Clot Chronicles: Evaluating and Managing Lymphedema

Hi, this is Clot Chronicles, and today we’re going to be discussing lymphedema evaluation and management. My name is Brett Carroll. I’m the Director of the Section of Vascular Medicine at Beth Israel Deaconess Medical Center. 

I think it’s so important for vascular medicine physicians to be familiar with the evaluation and management of lymphedema, particularly because we see a lot of patients that have a prior history of DVT and have developed post-thrombotic syndrome. Sometimes it can be difficult to differentiate what’s really driving their underlying symptomatology, and often the treatment overlaps with post-thrombotic syndrome and lymphedema. So, a lot of this can be geared to the general vascular medicine physician as well. 

It’s important to understand a little bit about the pathophysiology of lymphedema to fully embrace why lymphedema is such a problem for patients, and it really is more than just a plumbing problem. This is a microscopic view of lymphatic vessel, and you can see at the top is the collecting ducts that drain the fluid down into the collecting lymphatics that have their own pumps and valves. 

Over time when there’s a disruption in lymphatic flow—whether it’s from a lymphadenectomy, an infection, trauma, or just primary congenital lymphatic dysfunction—you develop inflammation. And this inflammation, over time, leads to thickening of the basement membrane, impaired flow of the fluid, and also fat deposition in thickening of the skin. And these, between the inflammation and these changes that occur over time, lead to a fair amount of morbidity in terms of the symptoms patients develop with heaviness and fullness, discomfort, along with the skin changes that are common with lymphedema. 

This is a microscopic view of the skin changes that occur. You have hypercellularity of the dermal/epidermal junction, thickening of the epidermis, and hyperkeratosis, an expansion of the subcutaneous adipose tissue. And that’s important, as I’ll note, when we start thinking about the management of these patients, how much adipose tissue does get infiltrated into the affected limb. There’s also dermal fibrosis. 

So, these are just two examples of patients with very large limbs due to lymphedema. This patient here following breast cancer treatment with a very large left arm compared to right arm, and here’s a patient with primary lymphedema with a right leg that’s probably more than twice the size of their left leg. When we think about how best to manage these patients, really a multidisciplinary approach is best, which may include members from vascular medicine, physical therapy, radiology, plastic surgery, and adjunctive services like infectious disease or vascular surgery. 

When we reviewed the first 600 or so patients that came through our clinic, we found that though most patients did have lymphedema, about 15 to 20% did not have lymphedema. And of those with lymphedema, almost a quarter had a multifactorial cause of the lymphedema. So, it’s important to go through the process of making sure that the patient actually has lymphedema, if that’s why they’re being referred, because there are a variety of other things that can cause similar symptoms. 

That’s where objective evaluation with imaging comes in. This is a nuclear lymphoscintogram where we inject a tracer in the feet. We watch how it travels up the limbs. You can see here that patient’s left leg is much larger than their right, and they have good uptake of the tracer in their right groin and no uptake on the left. There’s also growing utilization of near infrared fluorescence or NIRF. This is also done with an injection intradermally, and we can see how there is flow. This would be normal channels up here. And then in the lower panels, disruption in flow and eventually we get this starry sky stardust appearance, which demonstrates that the tracer’s really not getting pulled in by those lymphatic capillaries into the lymphatic collecting tubules. 

There’s also a role for invasive lymphangiogram in patients – maybe more so in those with primary lymphedema to better define their anatomy. MRI is increasingly being used as well, though MR lymphangiograms can be performed to evaluate the lymphatics themselves. They also offer a nice view of the limb kind of below the skin and demonstrate how much is fluid and how much is fat. So, you can see in this patient a much larger limb on their left side – we can see a lot of fluid but also a fair amount of fat hypertrophy as well is contributing to the increased diameter of their limb. 

