Head and neck lymphedema is a very common side effect of head and neck cancer treatment. It is a chronic condition with potentially severe implications for quality of life. In my own experience as a physiotherapist and Combined Decongestive Therapist who has treated patients with various types and stages of lymphedema over the past decade, head and neck lymphedema patients can be among the most heavily impacted by their condition.
Unfortunately, general knowledge of the disease is poor and clinical studies remain sparse. In this article I will offer you an overview of our current clinical understanding of the condition, along with the accepted best practices for managing it.
Want to know how your experience compares to that of other patients? Check out: “Is my head and neck lymphedema ‘normal’?”
Part 1. Clinical characteristics of head and neck lymphedema
What is lymphedema?
Lymphedema is a chronic swelling condition caused by damage to the lymphatic system (“secondary lymphedema) or malformation of the lymphatic system (“primary lymphedema”). Disrupting normal function of the lymphatic system in an area of the body results in insufficient clearance of lymphatic fluid (lymph) from that area. When blood transports oxygen and nutrients to the cells of the body and collects waste products for excretion, 80% of the fluid returns to the bloodstream while 20% enters the lymphatic system as lymph, a colourless fluid constituting this left over volume. The lymph eventually makes its way back into the blood stream at the heart.
More than just a fluid collection system, the lymphatic system is a vital component of the immune system, transporting immune cells around the body. Consequently, blockage of lymph flow causes a build up of fluid as well as other harmful changes including localized immune depression. Lymphedema is typically observed in the extremities (arms and legs), but is also very common in persons treated for head and neck cancer. Learn more about lymphedema here.
What is head and neck lymphedema and how is it caused?
Secondary lymphedema of the head and neck typically arises as a result of damage to the local lymphatic system during cancer biopsy or treatment (see Fig.1 for an illustration of some of the lymph nodes and vessels of the head and neck). Removal of lymph nodes during cancer biopsy or tumour resection is the primary cause of secondary lymphedema in head and neck cancer patients. This is a direct consequence of the essential role played by lymph nodes in collecting lymph fluid from smaller lymphatic vessels and draining it into larger vessels leading back to the heart.
Radiation therapy can also cause damage to any remaining lymph nodes and lymphatic vessels located in the radiation field, another potential cause of lymphedema. Unfortunately the extensive number of nodes in the head and neck makes radiation induced damage more difficult to avoid. Obstruction of normal lymphatic flow in the head and neck results in a build up of lymphatic fluid, and subsequently a chronic and progressive swelling that can affect various areas of the head, neck, and face.
As with lymphedema of the arm or leg, head and neck lymphedema should be caught and treated early to relieve symptoms and prevent or delay disease progression.
Unmanaged swelling causes additional damage by overtaxing the remaining normal lymphatics, and initiates a cascade of deleterious events including localized inflammation, immune system impairment, and eventually a hardening of the swelling (which is called “non-pitting lymphedema”), further exacerbating the issue.
Hardening is the outcome of compositional changes that occur in the tissue including fibrosis (scarring), connective tissue proliferation, and the accumulation of fat (and to a lesser extent muscle and bone) – a possible result of altered stem cell behaviour. This advanced stage of the disease is less responsive to typical treatments, and may instead require surgical intervention (see more below).
What are the symptoms of head and neck lymphedema?
The primary symptom of head and neck lymphedema is swelling that typically develops 2-6 months after cancer treatment. During the early stages of head and neck lymphedema swelling may be difficult to observe. Head and neck lymphedema can develop internally in the larynx and pharynx (which constitutes various structures that comprise the mouth and throat), or externally in the neck and face, or a combination of both, depending on which lymphatic channels are obstructed.
Less frequently, swelling can progress to the point where it creates serious functional problems such as difficulty breathing and swallowing, as well as the obvious body image concerns and associated social withdrawal arising from deformation of the neck and face (ref. 2). Overall, head and neck lymphedema has the potential to severely degrade the quality of life of affected individuals. To see how your symptoms compare to a group of 1202 patients treated for head and neck cancer at the MD Anderson Cancer Center (MDACC) in Texas, check out our post: “Is my head and neck lymphedema ‘normal’?“.
