More than 25 years have passed since Ann Fonfa, 71, first received a diagnosis of breast cancer. After a grueling series of treatments and recurrences, she can finally say she’s cancer-free. Yet, Fonfa and countless other survivors experience a problematic and dreaded complication of breast cancer treatment that significantly lowers their quality of life.
Lymphedema, a buildup of lymph fluid in the soft tissues just under the skin, affects roughly 30% of individuals who undergo surgery or radiation for breast cancer. Symptoms include swelling — usually in the arms or legs — along with feelings of heaviness or tightness, a restricted range of motion, recurring infections and discomfort. Having an oversize limb can lead to body image and self-esteem issues, and clothes or shoes may no longer fit.
In 1993, Fonfa noticed swelling in her left arm almost immediately after a lumpectomy and removal of her axillary lymph nodes, which are located in the armpit. When she showed her doctor, he responded dismissively that she had lymphedema — it was common and had no cure. Before her surgery, she was not told that the condition could be a side effect, and Fonfa remains resentful.
“My left side doesn’t get as strong as my right side, even with exercise. It has been permanently harmed and will always be weaker,” says Fonfa, a resident of South Florida. “I didn’t even want to hold my baby grandson at first because I was afraid of dropping him.”
Judy Cooper, a 61-year-old Los Angeles resident who in 1996 received a diagnosis of a very aggressive form of breast cancer, had a similarly disappointing experience. Despite a poor prognosis, she survived inflammatory breast carcinoma after an intense treatment regimen that included multiple surgeries and courses of chemotherapy, a stem cell transplant and radiation. Four years later, when visiting her oncologist, she asked him about the swelling in her right arm.
He said, “Oh, that’s just lymphedema,” Cooper recalls. Only when Cooper asked for details did she learn that lymphedema is a relatively common — and incurable — side effect of breast cancer treatment. “I was extremely upset that, after working with numerous doctors and health care professionals battling cancer for four years, no one had ever made me aware of this condition,” she says.
Her lymphedema began with minor swelling that progressively worsened. Despite several rounds of intense physical therapy over the years to reduce the swelling and heaviness in her arm, she developed recurring infections that caused high fever, painful and hot swelling, and nausea. After going through 10 debilitating infections that required multiple hospital visits and IV antibiotics, Cooper had lost hope for a normal quality of life.
Both Fonfa and Cooper managed their lymphedema as best they could with the recommended treatment — massage, skin care, exercise and compression garments such as bandages or a lymphedema sleeve or stocking — but their condition stayed more or less the same for decades. They were disheartened, knowing that despite surviving cancer, they would have to cope with the discomfort and distress of lymphedema for the rest of their lives.
Over the past several years, as more patients spoke up and research interest in lymphedema rose, new treatment options began to emerge in the U.S. In particular, two surgeries — lymphovenous bypass and vascularized lymph node transplant, or transfer — have grown in popularity, because they relieve symptoms that do not respond to other treatments. Lymphovenous bypass is an intricate microsurgery technique that builds connections between lymphatic vessels and veins, allowing fluid to get around the damaged area. Vascularized lymph node transplant replaces lost lymph nodes, along with their associated microvessels, with those from elsewhere in the body. The procedures aren’t labeled curative and remain limited to a handful of cancer centers, but early results appear promising.
“This procedure has alleviated a lot of the fear and anxiety I’ve had about my lymphedema. My arm looks and feels better than it has in 19 years. I consider it a miracle,” says Cooper, who underwent lymphovenous bypass in January. “It’s not a cure, but it has made such an improvement in my outlook on life and optimism for the future that I want people who suffer from lymphedema to be aware that there is help out there.”
An Abnormal Buildup of Fluid
Over 250 million people worldwide have lymphedema, mostly as a result of parasitic infection from roundworm. However, in developed countries, the leading cause is damage to the lymphatic system because of cancer treatment. The condition can arise after surgery or radiation treatment for nearly any type of cancer but is most commonly linked to breast, prostate and pelvic area cancers (such as bladder, penile, testicular, endometrial, vulvar and cervical cancers), lymphoma, melanoma, and head and neck cancers.
The lymphatic system consists of a complex network of vessels, tissues and organs that helps the body maintain fluid balance and defend against infection. Lymph fluid travels through the system similarly to how blood travels through the circulatory system. Lymphedema occurs when lymph fluid can no longer flow normally due to damage or a blockage in the system. Fluid builds up in the tissues and causes regional swelling; consequently, patients feel discomfort, heaviness, tightness and a loss of mobility. They also have a higher likelihood of infection from cuts, scratches or insect bites, because their lymph fluid is unable to effectively filter out bacteria and toxins.
