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Lower extremity lymphedema

Lower extremity lymphedema
Authors:
Russell L Ashinoff, MD, FACS
Eric I Chang, MD, FACS
Section Editors:
John F Eidt, MD
Joseph L Mills, Sr, MD
Charles E Butler, MD, FACS
Deputy Editor:
Kathryn A Collins, MD, PhD, FACS
Literature review current through: Jun 2022. | This topic last updated: Oct 14, 2021.

INTRODUCTION — Lymphedema is defined as the abnormal accumulation of interstitial fluid and fibroadipose tissues resulting from injury, infection, or congenital abnormalities of the lymphatic system. Lymphedema most commonly affects the extremities but can also occur in other areas of the body (eg, abdomen, genital region, face, neck).

Issues relating to lymphedema affecting the lower extremities, including etiologies, clinical evaluation, and treatment outcomes, are reviewed. An overview of lymphedema, breast cancer associated lymphedema, general conservative measures, and surgical treatment of lymphedema is provided separately. (See "Clinical features and diagnosis of peripheral lymphedema" and "Clinical staging and conservative management of peripheral lymphedema" and "Breast cancer-associated lymphedema" and "Surgical treatment of primary and secondary lymphedema".)

CLASSIFICATION AND ANATOMY — Lymphedema can be categorized as either primary or secondary. The direct cause of primary lymphedema is unknown and may develop at any point in life. Secondary lymphedema develops as a consequence of the natural history and/or treatment of another unrelated condition. (See "Clinical features and diagnosis of peripheral lymphedema", section on 'Lymphedema'.)

Lower extremity lymphatics — The superficial lymphatic system drains the skin and subcutaneous tissue, paralleling the pattern of venous drainage. The superficial lymphatic vessels then drain into the deep lymphatic system, and then into the lymph nodes of the pelvis.

The lymph nodes of the lower extremities consist of the popliteal and inguinal nodes (figure 1).

Popliteal nodes – The popliteal nodes are small, deep lymph nodes located posterior to the knee, close to the popliteal vessels. They drain lymph from superficial vessels and deep areas of the leg and foot. The popliteal nodes drain into the deep and superficial inguinal nodes [1].

Inguinal nodes – The lymphatics of the inguinal region are composed of a network of lymph nodes and vessels, connecting the lower extremities to the pelvic region and abdomen (figure 2). The inguinal lymph nodes are located in the femoral triangle and are grouped into superficial and deep (subinguinal) lymph nodes, relative to the deep fascia [2].

Superficial inguinal nodes – The superficial inguinal lymph nodes arise from the superior part of the femoral canal immediately below the inguinal ligament. These facilitate drainage of the penis, scrotum, labia, perineum, buttock, and lower abdominal wall [3,4].

Subinguinal nodes – The subinguinal nodes are classified as either superficial or deep.

-The superficial subinguinal nodes can be identified on both sides of the superior aspect of the great saphenous vein. These lymph nodes mainly receive afferents from the superficial lymphatic vessels of the lower extremity and also provide adjunctive drainage to the penis, scrotum, labia, perineum, and buttock.

-The deep subinguinal nodes are situated below the deep fascia, along the medial to the femoral vein. These nodes receive afferents from the deep lymphatic trunks, including the femoral vessels, the lymphatics from the glans penis or clitoris, and some of the efferents from the superficial subinguinal glands [1,3,4].

ETIOLOGIES — Lymphedema of the lower extremities can be primary or secondary, but secondary lymphedema is much more common in the lower extremity than primary lymphedema and often associated with treatment of malignancy, trauma, or infection [5].

Primary lymphedema — Lymphedema that presents without an inciting factor is termed primary lymphedema [6]. Primary lymphedema, which is associated with pathologic development of the lymphatic vessels, most commonly involves the lower extremities but can occur in the upper extremities. (See "Clinical features and diagnosis of peripheral lymphedema", section on 'Primary'.)

There are many chromosomal or genetic conditions in which lymphedema is a reported feature [7]. If there is a genetic inheritance pattern arising without an etiology, the term familial lymphedema can be used to categorize this type of lymphedema. Some genetic disorders such as Fabry's disease (Anderson-Fabry's disease) may be associated with the development of lymphedema, but it is considered secondary lymphedema [8]. (See "Clinical features and diagnosis of peripheral lymphedema", section on 'Epidemiology and risk factors'.)

Primary lymphedema is categorized according to the age at which lymphedema symptoms first present.