Symptoms of mild to moderate head and neck lymphedema may include:
- Visible swelling under the chin, in the neck, or in the face including eyes and lips.
- A feeling of tightness or restricted movement in these areas, including the jaw or shoulders.
Symptoms of moderate to severe head and neck lymphedema may include:
- Pronounced swelling in the face and neck
- Swelling feels hard and solid to the touch
- Visual or auditory impairment
- Difficulty breathing, swallowing, eating or speaking
- Nasal congestion, chronic ear ache
How is head and neck lymphedema diagnosed?
Lymphoscintigraphy or other imaging techniques involving the injection of contrast agents to visualize lymphatic fluid flow abnormalities can lead to a definitive diagnosis of lymphedema, but it is commonly diagnosed using less evasive means. The presence of lymphedema is typically assessed based on observational findings from a physical examination, patient symptoms, and a review of patient history. Ruling out alternative causes of swelling is necessary. Accordingly, early stages of the disease can be especially difficult to diagnose.
Subjective findings from the patient (such as feelings of ‘tightness’ or ‘heaviness’) are often the earliest indicators, whereas objective measurements of volume changes can be more difficult to obtain. This is particularly true for early internal lymphedema, and early external lymphedema in areas such as under the chin where there is no symmetrical counterpart that can be used for comparison (unlike for example swelling on one side of the face that can be compared to the other unaffected side).
External lymphedema is often classified according to one of the four common scales. However, standardization is lacking, and these scales appear to be imperfectly suited to capturing the stages of head and neck lymphedema (ref. 3).
A modification of Foldi’s scale for categorizing external lymphedema does a good job at capturing the range of soft-tissue changes observed during progression of untreated head and neck lymphedema (see Table 1), although further validation of the scale is warranted (ref. 4).
Practically speaking, head and neck lymphedema secondary to cancer treatment can be defined as swelling that develops three or more months following treatment (ref. 5). This is beyond the time that normal acute post-treatment swelling is typically seen.
External lymphedema is identifiable by a visible swelling of the face or neck, and can be best quantified using a “composite facial score” comprised of seven measurements, as well as a “composite neck score” comprised of 3 measurements (ref. 4). Measurements are necessary to establish a baseline, monitor changes and track improvement.
In contrast, internal lymphedema is identified by a visible swelling in the mucosa and soft tissue of the pharynx and larynx. Internal lymphedema is more difficult to quantify. Endoscopy or mirror examination by an experienced practitioner can be used to measure 11 internal anatomical sites and two spaces according to the Patterson’s scale (ref. 5).
Risk factors for head and neck lymphedema following cancer treatment
Fundamentally, the risk of lymphedema increases as a function of lymphatic damage. Possibly the best single predictor of likelihood is the number of lymph nodes that were removed during surgery; the more nodes removed, the greater the chance of developing head and neck lymphedema. Studies of head and neck lymphedema suggest that total dose of radiation therapy as well as number of days of radiation also increases your risk of developing lymphedema (ref. 6).
In addition to the number of lymph nodes removed, additional factors may modify your chances of developing head and neck lymphedema. Studies of arm lymphedema secondary to breast cancer suggest that the chance of lymphedema increases for patients who:
- Have received chemotherapy before or after surgery (ref. 7),
- Have high body mass index (BMI)
- Have genetic factors associated with lymphedema
- Report lower overall health scores (ref. 7)
It is possible that these same risk modifiers may apply to head and neck lymphedema following cancer treatment. Thankfully, there is also research to suggest that there are steps you can take that may help reduce your risk of developing lymphedema (read below).
Prevalence of head and neck lymphedema following cancer treatment
Head and neck cancer makes up only 3-5% of all cancers (ref. 4), but within this population the incidence of head and neck lymphedema is considerable. It is commonly reported that the incidence of head and neck lymphedema following treatment for head and neck cancer is around 50%. This figure appears to be derived from four European studies that suggested between 12 to 54% of patients with head and neck cancer will go on to develop lymphedema as a side effect (ref. 8-11).
