The key to early diagnosis and intervention for secondary lymphedema, non-cancer related, is increasing awareness and education about lymphatic system pathophysiology and anatomy so that every physician, regardless of specialty, recognizes the possibility of lymph system dysfunction as the cause of chronic limb edema.

This presentation will review actual clinical cases that illustrate some of the "uncommon" causes of secondary lymphedema, non-cancer related. The following categories will be represented:

Orthopedic injuries/surgeries - i.e. fractures, meniscal/ligamentous injuries/repairs, joint replacements, and tendon releases.

Neurological conditions - muscle paresis - CVA, post polio, spastic paralysis.

Vascular injuries/surgeries - vessel injury from trauma or unintended trauma from catheterization, removal of a vein/veins for CABG or leg bypass.

Integumentary injury - trauma to skin, degloving injuries, burns, damage to skin from long term steroid use, i.e. for COPD or RA.

Infection

Scar tissue formation - multiple abdominal surgeries, i.e. Gall bladder, TAH, umbilical/abdominal hernias.

Lipedema

Most often it is the combination of factors that causes the lymphedema to develop. A precise chronological history of symptoms, surgeries, traumas, medications used, therapies received, and co-morbidities can clarify the "unexplainable" and diagnose the problem, leading to a plan to manage the lymphedema, thus improving the patient's quality of life.

The lymphatic system is a regional system. Individual lymph drainage territories of the skin are independent of one another. Collateral connections between these areas exist but are small and are not needed when lymph transport capacity exceeds lymph load in the unimpaired lymphatic system. When there is disruption to lymph vessels/nodes from trauma, surgery, infection, scar tissue, etc. or when the elasticity of the skin and subcutaneous tissues is poor or when muscle tone is poor, then lymph load may exceed lymph transport capacity and lymphedema results. Usually, edema begins distal to the site of the damage/obstruction.

A basic understanding of the normal anatomy of the lymphatic system is critical for anyone evaluating an individual with edema of unknown origin, where testing has already ruled out most of the common causes of edema, namely cardiac, renal, hepatic, thyroid, venous, or drug induced. The major lymph node basins for the extremities are the axillary (armpit) for the upper extremities and the inguinal (groin) for the lower extremities. Lymph from both lower extremities and the genitalia drains into the pelvic and abdominal lymph node basins. From here, the lymph eventually moving into the thoracic duct which is the major lymph vessel that transports lymph fluid form the lower half of the body into the left venous angle between the left jugular and left subclavian veins, and into the right side of the heart. Lymph from the right upper extremity drains into the right axilla and then into the right lymphatic duct which empties into the right venous angle between the right jugular and right subclavian veins, into the right side of the heart. Anatomically, one can see that disruption from infection, scarring, inflammatory processes, accidental injury during a surgical procedure in the pelvic/abdominal areas can result in lower extremity lymphedema as well as abdominal and genital edemas. In the same way, any injury along the path from the upper extremity to the axilla and then into the chest can result in lymphedema in the arm/hand. Trauma, infection, or the formation of a blood clot in the area around the venous angles can cause lymphedema.

An individual who undergoes multiple abdominal surgeries with scarring near/around the abdominal lymph nodes and the afferent vessels carrying lymph from the lower extremities, may develop lymphedema in the legs. An individual who sustains a severe fracture/dislocation of the ankle disrupting pathways from the foot/ankle may develop lymphedema of the foot and ankle which may progress to the calf due to an overloading of the functioning vessels in the lower leg. A woman having cosmetic surgery to remove excess fat pads in the anterior axillary line may incur damage to the axillary nodes if the surgery extends too far into the axilla. Does this sound far-fetched? I consulted with a nurse who had this surgery (after a pregnancy left her with large bulging fat pads at the anterior axillary line) and developed lymphedema in her left hand post operatively. When she obtained the pathology report, the specimen from the right side contained fatty tissue only, but the specimen from the left side contained fatty tissue and three axillary lymph nodes.

In most cases of secondary lymphedema non-cancer related, the patient endures multiple physician visits and diagnostic tests that rule out the heart, thyroid, kidney, liver, and cardiovascular system as causes of the edema. CAT scans, MRI's and bone scans rule out occult cancer as the cause of the swelling. While the patient is heartened that he/she does not have cancer or a life-threatening condition, they are frustrated by the lack of diagnosis of their problem. They know what they don't have, but they still don't know why their limbs are swollen. An obvious diagnosis may be venous dysfunction. Oddly enough, chronic venous insufficiency may be part of the problem or the root cause of the secondary lymphedema, which is still often not diagnosed and properly treated.

