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Lymphedema

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A condition manifested by local fluid retention and tissue swelling caused by a compromised lymphatic system.

Characterized by increased protein trapped in the interstitium, leading to water retention and nonpitting edema,due to obstruction or dysfunction of the lymphatic system.

The pathophysiology involved in lymphedema is mechanical insufficiency of the lymphatic vessels because of obstruction of lymphatic pathways or surgical removal of lymph nodes.

Overall incidence 16.3% after treatment for melanoma, 10.1% after genitourinary cancers, 19.6% after gynecologic cancers and higher when the lower rather than the lowere extremity is affected.

Association with breast cancer ranges from 0-3% after lumpectomy, 65-70% after modified radical mastectomy with regional nodal irradiation.

The lymphatics returns interstitial fluid to the thoracic duct and then to the blood stream, where it is recirculated back to the tissues.

The soleus muscle contracts with walking and squeezes lymph vessels to propel lymph towards the thoracic duct.

Valves present in lymphatic vessels prevent lymph from returning to the lower extrremities.

Inguinal nodes filter the lymph from the lower extremities on its way to the thoracic duct.

Abnormal lymph nodes may be associated with lymphedema.

The thoracic duct returns lymph to the venous circulation at the left subclavian vein.

Tissues with lymphedema are at risk for infections.

Associated with impaired survivorship.

Patients may suffer with fatigue ans complain of heaviness of a swollen limb or pressure related to swelling in other body areas.

May be and inherited process, primary type, or caused by injury to the lymphatic vessels, a secondary type.

Most common following lymph node dissection, surgery and/or radiation which can damage the lymphatic vessels during treatment for malignancies, most commonly breast cancer.

Superficial lymphatic capillaries drain interstitial fluid from the skin and subcutaneous tissue into larger deep collecting lymph vessels, which in turn  flow into axillary lymph nodes.

It is the disruption of this process by axillary interventions or lymphangitic infiltration that leads to lymphedema.

Eventually fibrosis results from the leakage of protein rich lymphatic fluid into the interstitial space.

May develop months or years after breast cancer treatment with around 75% of cases occurring in the first year.

80-90% of women who develop breast cancer related lymphedema do so within 3 years of treatment, and the remaining 10-20% of patients will develop lymphedema at about 1% per year.

Breast cancer survivors engaging in slow, progressive weight lifting program, strengthened their affected arms and had a lower incidence and severity of lymphedema.

Excess body weight is associated with a higher likelihood of onset and a worst clinical course of breast cancer related lymphedema.

In a randomized clinical trial of overweight breast cancer survivors with lymphedema, control group, exercise intervention, weight loss intervention, and combined exercise and weight loss intervention status was not associated with significant between group differences in the 12 month percentage of change in inter-name difference (The WISER Survivor Randomized Clinical Trial).

Approximately 50% of women will develop lymphedema by 20 years.

Surgical procedures and radiotherapy contribute to the development of arm lymphedema.

The risk and severity of edema correlate with the extent of axillary surgery.

Risk of arm edema after radiotherapy increased by advanced stage at diagnosis, obesity, prolonged presence of seroma and cellulitis in the operated arm.

Additional risk factors for breast cancer associated lymphedem include postoperative infections, seroma, hematoma, chemotherapy with taxanes and obesity.

Black race, Hispanic ethnicity, neoadjuvant chemotherapy, older age, and longer follow up are independently associated the risk of breast cancer related lymphedema.

Higher  body mass index of greater than 30 kg /meter squared nearly triples the risk of breast cancer related lymphedema.

The risk of breast cancer associated lymphedema is nearly 4 times higher in women who undergo a complete axillary lymph node dissection compared with those who undergo sentinel lymph node biopsy alone (20% versus 5.6%, respectively).

The addition of supraclavicular radiation significantly increases the risk of lymphedema compared with breast and/or chest radiation alone.

The risk of breast associated lymphedema is significantly higher, at 41%, among women who undergo go both complete axillary dissection and axillary radiation.

The risk of lymphedema is directly proportional to the extent of axillary surgery (sentinel lymph node biopsy versus complete axillary lymph node dissection) and the extent of axillsry radiation (axillary versus addition of regional nodal irradiation).

Postoperative morbidity associated with sentinel lymph node biopsy is negligible compared with that of axillary dissection.

Pateints should avoid any injury to the arm such associated bruises, cuts, burns, insect bites, sunburns or sports injuries.

Causes swelling and discomfort, impairing arm function and quality of life.

Of patients who undergo sentinel lymph node biopsy, 5% to 7% develop lymphedema.