Recent research suggests that these earlier studies may actually underestimate the real prevalence of head and neck lymphedema because they did not comprehensively assess both internal and external lymphedema (ref. 5).
A larger and more comprehensive study of both internal and external lymphedema suggests that the prevalence of head and neck lymphedema following cancer treatment may be an alarming 75.3% (61 of the 81 study participants) (ref. 5). This included: 9.8% with external lymphedema only, 39.4% with internal lymphedema only, and 50.8% with combined external and internal lymphedema. This more comprehensive study was still limited by a non-robust study design (it was not a prospective study where patients were followed over time), and was performed on primarily a Caucasian population at a single large cancer center in the United States.
Part 2. Prevention and treatment of secondary lymphedema of the head and neck
Prevention and self-management of head and neck lymphedema following cancer treatment
Damage to lymphatic nodes and vessels causes a predisposition to developing lymphedema. However, many individuals with lymphatic damage do not go on to develop lymphedema, and many more develop only mild lymphedema or enjoy many years without occurrence.
Prevention of lymphedema onset and minimization of its effects can be helped by reducing lymphatic stressors. In one study it was reported that early intervention could restrict mild lymphedema (of the arm) to low levels for at least 10 years (which was the full length of the study), and likely longer (ref. 12).
Head and neck lymphedema prevention and management strategies:
- Reduce the risk of injury and infection to your head and neck. Injury and infection causes local inflammation which increases lymphatic load in the area. This includes: sunburns and other burns; bites and scratches from insects and animals; bruises and injuries; and cuts, including inside the mouth. With respect to personal grooming, precautions should be taken to avoid breaking the skin barrier. Men should consider switching to an electric razor rather than razor blade, and women should be careful with waxing and electrolysis. All cuts should be washed, treated with an antibiotic ointment, and monitored for infection. Signs of infection should be investigated by a physician as quickly as possible and will likely be treated with antibiotics.
- Consider wearing a compression garment. Compression garments are a central component of Combined Decongestive Therapy (CDT), the gold standard conservative treatment for lymphedema (more on this below). Compression garments should be recommended during the period immediately following head and neck cancer treatment (especially if lymph nodes were removed or irradiated), as well as following cosmetic liposuction of the neck and chin. Use of compression immediately following surgery can help reduce temporary post-surgical swelling and improve aesthetic outcomes. It may also help prevent transient swelling from becoming chronic. For patients with established lymphedema, regular use of compression garments can help prevent the condition from worsening. Keep in mind that compression garments can only benefit the tissues that they compress and offer no benefit for swelling in inaccessible areas such as at the back of the throat.
- Self-prescription of compression garments is not optimal. You may benefit most from a certain level of compression, a specific style, or only after swelling volume reduction using manual lymphatic drainage. A CDT therapist will be able to best advise you on which garment would be best for you (if any), as well as when and how often to wear it. However, if you have difficulty accessing care, wish to be proactive, or have recently had surgery, you may consider purchasing a generic and relatively inexpensive compression garment such as the Jobst Facioplasty. Size small is a common size for this particular garment, fitting people with a crown to chin circumferences of under 25 inches. [Small disclaimer: if you purchase an item through this link we may receive a very small fee which we use to help support our blog. But feel free to look around for a garment that suits you best, better yet, ask a local CDT therapist for personalized advice before purchasing a garment.]
- If you do wear compression, wash it every one or two days, by hand, to help it maintain its shape and elasticity. Discontinue use immediately if it causes pain, friction or discomfort, if you experience skin sensitivity or show signs of infection, or if you experience an increase in swelling anywhere in the area.
- Exercise regularly following the guidelines provided by your CDT therapist to help aid lymph drainage.
- Maintain a healthy body weight.
- Ensure ergonomic sitting positions (particularly when using a computer, for example) to reduce tightening of the neck and shoulder muscles and ensure optimal lymphatic flow through this critical area. Maintain low stress levels which can cause tightening in these muscles as well.