Patients who undergo surgery that includes removal of lymph nodes or have radiation therapy to the area where lymph nodes are located have a higher risk of lymph- edema. The risk increases with the number of lymph nodes affected and decreases with the removal of just sentinel lymph nodes (the first few lymph nodes into which a tumor drains). Other risk factors include being overweight or obese; slow healing of skin after surgery; a tumor that affects or blocks the left lymph duct or lymph nodes or vessels in the neck, chest, underarm, pelvis or abdomen; and scar tissue in the lymph ducts under the collarbones, caused by surgery and/or radiation therapy.
Most lymphedema develops within three years of cancer treatment, but it could appear within days for some people, as it did with Fonfa. The condition is typically diagnosed by physical exam, such as measuring changes in the circumference or volume of limbs, although imaging techniques can also be used.
“Sometimes patients report signs or symptoms to us, such as heaviness or fullness. Rings, watches and jewelry feel tight,” says Dr. Mark Schaverien, a plastic surgeon at The University of Texas MD Anderson Cancer Center in Houston. “We also have a lymphedema screening program, where the volume of patients’ arms is measured using a noninvasive device called a perometer before and after surgery for breast cancer. There are ways to catch swelling before it becomes clinically apparent, and the earlier we can catch lymphedema, the better the outcomes of treatment.”
At MD Anderson, all patients whose lymph nodes are removed as part of breast cancer surgery receive educational counseling from a physical therapist specializing in lymph- edema. They learn about arm exercises to help with range of motion, skin care, massages to move the lymphatic fluid and options for compression therapy. If their lymphedema persists, surgery becomes a viable choice.
Growing Demand for Surgical Options
Three surgical procedures exist for lymphedema, which can also be combined to improve effectiveness: lymphovenous bypass, vascularized lymph node transplant and suction-assisted lipectomy, also known as liposuction. Lymphovenous bypass, which connects lymphatic vessels to small adjacent veins to bypass the damaged area, is a minimally invasive procedure with 1- to 2-centimeter-long incisions. The surgery takes two to three hours under general anesthesia, with a hospital stay of less than 24 hours. Risks are minimal, Schaverien says. The surgery was first described by Japanese surgeon Yukio Yamada in 1969.
“Bypass tends to work best for folks with relatively early-stage lymphedema. The reason is because bypass does rely on the native lymphatic system to be partly working, since it is a plumbing-type procedure,” says Dr. Ketan Patel, surgeon and director of the Center for Advanced Lymphedema Surgery and Treatment at Keck Medicine of the University of Southern California. “You need water going through the pipes if you want to manipulate the plumbing.”
More advanced lymphedema requires vascularized lymph node transplant, sometimes combined with lymphovenous bypass. The procedure was pioneered by French surgeon Corinne Becker in 1991. Healthy lymph nodes are harvested from the abdomen (which can be performed laparoscopically), neck, trunk or groin area and reattached to the limb affected by lymphedema via microsurgery. Lymph node transplant, a more complex procedure, can take six to eight hours under general anesthesia and requires a hospital stay of around four days. Recovery takes roughly three to four weeks.
A major risk of lymph node transplant is that it simply may not work for everyone, for reasons yet unknown. Schaverien says about 1% of transplants fail to reestablish circulation. Additional risks depend on where in the body the harvest occurred. For instance, lymph node removal from the abdomen involves risks that come with any abdominal surgery, such as bowel obstruction, hernia, intra-abdominal injury and ileus; however, there appears to be no risk of causing lymphedema from where the lymph nodes are taken.
Taking lymph nodes from under the arm, groin or neck may cause lymphedema in those areas, so surgeons today sometimes employ an innovative technique called reverse lymphatic mapping. This strategy involves injecting different-colored dyes into the body to determine which lymph nodes can be safely removed. Lymph nodes draining the limb are avoided, while those draining the trunk may be harvested.
“I first described and developed reverse lymphatic mapping, which allows us to identify and avoid the lymph nodes draining the arm or leg,” says Dr. Joseph Dayan, a reconstructive surgeon at Memorial Sloan Kettering Cancer Center in New York City. “We use lymph nodes from inside the abdomen that do not drain any extremities, so there is no risk of causing lymphedema.”