Pure venous edema reduces on elevation. Lymphedema may reduce with elevation in early Stage 1, but when fibrosis begins to develop the limb no longer completely reduces with elevation and skin and subcutaneous tissue changes occur, leading to decrease oxygenation, an increased risk for infection and a decrease in limb mobility.

The key to early diagnosis and intervention for secondary lymphedema non-cancer related is an Increased awareness/education regarding lymphatic anatomy/physiology and pathophysiology for every physician and health-care professional, regardless of specialty. The cases I will review have seen internists, dermatologists, podiatrists, cardiologists, neurologists, orthopedists, and physiatrists, physical and occupational therapists. Many missed the diagnosis all together. Unfortunately, progressive skin changes and bacterial and fungal infections have been misdiagnosed as dermatitis, eczema, gout, etc. resulting in improper drug prescription or other treatments that not only didn't improve the situation but allowed the lymphedema to progress unchecked, to the detriment of the patient.

What is the solution to this problem? First, a good basic overview of the lymphatic system (presented by a lymphologist who can explain common clinical presentations to the students) needs to be included in medical/allied health curricula. The NLN recently offered a 4-hour instructional course during their conference held in August 2002 in Chicago: "Lymphedema management for the practicing physician". This session was attended by --- physicians and ---. It provided a wonderful overview of the lymphatic system anatomy, physiology, and pathophysiology, and was presented by some of the leading lymphologists in this country.

The NLN is trying to set up a Speakers Bureau of experts who would agree to be available to present at conferences, Grand Rounds, Awareness Events, etc. After discussing some basic lymphatic anatomy and pathophysiology with the physician/husband of a patient with breast cancer related lymphedema, he excitedly admitted that he and his colleagues have seen many patients with unexplained extremity edema. He was amazed to realize the implication of the cumulative effects of surgeries, obesity, trauma, chronic inflammation requiring long-term steroid treatment, vein harvest for bypass surgeries, (the list goes on and on) on the lymphatic system. Of course he is sensitized, having watched his wife deal with progressive upper extremity lymphedema, which progressed despite months of ineffective "treatment". Once she received appropriate treatment and her lymphedema began to reduce, he realized the importance of specialized training/knowledge to treat lymphatic system disorders. He certainly will never stop his clinical investigation short of the lymphatics when diagnosing edema of unknown origin.

A Review of 24 randomly selected cases revealed the following causes of lymphedema (several cases had more than one cause):
CVA or neuromuscular spasticity - 2 Knee injury/sprain - 1,
Lipedema - 2, Total hip replacement - 1
Fracture - 5 Total knee replacement - 4
Leg bypass - 1 Coronary bypass - 2
Infection - 9 Abdominal surgery - 5
Chronic steroid medication - 3 Skin ulceration - 3
Arthroscopic surgery of the knee - 2 Vein ligation - 1.

The following are representative examples of secondary lymphedema non-cancer related.

Case 1 - PP - 49-year-old male, sustained a compound proximal and distal tib/fib fracture, trimalleoar fracture/ dislocation of the ankle. He had ORIF and 4 months of rehab. He did not progress in rehab due to severe pain, swelling, heat and redness in the affected lower leg. He had several episodes of redness, heat and fluid leakage from the sites where the external fixation device had been removed. He had seen 3 orthopedists, a physiatrist who prescribed an ankle foot orthosis to support the distal ankle, and a dermatologist who diagnosed dermatitis instead of cellulitis and prescribed a topical steroid cream. He could not progress in his rehab due to the chronic redness, pain, swelling, and limited mobility in his ankle and foot. He could only walk short distances, had great difficulty negotiating stairs, and performing any ADL's involving weight bearing. He was on disability from his job as a police officer. He was self- referred to a lymphologist after a friend and informed breast cancer survivor recognized that he might have lymphedema. His lymphedema was secondary to the trauma of the fracture/dislocation, surgery, and chronic, untreated cellulitis in his leg. The swelling was labeled as "post-operative" and the cellulitis was misdiagnosed as a skin irritation. He lost 4 months of his life and his return to work and his life in general was delayed that much longer.