Congenital lymphedema – Congenital lymphedema is defined as lymphedema that is present at birth or presents within the first year of life. Congenital lymphedema may be either sporadic or familial. It is estimated that congenital lymphedema accounts for about 10 to 25 percent of primary lymphedema cases, affecting females twice as often as males [6].

Lymphedema praecox – Lymphedema praecox is the most prevalent type of primary lymphedema, presenting between ages 1 to 35, most often during puberty. Similar to congenital lymphedema, lymphedema praecox is more frequent in females than males (ratio of 4:1). Seventy percent of patients with lymphedema praecox present with unilateral lower extremity lymphedema [9].

Lymphedema tarda – Lymphedema tarda is the least common type of primary lymphedema, accounting for <10 percent of cases. Lymphedema tarda manifests clinically after age 35, typically affecting the lower extremities. As with other types of primary lymphedema, lymphedema tarda is more common in women than men [10].

Secondary lymphedema — Secondary lymphedema develops as a result of another condition or treatment. Secondary lymphedema may be secondary to cancer or cancer treatment, infection, trauma, surgery, or obesity. (See "Clinical features and diagnosis of peripheral lymphedema".)

Cancer and cancer treatment — Cancer-related lower extremity lymphedema has been reported as a complication following the treatment of extremity melanoma, gynecologic, or genitourinary malignancies. The incidence of cancer-related lymphedema varies by type of malignancy and treatment. In a systematic review that included 43 studies (genitourinary, gynecologic, lower extremity melanoma), the overall incidence of lower extremity lymphedema was estimated to be 20 percent [11].

Gynecologic cancers — The incidence of lower extremity lymphedema in patients with a history of gynecologic cancers is as high as 25 percent [11,12]. Treatment for gynecologic cancers often involves a combination of radiotherapy, chemotherapy, and surgical procedures. Surgical interventions that remove lymph nodes significantly increase the risk of developing lower extremity lymphedema. The risk is even higher when patients undergo postoperative radiotherapy [13]. Clinical interventions to prevent and manage postcancer lower extremity lymphedema are needed to preserve and improve the QoL in this patient population.

The incidence of lower extremity lymphedema following treatment for gynecological cancer varies depending upon the type of gynecological cancer [14].

Endometrial cancer: 1 to 38 percent

Cervical cancer: 17 to 81 percent

Vulvar/vaginal cancer: 6 to 75 percent

Ovarian cancer: 5 to 21 percent

Genitourinary cancers — The pooled incidence of lymphedema in patients with a history of genitourinary cancer (bladder, penile, prostate) is approximately 11 percent. The incidences for bladder, penile, and prostate cancers are approximately 16, 21, and 4 percent, respectively.

Patients who undergo radiation therapy in addition to lymph node dissection for the treatment of genitourinary cancers are at an increased risk of developing lymphedema [11]. The observed incidence of lower extremity lymphedema is higher among patients who undergo an extended pelvic lymph node dissection (ePLsD) compared with a standard pelvic lymph node dissection (sPLND) [15,16]. For the treatment of genitourinary cancers, in one review, the incidence of lower extremity lymphedema was 1.4 percent following sPLND and 8.8 percent following an ePLND [15].

Melanoma — Lower extremity lymphedema related to treatment of melanoma is primarily due to inguinal lymph node dissection required for the treatment of disease. However, direct nodal invasion can also result in lymphedema. The estimated incidence of lymphedema associated with melanoma is 16.3 percent overall, and 28 percent for lower extremity melanomas [11].

The incidence of moderate lymphedema among patients who have had a total lymph node dissection has been reported to be as high as 46 percent [17]. A high incidence of 42 percent has also been reported among patients with melanoma who have undergone a deep (iliac/obturator) lymph node dissection [11]. However, the adoption of sentinel lymph node (SLN) biopsy for cancer staging (figure 3) has allowed surgeons to avoid extensive lymph node dissections, reducing the risk of lymphedema in node-negative patients. Patients with melanoma are still at risk for developing lymphedema following SLN biopsy, but the risk of lymphedema was significantly lower for patients who undergo SLN biopsy alone compared with lymph node dissection in a prospective assessment of 182 patients [17]. At one year following lower extremity melanoma surgery (SLN biopsy and total lymph node dissection), the overall incidence of moderate lymphedema (limb volume change >10 percent) was reported to be about 27 percent.