Dayan has performed lymphedema surgery for about 10 years and has over 300 cases under his belt. During a microsurgery fellowship in Taiwan, he was taught the procedures by plastic surgeon Ming-Huei Chen at Chang Gung Memorial Hospital, which has served as a learning and innovation hub for lymphedema microsurgery. Dayan brought his expertise back to the U.S. and later performed one of the first lymph node transplants here. Patel was also taught the two procedures by Chen.
“One of the problems is that lymphology as a specialty is not taught in U.S. medical schools, and lymphedema itself isn’t even talked about as much to medical students,” Patel says. “So many people have learned the procedures in Taiwan, and now there’s a handful of us — 15, maybe 20 surgeons — who do a reasonable volume of this surgery. But we’re teaching others to do it.”
Patel sees about 400 to 500 patients with lymphedema per year, including Cooper, on whom he performed lymphovenous bypass. He sees signs that the field is growing exponentially in popularity to meet this high demand: More centers are advertising for the procedures, more practices want to incorporate them into their services and more trainees are interested in learning them.
The last surgical option for patients with highly advanced lymphedema is suction-assisted lipectomy. Candidates for this procedure have, along with lymph fluid, a large buildup of fibroadipose tissue to be removed. The minimally invasive procedure takes one to two hours, with a hospital stay of less than 24 hours. Patients must continue to wear compression garments right after surgery to prevent a recurrence.
“Most think of lymphedema as a plumbing issue. If a breast cancer patient had lymph nodes removed under their arm, there will be a blockage up there — but that’s really only part of the problem,” Dayan says. “There’s a whole other disease process that is triggered in some patients, where the immune system spews out harmful chemicals that scar the lymphatic vessels and lead to fat buildup. Massage and compression can relieve some things, but they cannot get rid of fat and scar tissue buildup.”
Although some evidence supports the effectiveness of lymphedema surgery, questions remain regarding which patients will benefit, how often the procedure works and why. As a result, insurance companies consider the procedures investigational and do not universally cover them. Dayan says that most lymph node transplants have been covered, but lymphovenous bypass tends to be more of a gray area.
Strategies to Keep Fluid Flowing
Many experts believe the future of lymphedema research lies in prevention rather than treatment. Because the main risk of developing cancer-related lymphedema is the removal of lymph nodes, doctors have been exploring ways to reduce the damage to the lymphatic system while treating cancer, such as removing 20 to 30 axillary lymph nodes instead of 40 to 50 or forgoing axillary node dissection.
“Many patients don’t need to have axillary node dissections, and we can also reduce the number of patients who have sentinel node biopsies,” says Dr. Sheldon Marc Feldman, chief of breast surgery at Montefiore Health System. “The reason for doing lymph node removal or biopsies is for staging of breast cancer to help us determine whether it has spread or not. That’s still important, but we can also learn a lot from a needle biopsy.”
Feldman has also performed a novel preventative procedure called LYMPHA (lymphatic microsurgical preventive healing approach) in about 50 patients in the United States since 2013 and continues to offer it at Montefiore, as well as teaching it to other breast surgeons across the country. The microsurgical tech- nique occurs at the time of lymph node removal and essentially involves a patient undergoing lymphovenous bypass ahead of time. Instead of tying off the lymphatic vessel after lymph node removal, Feldman connects it directly to a blood vessel so the lymphatic flow can continue. A study he conducted in 37 women at high risk of lymphedema found that nearly 90% avoided the condition after undergoing LYMPHA, whereas a control group whose members did not undergo the procedure had a 40% rate of lymphedema.
“Prevention is always better than treatment in any disease process. What’s very helpful for patients to know is, if they are going to have breast cancer surgery and their lymph nodes removed, they should understand why the lymph nodes are being removed,” Feldman says. “It used to be a very standard thing, but in some situations, it isn’t going to be helpful.”
Patients should ask about alter- natives to lymph node removal, including preoperative ultrasound and needle biopsy, or consider sentinel node biopsy rather than a complete axillary node dissection. They can also do research on more novel, investigational techniques for lymphedema prevention, such as LYMPHA.
Fonfa hasn’t thought about getting surgery for her lymphedema, which she mostly keeps under control with a healthy diet and exercise. Cooper realizes that the lymphovenous bypass wasn’t a cure — she still wears compression sleeves and performs lymphatic drainage on herself every day with the help of a machine — but her condition is much improved.
“I was extremely skeptical before meeting with Dr. Patel, because every doctor with whom I had ever consulted told me there was no surgery for this. But with the encouragement of family and friends, I did talk to him, and for the first time, I felt like I had some hope,” Cooper says. “Now, the word ‘hope’ is back in my vocabulary, and I hope this surgery becomes more well-known and brings relief to others.”