- Try sleeping with your upper body slightly raised. You may experience worse symptoms in the morning, which subside after being upright and active. Gravity appears to assist in lymph drainage of the head and neck, which is in contrast with lymphedema of a limb, where symptoms more often worsen with time spent upright.
- Moisturize skin to prevent drying.
- Wear loose clothing. Do not wear clothing or jewelry that is constrictive around the neck.
Basic manual lymphatic drainage massage techniques can be learned by the patient and performed at home. Here I demonstrate a basic series performed at the neck:
Professional management of head and neck lymphedema following cancer treatment
The gold standard treatment
The gold standard treatment for lymphedema is complete (or ‘combined’) decongestive therapy (CDT). CDT is provided by certified therapists, and includes manual lymphatic drainage, compression (bandages / garments), exercise program development, and education on skin care and infection precautions. Patient education should also include self-massage guidance, and flare-up prevention practices. Learn more about CDT here. Standard CDT practices may need to be modified to optimally treat this population, in particular compression which can be especially difficult in this region.
Seeking guidance and care from a CDT therapist should be done as soon as symptoms present. It has been suggested that CDT is most effective for early and milder cases of lymphedema (in this case for breast cancer related lymphedema) and as mentioned above, early intervention in the disease process may help limit lymphedema to low levels for an extended period of time.
Preferably, head and neck cancer patients should meet with a CDT therapist pre-emptively, before symptoms are present. Judicious adherence to preventative strategies combined with self-management guidance including exercise and self-massage may prevent the onset of lymphedema. CDT therapists should be able to offer this guidance in a single session, before, during, or after cancer therapy.
For advanced cases of lymphedema where head and neck swelling is extensive (ie the hardening process has occurred), CDT therapy may be less effective, and in some cases insufficient to adequately alleviate symptoms. Given the potentially severe consequences of unmanaged swelling in the face and neck (including difficulty breathing, swallowing, speaking, etc.), patients with advanced head and neck lymphedema should be closely monitored by their physician, and surgical intervention should be considered if feasible and necessary.
While there is currently no surgical solution for lymphatic insufficiency, a variety of surgical procedures have shown some benefit for relieving swelling and improving lymphatic function. Surgical options (if available in your area) may include liposuction (see: “Liposuction for Head and Neck Lymphedema – Does it Work? For Which Patients?“) to remove accumulated lymph and adipose tissue (ref. 13) or lymphaticovenous anastomosis (see: “Lymphatic Reconstructive Surgery for Lymphedema: No Cure Yet“)(ref. 14) where non-draining lymphatic vessels are surgically connected to adjacent veins to provide an alternative route for lymph drainage.
Pharmaceuticals and supplements
There are currently no medications or dietary supplements available to treat head and neck lymphedema. Given that lymphedema is caused by a mechanical disruption of normal lymphatic function, pharmacological ‘cures’ are not yet available – and will likely require significant medical advancements toward ‘regenerative’ medicine before they are.
However, other factors in addition to the mechanical issue can also contribute to the symptoms of head and neck lymphedema – such as inflammation – which may be interesting targets to help symptom management in some people.
If you search google you may find mention of a variety of supplements or compounds that some people believe may help reduce the symptoms of lymphedema. Unfortunately these are not yet supported by clinical research (and may never be), and so they are not included in recommended clinical management guidelines.
Three of the more interesting experimental compounds for treating the symptoms of lymphedema are: selenium (sodium selenite), Pletal (aka cilostazol) and Ubenimex (aka bestatin).
Here’s what we know so far about these experimental compounds and how they might improve the symptoms of lymphedema:
- Selenium is an essential mineral found in our bodies and in the food we eat. Based on our review of the clinic data, we find the use of selenium for lymphedema to be controversial, impractical, and potentially unsafe.