Kaposi sarcoma — Kaposi sarcoma is a rare sarcoma involving the cells lining the blood and lymphatic vessels that could contribute to secondary lymphedema [18,19]. The disease is typically limited to the lower extremities, although it may be more widespread in immunocompromised patients, such as patients with AIDS or those who have undergone a solid organ transplant. Lymphedema may occur in this population as a result of blocked lymphatic vessels, changes in the permeability of the lymphatics, regional lymph node involvement, and increased inflammatory cytokines [20].

Infection — Infection can be both a direct cause of lymphedema and a risk factor for developing lymphedema. Early postoperative infections following lymph node dissection significantly increase the risk of lymphedema [10]. Cellulitis and infections have also been reported to increase the risk of gynecologic-related lymphedema.

Parasitic infections may also result in lower extremity lymphatic obstruction. The most common cause of secondary lymphedema worldwide is filariasis, a disease in which parasitic worms occupy and occlude lymphatic vessels [21]. The Centers for Disease Control and Prevention estimate that lymphatic filariasis affects over 120 million people in 72 countries throughout the tropics and subtropics of Asia, Africa, the Western Pacific, and parts of the Caribbean and South America [22]. (See "Lymphatic filariasis: Epidemiology, clinical manifestations, and diagnosis" and "Lymphatic filariasis: Treatment and prevention".)

Chronic venous insufficiency — Lymphedema shares many clinical features with chronic venous insufficiency (CVI). A careful evaluation can indicate if edema is the result of a venous inefficiency, lymphatic insufficiency, or a combination of both. (See "Clinical manifestations of lower extremity chronic venous disease".)

Lymphedema caused by insufficiency of both the venous and lymphatic systems is known as phlebolymphedema (PLE). PLE may be caused by a congenital defect of the venous and lymphatic systems or secondary to CVI. CVI results in an excessive fluid load at the tissue level, creating additional load to the lymphatic system. The increase in lymphatic flow may become much greater than the lymph transport capacity [23,24]. (See "Overview of vascular intervention and surgery for vascular anomalies" and "Pathophysiology of chronic venous disease".)

Obesity — Obesity is an independent risk factor for lymphedema (ie, obesity-related phlebolymphedema). Body mass index (BMI) greater than 50 may lead to the development of lymphedema without a history of lymphatic surgery or injury [25]. Although the exact pathophysiology is not completely understood, obesity has been implicated in the development of lymphedema due to increased production and retention of fluid by adipose tissue [26,27]. Patients with severe obesity may also be at risk for developing massive localized lymphedema (MLL; pseudotumor, pseudosarcoma), which is the formation of a large lymphedematous mass found in the lower extremity (picture 1) [28]. MLL is a benign, painless mass, often enlarging over many years [29].

The impact of obesity on lymphedema rates following cancer and cancer treatment has also been studied [10]. The effect of BMI cannot be completely separated from cancer treatment effects, in part because of potentially more difficult lymph node dissection in patients who are obese [30].

Extremity trauma — Injury to the lymphatic system is the cause of post-traumatic edema in 10.5 percent of patients [31,32]. Post-traumatic edema may originate from injured lymphatics, deep venous thrombosis (high risk associated with extremity trauma), or inflammation.

Traumatic injuries to the lower extremity that can cause damage to the lymphatic system include degloving injury (Morel-Lavallee injury), multiple fractures, compartment syndrome, and joint replacement. (See "Severe lower extremity injury in the adult patient".)

Orthopedic surgery — In addition to surgical treatment for malignancy, the lymphatic system may become damaged during orthopedic surgeries. While postsurgical edema is common, patients undergoing orthopedic surgeries are at risk of developing chronic lymphedema. Total hip and total knee arthroplasty are both associated with a potential risk of developing lower extremity lymphedema [33]. (See "Total hip arthroplasty" and "Total knee arthroplasty".)

Others — Other potential etiologies of lymphedema include thyroid dermopathy and inflammatory disorders, dermatitis, and sarcoidosis [6].

Lymphedema is a rare extraarticular manifestation of inflammatory joint disease and is most often seen in patients with rheumatoid or psoriatic arthritis. (See "Overview of the systemic and nonarticular manifestations of rheumatoid arthritis", section on 'Lymphatic obstruction'.)

CLINICAL EVALUATION — There are a number of preoperative evaluations that aid in the diagnosis of lymphedema, confirm a suspected cause, and evaluate the impact of this condition on a patient's life. The results are used to determine which nonsurgical and surgical treatments may benefit the patient.

