“Lymphedema” Management and Treatment

DEFINITION
Lymphedema refers to excessive accumulation of interstitial protein-rich fluid, typically resulting from impaired regional lymphatic drainage.


PHYSICAL FINDINGS AND CLINICAL PRESENTATION
Edema
● Painless and progressive
1. Initially, the edema is pitting and smooth; however, with advanced cases, the edema becomes nonpitting            (this depends on the extent of fi brosis that has occurred).
2. Elevation of the extremity resolves the swelling in the early stages but not in the advanced stages.
● More often unilateral  but, depending on the cause, can be bilateral
● Not always restricted to the lower extremities but may involve the genitals, face, or upper extremities (e.g., arm
swelling after mastectomy)

● Stemmer’s sign (squaring of the toes caused by edema in the digits)
● Buffalo hump appearance of the dorsum of the foot
● Loss of the ankle contour, giving a tree trunk appearance
to the leg .
Skin
● Hard, thick, leathery skin secondary to fi brosis induced
by chronic stasis
● Occasional drainage of lymph
● Infections (cellulitis, lymphangitis, onychomycosis)
CAUSE
Lymphedema is caused by a reduction in lymphatic transport
and is classifi ed into primary and secondary forms.
Primary idiopathic lymphedema is thought to result from
developmental abnormalities such as lymphatic hypoplasia
and functional insuffi ciency or absence of lymphatic valves.
Subclasses of this type of lymphedema include the following:
● Congenital lymphedema
1. Detected at birth or recognized within the fi rst 2 years of life
2. Involving one or both extremities, usually the entire leg
3. May be familial (Milroy’s disease)
● Lymphedema praecox
1. Onset in teenage years
2. Usually unilateral
3. Most common form of primary lymphedema (up to 94% of cases)

4. More common in females (10:1), suggesting that estrogen plays a role in pathogenesis
5. May be familial (Meige’s disease)
● Lymphedema tarda
1. Usually occurs after the age of 30 years
2. Uncommon, accounting for less than 10% of cases of

primary lymphedema
Secondary lymphedema develops after disruption or obstruction of the lymphatic system as a consequence of the following:
● Surgery for malignant tumors (e.g., breast, prostate, lymphoma)
● Edema of the arm after axillary lymph node dissection is the most common cause of lymphedema in the United States.
● Incidence of lymphedema is approximately 14% in post mastectomy patients with adjuvant radiation.


TREATMENT
● Inflammation (streptococci, filariasis)
● Filariasis is most common cause of lymphedema worldwide
● Trauma
● Radiation with lymph node removal
DIFFERENTIAL DIAGNOSIS
● Lymphedema is primarily a clinical diagnosis made on the basis of physical features that distinguish it from other
causes of chronic edema of the extremities, such as the presence of cutaneous and subcutaneous fi brosis (peau d’orange) and the Stemmer sign.
● When physical examination is inconclusive, other available imaging tests can help make the diagnosis: isotopic
lymphoscintigraphy, indirect and direct lymphography, lymphatic capillaroscopy, MRI, CT, ultrasound
● Isoptopic lymphoscintigraphy is currently considered the gold standard for diagnosis of lymphedema.
● Exclude other causes of edema (e.g., cirrhosis, nephrosis, CHF, myxedema, hypoalbuminemia, chronic venous stasis, reflex sympathetic dystrophy, obstruction from abdominal or pelvic malignancy).
LABORATORY TESTS
● BUN, creatinine (Cr), liver function tests, albumin, urine analysis, thyroid function tests (TFTs) are obtained to exclude possible systemic causes of edema.
● Noninvasive venous studies help exclude venous insufficiency.
● Genetic testing may be practical in defining a specific hereditary syndrome with a discrete gene mutation, such as
lymphedema-distichiasis (FOXC2) and some forms of Milroy disease (vascular endothelial growth factor receptor
3 [VEGFR-3]).
IMAGING STUDIES
● Lymphoscintigraphy:
1. Diagnostic image of choice
2. Sensitivity and specifi city of 100% in diagnosing lymphedema
● CT scan: to exclude malignancy leading to obstruction
● Duplex ultrasound to rule out venous obstruction as a cause for edema
● Lymphangiography:
1. Available but rarely used
2. May be requested by surgeons considering repair or excision of tissue for lymphedema
3. Difficult to perform; most information can be obtained
from the nuclear lymphoscintigram.

TREATMENT
Complex decongestive therapy (CDT) is backed by long-standing experience as the primary treatment of choice for lymphedema in children and adults. It involves a two-stage treatment
program:
1. Reduce leg swelling and size:
● Leg elevation
● Limb massage
● Pneumatic leg compression
2. Maintain edema-free state:
● Elastic support stockings that are properly fi tted according to compression pressure and length are essential to
prevent edema from returning.
● Compression pressures are graduated; most of the pressure is distal, with less and less pressure from the stockings
moving proximally.
● Compression pressures range from 20 to 30 mm Hg, 30 to 40 mm Hg, 40 to 50 mm Hg, and 50 to 60 mm Hg. Most prefer 40 to 50 mm Hg for lymphedema.
● The length should cover the edematous site. Choices include below-knee, thigh-high, and pantyhose lengths.
● No drugs have been shown to be benefi cial. Diuretics, in particular, should not be used because they may promote
the development of volume depletion.
● Treat infections, such as lymphangitis (usually caused by group A streptococcus), with antibiotics. Clotrimazole 1%
cream should be applied daily to dried fi ssured areas between the toes to prevent fungal infections.
● In secondary lymphedema, treating the underlying cause is indicated (e.g., prostate cancer, breast cancer). If the cause is filariasis caused by the parasites Wuchereria bancrofti or Brugia malayi, treatment is diethylcarbamazine citrate.
● Surgery for chronic lymphedema should act as an adjunct to CDT or as an alternative if CDT has proved unsuccessful.
● Operative treatment is considered for the following:
● Continued increase in leg size, despite medical treatment
● Impaired leg function
● Recurrent infections
● Emotional lability secondary to the cosmetic appearance