A 69-year-old lady presented to the clinic with a history of right upper extremity (RUE) swelling of 3-month duration. She reports no pain but there is some discomfort because of the weight of the limb. And she reports being embarrassed because it is drawing attention from onlookers. The swelling started first on the upper arm and now includes the hand. Sometimes she feels slight warmth.
Past medical history: She has a history of breast cancer for which she had a partial mastectomy followed by radiation therapy 2 years ago. No history of diabetes or hypertension (HTN), Hx of hypothyroidism. No history of travel to foreign country.
Social history: She works as a home health aide and lives with family and two grown-up kids in a private house with no stairs.
Past surgical history: Tonsillectomy as a child. Aforementioned surgery 2 years ago.
Medications: Vitamins, D, B12 antioxidants, and synthroid 75 mcg daily.
Vital signs: BP 130/70 mmHg, RR: 14/min, PR: 65 per min, Temp 97º F, Ht: 5’5”, Wt: 140 lbs
Examination: WB, WN female in no AD and ambulates normally
Head, eyes, ear, nose and throat (HEENT)-extraocular movements (EOM) PERRLA, no ptosis, no pallor
General: Alert, oriented – 3, slightly anxious
Extremities: Entire length of RUE is swollen, no erythema slightly warm to touch, no tenderness. Edema was pitting in nature.
Measurements: 5 cm above antecubital fossa 29 cm
5 cm below antecubital fossa 18.5 cm
Motor examination: Power was 4/5+ in biceps, deltoid, triceps, brachioradialis forearm extensors and flexors and finger extensors and flexors.
Reflexes: 1+ and symmetrical in biceps, triceps, and brachioradialis
Sensory examination: Intact to LT in all dermatomes of the upper extremity (UE).
Laboratories: White blood cell (WBC) 7000 cells/mL, hemoglobin: 12.0 g/dL, complete blood count (CBC), SMA 18, and erythrocyte sedimentation rate (ESR)—normal.
X-ray of the left UE (LUE): No evidence of lytic or blastic lesions in humerus, radius ulnar, and wrist bones and digits.
Surgical report: Two years ago, patient was diagnosed with stage 2 intraductal breast carcinoma on R. A modified radical mastectomy and axillary lymph node dissection was done. She received radiation therapy and tamoxifen. No breast reconstruction was done.
This case is an example of lymphedema (LE) acquired secondary to breast cancer treatment. The approach to a patient with LE is unique. The initial focus should be to differentiate congenital from acute. The second focus should be to determine whether it is transient or persistent. The third focus should be to determine if any evidence of metastasis exists. The fourth is to determine the stage. Treatment of LE is held in two phases: (1) reduction of fluid and (2) maintenance.
Review of Proposed Pathology and Pathobiomechanics
LE is accumulation of protein-rich fluid (lymph) in tissues. It usually results from impaired lymphatic system. The lymph vessel function is impaired, thus by interrupting the drainage. Normally lymph vessels remove excess fluid from tissues and transport it back to circulation. Lymph capillaries are situated in the dermis, woven like a cobweb which then drains to the lymphatic vessels in subcutaneous tissues ultimately via the thoracic duct directly to the circulatory system ( Fig. 10.1 ). Any disruption of this process can lead to LE. In addition, immune cells mature in the lymph system. Therefore the lymphatic system is one of the most important defense mechanisms.
LE can be classified as primary or secondary:
Primary LE is caused by developmental abnormalities of the lymphatic system. Symptoms may develop at birth or later in life.
Secondary LE is caused by an acquired condition causing damage to the lymph system. The most common causes are infection, injury, and removal of lymph nodes in the underarm, groin, pelvis, or neck for treatment of cancer and radiation. In developed countries, surgical removal of lymph nodes for cancer treatment is the most common cause. Given that breast cancer is the most common cancer among women, breast cancer related LE (BCRL) is the most common type of LE. Other cancers that can cause LE in lower extremities are uterine cancer, prostate cancer, lymphoma, melanoma, vulvar cancer, or ovarian cancer.
The risk of LE increases with the number of lymph nodes affected. There is less risk with the removal of only the sentinel lymph node (the first lymph node in a group of lymph nodes to receive lymphatic drainage from the primary tumor).
Lymphatic filariasis is caused by parasitic infection/infestation by microfilaria of Wuchereria bancrofti and affects over 120 million people in 72 countries, including parts of the Caribbean and South America. It is considered globally as a neglected tropical disease. Adult filarial worms commonly cause subclinical lymphatic dilatation and dysfunction. This condition is also known as elephantiasis. The chronic manifestations of LE and/or hydrocele will develop in approximately 30% of infected persons. LE mostly affects the legs, but can also occur in the arms, breasts, and genitalia. Most people develop these symptoms years after infection has cleared. Recurrent secondary bacterial infections of the affected extremity, characterized by severe pain, fever and chills, hasten the progression of LE to its advanced stage, known as elephantiasis , ( Fig. 10.2 ).
Incidence of Lymphedema
BCRL is reported in 7% to 77% of patients who have undergone axillary lymph node dissection because of transection of lymph vessels. Those with sentinel node biopsy had a much lower (3%–7%) incidence. , Other risk factors identified as causing BCRL are occupation, infection, increased BMI, age above 65 years, and radiation. Breast reconstruction was not considered a risk factor. ,
The entire limb is swollen: upper limb on the same side of operation for breast cancer and lower limb in the other cancers mentioned earlier. Usually there is no pain unless infected. Patients may also be depressed because of the appearance and unwanted attention drawn to them. They also complain of garments being tight.
Neurologic symptoms such as tingling, numbness, and muscle weakness because of entrapment of peripheral nerves in the UE or tumor involvement of the brachial plexus may be experienced. Radiation therapy usually affects the upper trunk and tumor affects the lower trunk of the brachial plexus.
Grading of Lymphedema (Table 10.1)
The following system is used to diagnose and describe LE based on size of the affected limb. Grades 1, 2, 3, and 4 are based on size of the affected limb and how severe the signs and symptoms are:
Stage I: The limb (arm or leg) is swollen and feels heavy. The edema is of pitting type.
Stage II: The limb is swollen and feels spongy. This is nonpitting and feels hard.
There may be peau d’orange appearance ( Fig. 10.3 ).
Stage III: This is the most advanced stage. The swollen limb may be very large. Stage III LE rarely occurs in breast cancer patients. Stage III is also called lymphostatic elephantiasis (see Fig. 10.2 ).
Another method is from the International Society of Lymphology.
Staging of Lymphedema Adapted From the International Society of Lymphology
|0||Latent||Some damage to lymphatics; no visible edema yet|
|1||Spontaneously reversible, acute phase||Pitting edema; reversible with elevation of the arm. Usually, upon waking in the morning, the limb(s) or affected area is normal or almost normal size|
|2||Spontaneously irreversible, chronic phase||Spongy consistency and is “nonpitting.” Fibrosis found in Stage 2 lymphedema marks the beginning of the hardening of the limbs and increasing size|
|3||Elephantiasis; irreversible, end stage||Irreversible and usually the limb(s) is/are very large. The tissue is hard (fibrotic) and unresponsive; consider debulking surgery at this stage|