Case 2 - MA - 75 year old female who sustained a fracture of the left second and third metatarsals when a brick retaining wall in her garden fell on her as she was weeding. The fractures were casted in a short leg cast and in 4 weeks she developed an infection, severe pain in her leg and foot, and an ulceration on her heel because her leg swelled in the cast.
Dopplers and x-rays were negative for any new pathology. Fortunately, her orthopedist had a patient who had received CDT treatment and had done well. She was referred to that lymphologist after 8 weeks of physical therapy failed to heal the ulcer or reduce the pain, swelling and limited mobility in her left foot/ankle/lower leg. Her lymphedema was secondary to the trauma of the incident and the infection. After one month of CDT treatment, her ulcer was completely healed, range of motion of the foot and ankle were improved, she could walk without her cane, fit into her shoes, and pain was 2/10 reduced from 10/10. Most importantly, she was able to get back to her gardening.

Case 3 - PM - 56 year old female with a history of severe rheumatoid arthritis for 20 years, with a nine-month history of progressive swelling in both legs following 18 months of oral steroid medication for her arthritis. Because she had been on many toxic medications to treat the arthritis, she was referred to a hematologist for a bone marrow and bone biopsy which were negative. She was seen by a vascular surgeon who did Dopplers that were negative for DVT. Her internist ordered a CAT scan of the abdomen and pelvis that was negative. She developed progressive weakness and decreased mobility in her lower extremities. The skin of both lower extremities was thin, taut and shiny, and the patient reported that they were chronically red and warm. She had been hospitalized for an infection in her legs in June of 01. There were several small ulcerations on the right lower leg which were had been leaking lymph for approximately two months prior to her referral to the lymphologist. There was a 4-cm diameter 1-cm deep ulceration on the plantar surface of the MP joint of the left great toe. Her podiatrist had "shaved" a callus from this area, and she reported that she had been treated for a "staph" infection by the podiatrist, but that he said that it was resolved.
At the time of her referral, she was dependent in all ADL's, could not negotiate stairs (she turned her living room into a hospital room), spent most of the time in a wheelchair, and could only ambulate 10 feet with a rolling walker. She developed flexion contractures in her hips and knees from the prolonged sitting and immobility. Her husband had to dress her lower body. Her pain was 10/10 and she was severely depressed. Wound cultures revealed MSRA in the left great toe ulceration, and in the small ulcerations on her right lower leg. No one could figure out what was wrong with this woman. She had lymphedema in her legs secondary to prolonged steroid treatment that worsened after the chronic antibiotic-resistant infection remained untreated for several months. The elasticity of her skin was severely weakened and the skin itself was paper thin and opened easily. After nine months, her rheumatologist referred her to a lymphologist after researching on the Internet.
Once the lymphedema was addressed (the patient underwent a course of CDT) and the infection appropriately treated with a drug that MRSA was sensitive to, the wounds healed, the edema and pain resolved, and the patient began to increase strength and mobility. After 4 weeks of CDT, the patient was able to ambulate 50-100 feet with her rolling walker. At the one-month follow-up appointment, the patient ambulated with a straight cane, was independent in most of her ADL's, and was able to drive herself to the appointment. She was referred to PT for strengthening and mobility exercises. Her rheumatologist was astounded and vowed to discuss this case with all her colleagues and spread the word about remembering to include the lymphatic system when evaluating edema of unknown origin.

Case 4 - RL - a 60 year old male, retired airforce test pilot, with a six-year history of progressive lower extremity edema, onset 6 months following coronary artery bypass using donor veins from both lower extremities. As the edema worsened, it extended into the abdomen. Unfortunately for this patient, he was obese, had a large abdomen, and did have a history of heart disease. He was told that the edema in his legs was just water retention because he was fat and had some heart disease. The patient had been sleeping in a recliner chair for 6 years. He had been diagnosed with sleep apnea. The patient referred himself after searching on the Internet for months. His physician refused to give him a referral because he didn't believe he had lymphedema because he never had lymph nodes removed! He traveled from New Hampshire to New York to consult with a lymphologist and receive CDT treatment. His only treatment prior to that had been diuretics. He was dependent in lower body dressing, only ambulated 10 feet before stopping to rest and lean on furniture due to pain and SOB. His pain was 10/10. He was severely depressed. He scored his functional impairment a 10/10. The lymphologist referred him to his cardiologist to rule out abdominal ascites or CHF as the cause of his SOB. His cardiologist did an abdominal US that found no fluid in the abdominal cavity. However, he did have abdominal lymphedema!