Lower extremity clinical features — The treating clinician should first conduct a review of the patient's medical history and perform a physical examination. The history should include age of lymphedema onset, affected areas, details of disease progression, history of trauma, medical history, surgical history, and family medical history [34]. Physical examination should assess swelling, skin changes, and infection. The physical examination includes an assessment of the skin, soft tissues, and vascular system of the affected limb(s) [35]. (See "Clinical features and diagnosis of peripheral lymphedema", section on 'History and physical'.)

The inability or failure to pinch or pick up a fold of skin at the base of the toe (ie, Stemmer sign) is indicative of lymphedema complicated by skin fibrosis; however, a negative Stemmer sign does not rule out lymphedema. Because lymphedema provokes fat deposition, pitting will not occur with later stages of lymphedema, though it may be present early on. With increasing disease severity in the lower extremity, skin changes and fat deposition are progressive, leading to subcutaneous fibrosis, cobblestoning, skin overgrowth, and wart-like changes (lymphostatic verrucosis, lymphostatic papillomatosis).

In the lower extremity, foot swelling ("buffalo hump") or toe swelling ("boxcar toes") may be present but are not required to make a diagnosis of lymphedema. Foot involvement is more likely to be seen with primary lymphedema compared with secondary lymphedema. The presence of dysmorphic toes, transverse digital creases, and hypoplastic or upturned ("ski-jump") toenails is more indicative of primary lymphedema [36]. But while primary lymphedema is typically associated with marked foot/toe swelling and dysmorphic features, different primary lymphedema phenotypes may have varying degrees of foot swelling, and some patients with primary lymphedema can have foot sparing. Secondary lymphedema is typically but not invariably associated with only modest foot/toe swelling and no dysmorphic features, but there are exceptions.

Functional status should also be assessed during the examination. Some functional observations for patients with lower extremity lymphedema may include the presence of a limp, use of an ambulation aid, ability to bend over to remove shoes/socks, and ability to make a forward stride. If functional limitations are observed, the patient should be referred to physical therapy for evaluation and treatment [37]. Validated questionnaires, such as the Lymphedema Life Impact Scale, may also be used to measure patient-reported functional impairments, as well as physical and psychosocial impairments caused by lymphedema [38].

Lower extremity measurements — Lower extremity circumference measurements can be easily obtained, and the circumference values can be used to calculate limb volume. All methods of limb volume measurement are effective and accurate when properly performed [35,39].

Limb circumference measurements can be taken at any point on the leg, as long as the anatomic landmarks used are consistent [34,35,39]. Measurements should be taken from the affected as well as unaffected extremity for comparison. Extremity measurements should be taken during the initial evaluation and during follow-up visits to assess the status of the disease and its response to treatment (ie, pre- and post-treatment measurements). (See "Clinical features and diagnosis of peripheral lymphedema", section on 'Limb circumference'.)

In addition to calculating limb volume from circumference measurements, perometry and water displacement may be used to measure limb volume. Water displacement is considered the "gold standard" for assessing volume but is cumbersome and not frequently used [39]. Perometry uses infrared light to scan a limb and calculate highly accurate volumes but is expensive due to a high equipment cost. (See "Clinical features and diagnosis of peripheral lymphedema", section on 'Limb volume'.)

Another way to assess lymphedema is by using bioimpedance spectroscopy (BIS). This device measures fluid content, calculating the ratio of extracellular fluid by measuring the resistance to an electrical current in the measured limb. A benefit of this measurement is that fluid can be detected as it starts to accumulate, even before it may be physically noticeable to a patient or provider [37].

Diagnosis and clinical staging — For some patients with lower extremity lymphedema, a diagnosis can be made with history and physical examination alone (eg, most cases of secondary lymphedema). However, further imaging is used whenever the diagnosis is in question, which is more typically in patients with primary lymphedema or lipedema.

Several staging systems are used to classify the severity of lymphedema. Using the International Society of Lymphology (ISL) criteria (table 1), lymphedema is staged based on the examination of the lower extremity and the volume difference between the extremities as stage 0 through stage III, which correspond to subclinical lymphedema, mild lymphedema, moderate lymphedema, and severe lymphedema [40].The Campisi staging system stages lymphedema from stage 1 to stage 5 (table 2). Clinical staging is important as it affects the treatment plan for lymphedema. (See "Clinical staging and conservative management of peripheral lymphedema", section on 'Clinical stage'.)

Vascular and soft tissue imaging — Duplex ultrasound should be performed on every patient with lymphedema to assess the patency and competency of the venous system (ie, rule out deep venous thrombosis) and to identify the presence of venous reflux. The grayscale (B-mode) evaluation of tissue layers in the affected limb can also provide information on the etiology, as well as the severity of lymphedema [41].