So, when looking at lymphedema treatment—really the patient’s obviously at the center—but you need both an adequate medical assessment, physical therapy is so key, social support, hygiene exercise, and in appropriate patients, a surgical assessment. So, initial therapy is really surrounded by complete decongestive therapy. Phase 1 is the reduction phase. This is where you’re really trying to reduce the degree of edema in the arm, and then phase 2 is the maintenance phase where you’re trying to maintain what had been alleviated in the decongestion phase. And that’s something patients will continue on chronically, which tends to involve a very well-fit custom garment if the patient can afford that; unfortunately, it’s not covered usually in the US. 

There’s an extremely large patient role in performing physical therapy. Although the physical therapists are phenomenal, the patient has to take on a lot of work at home as well to maintain the results. I say a good-fitting garment is always better than the best-fitting garment. Sometimes the best-fitting garment the patient won’t wear – it’s too tight, it’s too uncomfortable. You’ve really got to find something that the patient will tolerate. I’d rather them wear something most of the day that is maybe not perfect than have them wear something that’s perfect just a couple of hours a day. So, working with physical therapy, patients can find a variety of garments that may be good options. 

To jump on the surgical options a little bit, there are really two big buckets – physiologic procedures and debulking procedures. The physiologic procedures include a lymphovenous bypass (or an LV bypass). This includes anastomosis of a lymphatic vessel which is proximal to the obstruction, for example, after axillary lymph node dissection. The obstruction is in the axilla so an LV bypass is performed down lower in the arm to try to reroute the lymphatic drainage out of the arm via the venous system. It has a modest reduction in symptoms and volume; it’s unclear if it has any reduction in cellulitis. But patients may also see a slight decrease in their need for compression. 

A lymph node transplant is a more invasive procedure; it involves moving lymph nodes and is most commonly done now out of the mesenteric region into the affected limb. It’s believed this new lymph node “hub” grows its own lymphatic network and helps drain fluid through that new hub that’s developed. You generally have a significant decrease in symptoms, a decrease in volume, and a decrease in cellulitis with the largest impact to a patient’s quality of life being the reduction in need for compression. 

Debulking, rather than going after the lymphatic dysfunction itself, tackles the downstream effect of having lymphedema with that fat hypertrophy that has accumulated. This is a dedicated liposuction for the limb. This involves a significant reduction in symptoms and volume. The downside is patients need to remain in compression—essentially 24 hours a day—or the fluid will slowly reaccumulate over time. 

This is a systematic review of 18 studies, including about 300 patients, that demonstrates the effect of a lymph node transplant. You can see about 90% of patients had a smaller limb by circumference and volume, and improved flow in lymphoscintigraphy. Patients were, generally, satisfied, and about half the patients had a decreased need for compression. The majority that had previously had infections had a decrease in their frequency of infections. 

Debulking, like I said, involves a dedicated liposuction that’s done with a tourniquet around the proximal portion of the limb to maintain a decrease in bleeding. A large amount of fluid and fat is removed, which is generally done by a plastic surgeon with particular experience in this type of liposuction. It’s different than what we think of, generally, as liposuction. They have special cannulas that are made specifically for this role. You can see here the dramatic change in this gentleman’s girth of his left lower extremity. It was twice—maybe three times—the size of his right lower extremity. And here following the procedure, near equalization of the size of the limb. 

This is a study out of our center that looked at about 30 patients that had undergone debulking with liposuction. You can see improved quality of life for just about everybody. The average volume debulked in the upper extremity was just under a liter of fat; and in the lower extremity, 2.5 liters of fat. So, significant changes in volume, undoubtedly, at one year. 

So, to wrap up, developing a lymphedema program involves multiple resources within your center to optimize care, which is so important because it’s such an unmet need. We see many patients that have been floundering and haven’t had a good medical assessment for sometimes going on decades. Like I mentioned, it’s important to involve as many different services as you have available – especially physical therapy, as they’re really the ones doing a lot of the work with the patients, sometimes seeing the patients several times a week to maintain the volume reduction that can occur with good compression and decongestive therapy.

It’s important to focus on the patient’s goals and needs and understand what bothers them about lymphedema the most and how you can try to get them to their ultimate goals. And that may include the growing surgical options that are available. Thank you for your time and attention.