Perspectives in Circulation Today
Swollen Legs - Multiple Etiologies
Sometimes Lymphedema of One Kind or Another
Pneumatic Boot Therapies
Primary Lymphedema: Lymphedema resulting from a congenital impairment of lymph flow through the lymphatics is termed primary lymphedema. It may result from aplasia, hypoplasia or hyperplasia of the lymph channels or from fibrosis or agenesis of the lymph nodes. The name given to a specific condition commonly is related to the age the condition is recognized and diagnosed (Table 1).
Table 1 |
| Commonly divided into three common forms depending on age of presentation. All likely due to a developmental abnormality present at birth and eventually symptomatic when challenged. Other rare forms also included below. | |
| Congenital lymphedema | 10-25% of primary lymphedema cases with 2:1 predominance of females over males and a 3:1 predominance of leg involvement over arms. Commonly no subcutaneous lymphatic trunks but a normal dermal plexus |
| A subset of congenital lymphedema. Familial sex-linked accounting for about 2% of primary lymphedema cases | |
| Lymphedema praecox | Presents after birth and before age 35, especially at puberty. 65-80% of all primary lymphedema cases with a 4:1 pedominance of females over males. 70% unilateral involving the left leg especially. |
| Lymphedema tarda or Meige disease | Presents after age 35 and comprises about 10% of primary lymphedema cases. May have hyperplasticc tortuous lymphatics with no or incompetent valves. |
| Lymphedema-distichiasis syndrome | Autosomal dominant - lymphedema of limbs and double eyelashes, potentially associated with cardiac defects, varicose veins, cleft palate, spinal epidural cysts, renal disease and diabetes |
| Noonan syndrome | Lymphedema associated with congenital heart malformation, short stature, mental retardation, indentation of chest, impaired clotting and typical facial features. Likely autosomal dominant inheritance and variable expression. |
| Turner's syndrome or gonadal dysgenesis | A missing or defective X chromosome occurring in about 1 out of every 2500 girls and resulting in characteristic physical abnormalities: short stature, swelling, broad chest, low hairline, low-set ears, and webbed necks. The non-working ovaries result in amenorrhea and sterility. Other health concerns are also frequently present. |
| Cumming syndrome | Rare syndrome likely a autosomal recessive inheritance resulting at birth in generalized hydrops, campomelia, cervical lymphocele, and polycystic dysplasia of the kidney, liver, and pancreas. |
| Hennekam syndrome | A developmental disorder of the lymphatics presenting as congenital lymphedema and characteristically associated with intestinal or pleural lymphangiectasia, hypoproteinemia, hypogammaglobulinemia, and lymphocytopenia. Other features include flat face, flat nasal bridge, hypertelorism, epicanthal folds, small mouth, tooth anomalies, and ear defects. |
| Other rare congenital syndromes in which lymphatic obstruction may be found: | Klippel-Trenaunay syndrome,Cardiofaciocutaneous Syndrome and Cholestasis-oedema syndrome, Norwegian type. |
Secondary Lymphedema: Worldwide the major cause of lymphedema is Filariasis, a mosquito-borne infection endemic in areas of Asia, India and Africa where up to 200 million people may be infected. The larvae from the latter mature into adult worms in the peripheral lymph channels resulting in severe lymphedema in the extremities and genitalia (Elephantiasis). In the United States, there are perhaps two million cases of secondary lymphedema most commonly having followed surgery for cancers associated with the removal or radiation of the regional lymph nodes. Lymphedema of the arm following removal of lymph nodes after breast surgery is most common (approximately 20-25% postop breast patients); the risk is increased about 25% with the addition of radiation. Tamoxifen therapy is commonly prescribed for the breast cancer patient and occasionally is complicated by lower extemity lymphedema secondary to blood clots and deep venous thrombi. Secondary lymphedema may also follow the absorption of large quantities of blood and iron by the lymphatics following trauma, crush injuries and knee or hip surgery/fracture. Occasionally, it is seen following vein grafts from cardiac surgery, chronic infections, Lymphogranuloma venereum and longstanding venous stasis disease.
Table 2 |
| Bladder cancer: 10-23% | Gynecological cancers: ~50% | |
| Melanoma: 40-75% | Penile cancer: 14-57% | |
| Prostate: 1-66% | Sarcoma: 12-20% | |
| Lymphangitis carcinomatosa: 90-100% | Rheumatoid disease: rare |
Time Course and staging: Nature abhors a vacuum and the body may be said to abhor an empty fluid volume. Lymphatic fluid contains considerable protein and is a good culture medium. Given time, fibrocytes invade excess tissue fluid and what was reversible swelling becomes hard, firm and permanent significantly affecting the function and comfort of the extremity. Breaks in the skin allow invasion of bacteria and cellulitis becomes a hazard that even when cured may scar and worsen the condition of the extremity. Proper diagnosis and treatment are clearly desirable early in the disease process and not after a period of observation that may be requested by insurance entities.