This patient received one month of CDT treatment achieving a 53.3-cm reduction in his left lower extremity and a 44.5-cm reduction in his right lower extremity. His weight reduced from 298 to 273 pounds. At one-month follow-up, he reduced another 21.8 cm on the left leg and 13.3 cm on the right. He slept supine in a bed with his wife for the first time in 6 years and he was able to play with his grandchildren. He scored his post-treatment functional impairment a 3/10 because he needed assist to don his compression stockings. He did acknowledge that although he hated the stockings, they were a small price to pay for getting his life back. His lymphedema was secondary to the disruption of the venous system and scarring/probably damage to lymphatics from the vein harvest, the subsequent infections, and his obesity. Once again, an individual who was receiving regular medical care from an internist and cardiologist suffered needlessly because the lymphatic system was not considered when lower extremity edema developed.

Case 5 - MR a 78-year-old recent widower with a one-month history of severe swelling in his left forearm/hand since IV infusions for dehydration caused by depression. He had seen vascular specialists who ruled out DVT as the cause of the edema. His ADL's were impaired 5/10 and he reported chronic pain and a feeling of bursting in his left hand 5/10. His grip strength was poor in the left hand and he was having difficulty dressing and doing his daily chores. His significant past medical history included intestinal resections in 1960 and 1963 for severe Crohn's disease for which he required chronic steroid treatment to be able to function. The skin of all extremities was thin and there were multiple scars/bruises from old skin tears/traumas.
MR had lymphedema of his left upper extremity secondary to damage to the skin lymphatics from chronic steroid treatment. He responded well to lymphedema treatment, regaining his strength and mobility in his left hand/arm. He was able to discontinue his compression sleeve/glove after 6 months. Unfortunately, the lymphedema recurred in 9 months when he again required hospitalization for dehydration and IV therapy. Except that this time, MR did his own self MLD and compression bandaging and then wore his compression garments for about 3 months and was able to discontinue their use.

Case 6 - LK is an independent 85 year old widow with a long history of osteoarthritis and a 3-year history of chronic swelling in her left lower extremity. The swelling limited her safe ambulation and caused impairment in her strength and range of motion of her left leg/foot. She required the assistance of a home health aide for her ADL's. She could only walk short distances with a quad cane. She had had a left total knee replacement in 1995, followed by the removal of a Baker's cyst from the left popliteal area in 1997. Two months after that operation, she developed a fungal infection around the left knee prosthesis, necessitating its removal in 12/97. She underwent a second total knee replacement on the left in 2/98. She was told that she had "post-op edema" which had unfortunately lasted for 3 years! She had consulted several vascular surgeons and another orthopedist, none of whom could diagnose the cause of her chronic edema, other than to say that she should expect it at her age, after the repeat surgeries she had had. Once the lymphedema (which was secondary to the traumas of the repeat surgeries and the infection) was treated, LK was able to "fire" her home health aide and she continues to live independently in her own home, needing only a straight cane for balance when walking.

Case 7 - AS is a 73 year old female with an 18-year history of primary lateral sclerosis/familial spastic paresis. She was dependent in all areas of ADL and required a reclining wheelchair for optimal positioning. In 4/00 she had a Baclofen pump inserted to control her severe spasticity. She developed cellulitis in both legs shortly after that procedure. Relevant past medical history included a total abdominal hysterectomy in 1990 and the repair of an umbilical hernia after that developed after that procedure. As was advised that she was not a candidate for "traditional CDT" but she was relieved to have a diagnosis for her problem. Her lymphedema was most likely secondary to the severe spasticity in her lower extremities complicated by three abdominal surgeries and cellulitis in her legs.

Case 8 - MS is a 66 year old female with a 26 year history of chronic edema in both lower extremities. MS was always "overweight" since her teen years. The swelling in her legs and feet progressed over the years and no longer reduced on elevation. In the last few years, she has been hospitalized 5 times for IV treatment of cellulitis and ulcerations in her legs. MS was treated with diuretics and told to lose weight. She tried both but the swelling and skin changes kept worsening. MS has lymphedema in her legs secondary to longstanding lipedema.

To the trained lymphologist, these cases are self-explanatory. For each case there is a cause and effect. The challenge for every health-care professional is to become educated in the anatomy, physiology and pathophysiology of the lymphatic system so that no individual has to live with lymphedema undiagnosed and untreated.

Lymphedema Therapy
77 Froehlich Farm Blvd., Woodbury, New York 11797
1-800-MD-LYMPH or (516) 364-2200

Marvin Boris, MD
Stanley Weindorf, MD
Bonnie B. Lasinski, MA, PT, CI, CLT-LANA

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