- Pletal is an FDA approved drug that can help decrease muscle pain in intermittent claudication. It has been shown to promote growth of lymphatic cells in mice and in human lymphatic cells grown in a laboratory, so it could potentially promote lymphatic repair in lymphedema. Could cilostazol be a treatment for lymphedema? We don’t yet know. Clinical trials are lacking, and any potential benefits must be weighed against the significant side effects of taking this drug.
- The cancer drug Ubenimex could be more interesting, and is currently approved in Japan as a cancer treatment. Ubenimex is a potent inhibitor of a particular inflammatory pathway that was recently shown to be involved in lymphedema development in an experimental mouse model of lymphedema. As far as I am aware, Ubenimex is the only drug candidate that is currently undergoing clinical trials (the ‘ULTRA’ trial) sponsored by a pharmaceutical company. It is an early clinical study of efficacy and safety in patients with secondary lymphedema of the leg. We recently examined the data behind this drug to help better understand how Ubenimex might help patients with lymphedema.
Comments or Questions?
If you have any comments or questions, please feel to share or ask below! As you know, the lymphedema community is small (and the head and neck lymphedema community is obviously even smaller), so sharing your experiences or tips could help others. We have many people visiting this page each day, so your efforts will not go unnoticed. “Sharing is caring” :)
Want to know more about how your experience compares to other patients? Check out our article: “Is my head and neck lymphedema ‘normal’?”
- Gray’s Anatomy illustration: http://www.bartleby.com/107/illus602.html.
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- Smith B.G. and Lewin J.S. The role of Lymphedema management in head and neck cancer. Curr Opin Otolaryngol Head Neck Surg. 2010 Jun; 18(3): 153-8. link to article
- Deng J. Ridner S.H. et al. Prevalence of secondary lymphedema in patients with head and neck cancer. J Pain Symptom Manage. 2012 Feb; 43(2): 244-52. https://doi.org/10.1016/j.jpainsymman.2011.03.019
- Deng J. Ridner S.H. et al. Factors associated with external and internal lymphedema in patients with head-and-neck cancer. Int J Radiat Oncol Biol Phys. 2012 Nov1; 84(3). https://doi.org/10.1016/j.ijrobp.2012.04.013
- Miaskowski C., Dodd M., et al. Lymphatic and Angiogenic Candidate Genes Predict the Development of Secondary Lymphedema following Breast Cancer Surgery. PLoS One. 2013 Apr 16;8(4). https://doi.org/10.1371/journal.pone.0060164
- Dietz A., Rudat V., et al. Chronic laryngeal edema as a late reaction to radiochemotherapy. [in German]. HNO. 1998; 46: 731-38. abstract
- Buntzel J., Glatzel M., et al. Influence of amifostine on late radiation toxicity in head and neck cancer follow-up study. Anticancer Res. 2007; 27: 1953-56. http://ar.iiarjournals.org/content/27/4A/1953.long
- Schiefke F., Akdemir M., et al. Function, postoperative morbidity, and quality of life after cervical sentinel node biopsy and after selective neck dissection. Head Neck. 2009; 31:503-12. https://doi.org/10.1002/hed.21001
- Wolff H.A., Overbeck T., et al. Toxicity of daily low dose cisplatin in radiochemotherapy for locally advanced head and neck cancer. J CancerRes Clin Oncol. 2009; 135:961-67. http://doi.org/10.1007/s00432-008-0532-x
- Johansson K, Branje E. Arm lymphoedema in a cohort of breast cancer survivors 10 years after diagnosis. Acta Oncol. 2010; 49(2):166-73. https://doi.org/10.3109/02841860903483676
- Taylor S.M., Brake, M. Liposuction for the management of submental lymphedema in the head and neck cancer patient. Otolaryngol Head Neck Surg. 2012 Jun: 146(6): 1028-30. https://doi.org/10.1177/0194599812438337
- Mihara M., Uchida G., et al. Lymphaticovenous anastomosis for facial lymphoedema after multiple courses of therapy for head-and-neck cancer. J Plast Reconstr Aest Surg. 2011 Sep; 64(9): 1221-5. https://doi.org/10.1016/j.bjps.2011.01.006