One-third of patients with breast cancer require complete axillary dissection,​ which is associated with 13% to 47% incident lymphedema.

Breast cancer survivors at risk for lymphedema alter activity, limit activity, or both.

No evidence that slowly progressive weight lifting precipitates lymphedema among breast cancer survivors.14​ although that study had limited statistical power and follow-up.

Cellulitis can compromise the lymphatic system and cause lymphedema.

Filariasis, a parasitic infection is a common cause of secondary lymphedema seen in tropical areas of the world.

Primary lymphedema related to poorly develpoed or missing lymph nodes or vascular spaces.

Primary lymphoma may be present from birth, develop with puberety or not become apparent until adulthood.

Lower extremity primary lymphedema is the most common presentation, in men.

The most prevalent form of secondary lymphedema in women is in the upper extremities from breast cancer surgery, lymph node dissection and radation therapy.

Breast cancer related lymphedema can occur as late as 30 years.

Head and neck lymphedema may be seen following surgery and radiation for head and neck cancers.

Secondary lymphedema may be seen in the lower extremities and groin following colon, ovarian, uterine, prostate and testicular cancers when removal of lymph nodes or radiation is employed for cancer surgery.

Flying, due to increased cabin pressure, may precipitate onset of secondary edema in patients treated for cancer.

Early diagnosis may be difficult as there is no accepted criteria differentiating one extremity from another based on fluid volume or circumference size.

Bioimpedence measurement may provide a better measurement of fluid volume in an extremity and aid in the diagnosis.

WHO staging: Stage 0- lymphatic vessels had sustained some damage but is not yet apparent and transport capacity is sufficient so that lymphedema is not present. Stage 1-tissue is at the pitting stage and upon waking in the morning thhe limb or affected area is normal are almost normal in size Stage 2- tissues are spongy and non-pitting and fibrosis is present with hardening of the limbs and increasing size stage 3- swelling is a reversible and the limbs or affected tissues are very large, hard with fibrosis and unresponsive.

Grading: Grade 1- mild edema with lymphedema involving the distal parts such as a forearm and hand or lower leg and foot with a circumference of four centimeters or less.

Grade 2-moderate edema with lymphedema involving an entire limb or corresponding quadrant of the trunk. Different in circumference is more than 4 cm but less than 6 cm. Tissue changes such as pitting edema present. Erysipelas may be present.

Grade 3A-severe edema with lymphedema present in one limb and its associated trunk quadrant. The difference in circumference is graded in 6 cm and significant skin changes with cornification, keratosis, cysts, or fistula are present. Repeated episodes of cellulitis may be present.

Grade 3B-massive edema with similar findings as grade 3A, but with two or more extremities affected.

Grade 4-gigantic edema, that is known as elephantiasis with almost complete blockage of lymph channels.

Treatment consists of a combination of compression massage, compression garments, compression bandaging.

Elastic compression garments following the congestive therapy is utilized to maintain edema reduction.

Compression pump with a multi-chambered pneumatics leave with overlapping cells promotes lymph fluid movement.

Pump therapy is combined with manual lymph drainage and compression bandaging.

Complete decongestive therapy consists of manual manipulation of lymphatic ducks, stretch compression bandaging, therapeutic exercise and skin care.

Manual manipulation of lymphatic ducts includes rhythmic massaging of the skin to stimulate flow of lymph to return the fluid to the circulation.

Manual manipulation of lymphatic ducts is effective on non-fibrotic lymphedema cases and less effective with fibrosis.

Surgery cannot cure the disease or eliminate the need for the congestive therapy.

Surgery is utilized only in extreme cases to reduce the weight of the limb, minimize the frequency of inflammation, and Improve cosmesis, and potentially reduce the risk of secondary development of angiosarcoma.

Surgery does not benefit in long-term.

Surgical techniques include circumferential excision of lymphedematous tissues, longitudinal removal of the affected segments of tissue, a division of subcutaneous tissues and tunneling other dermal lympholymphatic anastomoses, lymphovenus shunt, lymphangioplasty enteromesenteric flap omental transfer, and liposuction.

In breast-cancer survivors with stable lymphedema weight lifting exercises, compared with the control group, resulted in better upper and lower body strength and lower incidence of lymphedema exacerbation (Schmitz KH et al).

Breast cancer related lymphedema is slowly progressive swelling either of  the proximal portion of the distal digits of the arm.

Breast cancer related lymphedema has been defined as a 2 cm increase in limb circumference, a 200 mm increase in limb volume, or 5 to 10% change in the limb volume compared with the unaffected side.

Breast cancer related lymphedema can manifest a swelling of the shoulder, breast, back, and thoracic areas.

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