What’s different about head and neck lymphedema? Evaluation and treatment of an often overlooked lymphedema
By Brad Smith
Likelymphedema elsewhere in the body, head and neck lymphedema (HNL) is chronic, progressive swelling that occurs due to an impaired lymphatic system. HNL can occur anywhere in the face, oral cavity, or neck, and while it can be a component of certain genetic conditions, primary lymphedema affecting the head and neck region is rare. Most commonly, HNL develops after treatment for head and neck cancer (HNC) with surgery and/or radiation, though it can also arise after chronic skin infections (cellulitis) or other sources of soft tissue and lymphovascular trauma. The focus of this article is the evaluation and management of HNL following cancer treatment.
Head and neck cancer
The head and neck region is anatomically very complex, featuring many structures crucial to breathing, swallowing, speech and voice production, vision, hearing, and other functions. A complex matrix of blood and lymphatic vessels, lymph nodes, nerves,
structures, and other soft tissues are interconnected in a relatively small space and must work seamlessly for the body to function normally. Cancer of the head and neck region may arise in one or more key areas.
Early and accurate diagnosis
Head and neck cancer is rare, accounting for approximately 4% of all cancers diagnosed each year. Individuals who are concerned that they may have head and neck cancer should be evaluated by an otolaryngologist/head and neck surgeon who has experience with cancer management to determine the proper diagnosis and treatment.
Surgery for HNC
The initial decision to treat tumours that arise in this area with surgery, radiation, or chemo-radiation will be based on the tumour location, size, presence of neck disease, and the expertise of the treating physicians. Surgery must remove the mass with the least amount of collateral damage possible, preserve function of the affected structures if possible, and minimize disfigurement.
Brad Smith is a speech-language pathologist and certified lymphedema therapist at the Baylor Sammons Cancer Center in Dallas, Texas. He is a frequent instructor at state, national, and international conferences, has authored a textbook chapter, and co-authored two peer reviewed articles regarding evaluation and management of head and neck lymphedema.
drainage, essentially damming the lymphatic flow and creating areas of “pooling” or outpouching above the scar, sometimes referred to as a “trap door effect”. This pattern of swelling can be more persistent than swelling that arises without scarring and may be more difficult to treat, since the typical drainage routes used during manual lymph drainage (MLD) treatment may not be as effective, requiring an alternative pathway.
In addition to major surgeries, surgical removal of lymph nodes (lymphadenectomy or neck dissection) is commonly performed in this population due to either the presence of active disease in the cervical lymph nodes or to prevent high-risk disease from spreading into the neck. While the intent of neck dissection is to prevent disease spread, risk of lymphedema is often increased due to the removal of the lymph nodes, ligation of the lymphatic vessels, and the associated neck scarring.
Typically, the more extensive the surgery, the greater the risk for HNL development. However, the neck contains more than 1/3 of the body’s lymph nodes, which create a very effective drainage system that allows excellent recovery of function in most cases. It is not uncommon for patients to undergo a neck dissection involving >40 lymph nodes and still not develop HNL. Interestingly, HNL is not commonly observed after non-cancer related surgeries involving the face or neck, presumably since the lymph nodes are not typically involved.
Chemotherapy
At this time, there is not enough evidence to suggest a direct correlation of HNL with either chemotherapy or immunotherapy, unlike the limbs, which have been documented to develop lymphedema after treatment with Taxane based chemotherapy. While there have been published accounts of facial edema following chemotherapy for lung cancer, there are no publications documenting HNL after receiving any type of chemotherapy for treatment of HNC in isolation. Likewise, there is currently not enough data to assess the impact of chemotherapy on HNL in those patients who received it in combination with radiation treatment.
