of the shoulder immobilizer, with a low threshold to favor reverse arthroplasty if needed in order to speed motion and recovery.
Our experience with shoulder arthroplasty in upper extremities with lymphedema has been published in Shoulder&Elbow. Despite all our efforts, lymphedema worsened for a few months in 50% of our patients, but eventually returned to baseline in many. Only 20% of our patients experienced permanent worsening of lymphedema. Infection was more common than in patients with no lymphedema, but for the most part the procedure was safe, and the overall improvements in terms of pain and function quite satisfactory. If you need our help, we are here for you!