Table 3 |
| Class/Stage | Properties |
| Stage 1 (spontaneously reversible) | Pitting edema which recedes over night |
| Stage 2 (spontaneously irreversible) | Spongy non-pitting swelling as fibrocytes invade the edema. Positive Stemmer sign (difficulty lifting skin from dorsum of fingers or toes compared to uninvolved side). |
| Stage 3 (lymphostatic elephantiasis) | Firm irreversible enlargement of limbs |
Table 4 |
| Heart Failure associated with: | ||
| Arteriosclerotic heart disease | Rheumatic valvular disease | Alcoholic cardiomyopathy |
| Cor pulmonale | Infective carditis | Constrictive pericarditis |
| Venous Obstruction due to: | ||
| Thrombophlebitis | | Large fibroids | |
| Pregnancy | Retroperitoneal fibrosis | Baker's cyst |
| Prolonged dependency | Paralytic conditions | > |
| Hypoproteinemia associated with: | ||
| GI malabsorption and inflammatory diseases | Cirrhosis | Nephrotic syndrome |
| Autoimmune and allergic conditions: | ||
| Eosinophilic fasciitis | Erythema nodosum | Topical allergies |
| Metabolic | Myxedema | |
| Other vascular | Compartment syndrome | Arteriovenous fistulas |
Diagnosis and TreatmentAn astute diagnostician with an awareness of the various possibilities in the charts above most frequently can make an accurate diagnosis considering the age of the patient, medical history, and concurrent physical findings. A variety of imaging techniques are available especially in specialty centers. In general, the proximal location of blockage in lymph drainage is at or just proximal to the transition point from normal/leg or groin to swollen leg.
Treatments for Lymphedema:
Planning the treatment program depends on the cause of the lymphedema. Obvious infection must first be treated with appropriate antibiotics and the oncologist must orchestrate appropriate therapy of any tumor. Next, the patient should be instructed in "Complex Decongestive Therapy (CDT): (a) manual lymphatic drainage; (b) bandaging; (c) care of skin dryness, fungi, drainage and weeping (d) compression garments (sleeves, stockings, devices such as Reid Sleeve and CircAid Leggings; (e) exercises both for physical fitness and to target the muscle pumps below and at the sites of lymph blockage; and (f) means to accomplish elevation of the swollen parts as much as possible during the day and night.
Pneumatic compression devices: The Lympha Press 201M has been the gold standard in gradient-sequential, non-programmable compression systems. It delivers instantaneous, wave like, directional sequential compression, while its short 30 second treatment cycle allows for maximum refilling and emptying of the lymphatic capillaries and minimizes treatment time. Like other non-cardiac triggered pumps, it is contraindicated in the presence of an inflammatory phlebitis process, pulmonary embolism, congestive heart failure, pulmonary edema, suspected deep vein thrombosis (DVT) and infection. Leg compression may commonly produce swelling of the genitalia. This complication can be reduced by appropriate preparatory therapy or manual lymph drainage to stimulate the lymphatics to receive the fluid soon to be displaced by the pumping. A compression garment should be put on when the pumping is completed. (
SurgeryMany procedures have been attempted with limited success. Homan's may be tried to reduce the size of an extremely enlarged lower leg. Again, liposuction might be considered.
Circulator Boot Therapy in difficult Cases:Generalized anasarca: Case 149, an example where the end-diastolic boot is indicated to assist the heart and promote diuresis when the usual lymphedema boot might prove lethal in returning fluid from the legs and in increasing afterload (compression of the legs throughout the heart cyscle impedes runoff into the legs).
 She began to diurese immediately with her boot therapy; her sign says, "weight down 5 lbs. "Her facial puffiness is also beginning to resolve. |
 Her sense of well-being was improving as she posed during her boot treatment. Her sign reads:"1-2-98 9 treatments since last photo on 12-17-97". Her weight had decreased from 134.3 to 115 pounds. |
Chronic secondary lymphedema: Case 139 with Lymphedema tarda or a lymphedema secondary and years remote to prostatic surgery. Here lymph blockage in the pelvis was thought likely as the explanation to his bilateral leg swelling. To avoid returning too much fluid, only the most swollen leg was treated with the Circulator Long Boot. A single bag extending from the toes to the high groin was chosen to send a firm fluid wave (water hammer effect) into the pelvis with each heartbeat. The treatment had a curative effect on both legs.
 The circumferences of his right leg were larger than those of his left: 13 1/8 vs 12 1/2 inches at the ankle, 18 vs 17 1/4 inches at the calf and 22 1/2 vs 22 inches at the thigh. |
 The circumference of both the right and left legs diminished after eight treatments given to the right leg only: 11 3/8 and 11 1/2 inches at the ankle; 16 1/4 and 16 1/8 inches at the calf, and 22 1/8 and 21 1/8 inches at the thigh respectively. |
 He returned for a few treatments in 1987 reporting that his legs were then just beginning to swell again. |
Longstanding lymphedema with no response to other boots:
 Case 200j: Yes, we can treat lymphedema. Our pulsations with each heartbeat deliver much more energy than other lymphedema boots and leave the left soft and pliable. |
 Longstanding lymphedema associated with fibrosis as in this lady has only a partial result with short courses of therapy, however. |