Radiation
Typical radiation treatment regimens range from 5-7 weeks, 5 days per week. During this process, most patients experience a somewhat predictable series of progressive side effects that usually begin at the midpoint of treatment. These include but are not limited to erythema (redness) and
peeling of the skin, dry mouth (xerostomia), taste changes (dysgeusia), mouth sores (mucositis), pain while swallowing (odynophagia), difficulty swallowing (dysphagia), malnutrition, dehydration, and weight loss. HNL is a common complication following radiotherapy, commonly developing between 8 and 12 weeks after completion of treatment. It is thought that the radiotherapy creates more diffuse tissue damage than surgical intervention and that it requires a longer period of time for tissues to begin thickening and become more firm, reflecting decreased fluid drainage and onset of lymphedema.
Prevalence of HNL
While a certain degree of edema is usually expected after surgery or during the acute phase of radiotherapy (when the skin can be severely damaged), it is anticipated that most post-treatment edema will resolve within the first 4-6 weeks. Swelling that persists beyond the expected timeframe after surgery or develops several weeks after completion
of radiotherapy is typically considered head and neck lymphedema (HNL). However, it is important that other sources of edema, such as allergic reactions, infection, venous obstruction, or tumour recurrence are ruled out before lymphedema treatment is initiated since not all swelling is lymphedema and it is not always appropriate to provide lymphedema treatment when swelling is observed.
HNL is encountered much less often than edema elsewhere in the body. This is because cancer of the head and neck accounts for approximately 4% of all cancers diagnosed each year and not all patients who undergo treatment for head and neck cancer will develop HNL. According to a study of 81 patients from Vanderbilt University, head and neck lymphedema was noted in up to 75% of patients who completed treatment for head and neck cancer at least 3 months earlier. All patients were evaluated with traditional external assessment methods and also endoscopic assessment. Results indicated 50% presented with edema in the external neck or face, AND internally within
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the posterior oropharynx or larynx. Only 9.8% presented with solely external edema, suggesting that many patients who are seen for traditional lymphedema management may have undiagnosed internal edema, since most evaluating therapists do not perform endoscopic assessments. An older study looking at a larger cohort of >800 patients reported 48% of their study group developed head and neck lymphedema after treatment for cancer of the head neck. Given these statistics, the number of people who will be diagnosed with head and neck lymphedema each year equates to approximately 1-2% of all patients diagnosed with cancer. As a result, head and neck lymphedema is not frequently encountered by most physicians or therapists. This lack of exposure sometimes results in decreased familiarity and misperceptions regarding the treatability of HNL.
However, interest in the management of head and neck lymphedema is increasing due to an unfortunate rise over the past 10-15 years in the number of patients diagnosed with oropharynx (posterior mouth and throat) cancer related to the human papilloma virus (HPV). It is anticipated that this rising trend of HPV+ oropharynx cancers will continue over the next 10-20 years. Since this type of cancer is most frequently treated with chemotherapy, radiation, and sometimes surgery, there will likely be increased demand for skilled lymphedema therapists who can treat HNL. This trend has already been observed, since this patient population is typically younger, between the ages of 40 and 60, and generally healthy, with many of them returning to work after their cancer treatment. Among this demographic, there is a decreased tolerance of disfigurement and disability, prompting more patients to request treatment for head and neck lymphedema in recent years.
HNL is different
Lymphedema of the head and neck is not identical to lymphedema elsewhere in the body. While lymphedema in the arm or leg is typically considered a lifelong condition, mild to moderate HNL typically responds very well to treatment, especially when manual lymph drainage (MLD) and compression are started during the early stages of swelling. Like edema elsewhere in the body, once the
tissues become firm and fibrotic, reversal of the edema becomes more difficult and swelling remains more persistent with more chronic functional impairment. When edema affects the lips, tongue, eyes, or throat, there may be substantial impairment of speech, swallowing, breathing, or tasks like walking or driving when vision is affected. Severe lymphedema affecting the head and neck is quite difficult to hide and aside from the functional deficits it creates, there may also be increased levels of anxiety and self-consciousness, sometimes resulting in isolation and decreased socialization. As a result, head and neck lymphedema can have severe consequences and its treatment is of utmost importance.
and neck measurement and staging protocols that are appropriate for this patient population. In addition, brief assessment of speech, swallowing, cervical range of motion, and upper extremity function are also important for patients with head and neck lymphedema. Since HNL can affect the oral cavity, efforts should be made to assess the lips, tongue, and remainder of the mouth in order to identify any edema that could be missed with a cursory examination. Patient feedback is of utmost importance, since the patient may perceive swelling that may be missed by the examiner with tactile and visual assessment.
HNL treatment
HNL evaluation
Once diagnosed with head and neck lymphedema by their physician, patients should undergo a thorough lymphedema evaluation prior to initiating any treatment. Ideally, this will occur during the early stages of swelling in order to provide the best chance for reversal and elimination of the edema. The evaluation should include a thorough case history to ensure that the edema is not related to a different etiology like allergies or infection, which require medical intervention. It is critical to obtain documentation of both size and firmness of the affected tissues using tape measurements and photographs to document baseline appearance and function, as well as changes over time. There are several published head
The approaches used for manual lymph drainage for the head and neck are slightly different than those used for the limbs and trunk, but the general premise is the same. It is important that the trunk be decongested before the edematous face and neck are treated in order to provide an adequate drainage pathway. Typically, the drainage target for patients with HNL is the axillary lymph nodes bilaterally. In cases when the axillary nodes are unavailable, drainage may be rerouted to the opposite axilla or inferiorly to the inguinal lymph nodes. The drainage pathway utilized during MLD will depend on the severity of scarring within the neck, but in general, decongestion is performed in the trunk first, then the neck, face, and oral cavity if required. The sequence is then reversed and followed by the use of compression padding, typically held in place by a compression garment or bandage. In cases of pitting edema, compression padding is also used immediately prior to treatment to soften and prepare the tissues for MLD by increasing the elasticity of the skin. This protocol has been shown to be quite effective, even with severe cases of head and neck lymphedema. Compression is usually used at night, because, contrary to that seen with limb edema, HNL tends to get worse with the recumbent position.
While outpatient treatment is always preferred, mild to moderate cases of head and neck lymphedema can frequently be treated effectively by the patient in a self-administered home program featuring a modification of MLD techniques in
combination with compression pads and garments. Previous studies from M.D. Anderson Cancer Center have shown excellent treatment results, whether patients performed a self-directed home management protocol, or if they received outpatient rehabilitation as well. Compliance with recommended protocol was the most significant factor associated with success, regardless of approach.
Why does HNL respond so well to treatment?
In general, HNL should respond well to treatment, especially in mild to moderate cases where treatment can often be discontinued over time and the edema not
return. One potential reason is related to the gravitational/positional benefit that occurs once the patient becomes upright and mobile each day. This is evident by observing increased levels of swelling in the head and neck with most patients when they first rise and a fairly rapid reduction of swelling during the first two hours of being mobile in the mornings. This pattern is not typically observed with edema elsewhere in the body. In patients with severe neck scarring, however, the pattern may be reversed, with more persistent edema and greater swelling at the end of the day due to the disruption of drainage pathways created by the scar.
Summary
Head and neck lymphedema is a common occurrence following treatment for head and neck cancer that may be quite mild and cosmetic in nature or can progress and become very severe, with functional impairments. It is often very responsive to treatment, especially with early intervention using manual lymphatic drainage and compression
is persistent and difficult to manage. It is important to remember that HNL differs from edema elsewhere and proper training is required for head and neck lymphedema management for maximum success. LP
A full set of references can be found online at www.lymphedemapathways.ca
Julia Chan. Family Caregiver, Scarborough, ON
Laurelea Conrad. Toronto, ON
Elizabeth Anthony. Family Caregiver, Victoria, BC
Jason Anderson. Professional Caregiver, Burnaby, BC
Sara Shearkhani. Family Caregiver, Toronto, ON
Darlene Kidd. Professional Caregiver, Sudbury, ON
Marilyn Cassidy. Professional Caregiver, Ottawa, ON
Lise-Nathalie LePage. Professional/Volunteer, Rimouski, QC
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