Cross-sectional imaging using magnetic resonance (MR) or computed tomography (CT) may identify enlarged lymph nodes or other lesions that can cause lymphatic obstruction. MR or CT imaging of the extremity can detect the presence of increased interstitial fluid (honeycomb appearance), but this finding is nonspecific [39,42]. Common CT findings in patients with lymphedema include thickening of the skin, thickening of the subcutaneous compartment, increased fat density, and thickened perimuscular aponeurosis. Common MR findings include circumferential edema, increased volume of subcutaneous tissue, and marked thickening of the dermis [43,44]. MR can differentiate the cutaneous edema of lymphedema from other types of limb swelling such as lipedema and phlebedema.

Lower extremity lymphatic imaging — In addition to vascular and soft tissue imaging, lymphatic imaging can be used to evaluate lower extremity lymphedema. Studies such as lymphoscintigraphy, MR lymphangiography, and indocyanine green (ICG) lymphangiography may be performed for a variety of reasons in patients with lower extremity swelling. For patients suspected of having primary lymphedema, these studies are instrumental in confirming the diagnosis. For patients with secondary lymphedema, these same studies are useful for gauging the severity of lymphedema. For patients who are proceeding with surgical treatment of the lymphedema, lymphatic imaging studies are used to identify patent lymphatic vessels. Although there are less invasive measures to monitor patients in the postoperative setting, lymphatic imaging can also be used to assess the degree of improvement after surgical intervention. (See "Clinical features and diagnosis of peripheral lymphedema", section on 'Imaging the lymphatic system'.)

Lymphoscintigraphy is the primary imaging test used for diagnosis and functional assessment of the lower extremity lymphatics. Radioactive tracer injected into the dermis in the foot may demonstrate slow or absent lymphatic flow, areas of dermal backflow, and abnormalities of lymphatic uptake [35]. This study can evaluate the larger superficial lymphatic vessels and nodes, but not the deep transport lymph vessels [39].

MR lymphangiography and ICG lymphangiography are used predominantly for mapping prior to surgery, rather than for diagnostic purposes. Identification of patent lymphatic channels is necessary to perform lymphovenous bypass. (See 'Preoperative lymphatic imaging' below.)

CONSERVATIVE CARE — The initial approach for the management of extremity lymphedema (upper or lower) begins with conservative management, which involves a combination of self-care (ie, skin care, weight management), physiotherapy, and compression therapy (ie, compression bandaging, compression garments) [35]. While these treatments do not address the underlying cause of lymphedema, they can control swelling and prevent development of long-term sequelae such as irreversible skin changes (ie, elephantiasis) [10]. Patients with mild lower extremity lymphedema may be adequately controlled with conservative measures.

The type and level or intensity of physiotherapy (simple lymphatic drainage, manual lymphatic drainage, complete decongestive therapy) and compression therapy (compression bandaging, compression garments, intermittent pneumatic compression) vary depending upon the stage of disease (table 1). (See "Clinical staging and conservative management of peripheral lymphedema", section on 'Conservative treatment by severity'.)

For patients with mild lymphedema (International Society of Lymphology [ISL] stage I), we suggest physiotherapy (simple lymphatic drainage, a commonly taught self-help maneuver) and compression garments, rather than more intensive therapy.

For patients with moderate-to-severe lymphedema (ISL stage II to III) and no contraindications, we suggest intensive physiotherapy, usually in the form of complete decongestive therapy, rather than less intense therapy.

Patients with severe lymphedema (ISL stage III) may also benefit from intermittent pneumatic compression (IPC) in addition to complete decongestive therapy.

Unfortunately, lower extremity lymphedema often progresses in spite of conservative treatment measures. Patient compliance can be difficult due to the time commitment, lifelong need for ongoing treatment, limited availability of certified lymphedema therapists, expense, insurance coverage issues, and patient discomfort due to the bulkiness of compression garments and use during high temperatures [6,13,45].

Conservative treatments are also recommended following surgical treatments to achieve optimal outcomes [13]. (See 'Surgery for lower extremity lymphedema' below.)

Skin care — Patients with lymphedema have an accumulation of protein-rich fluid in the interstitial space. This increased protein concentration can trigger inflammation, causing skin changes in patients with advanced stages of lymphedema. These changes may include dry skin and decreased elasticity, making a patient more susceptible to infection and ulceration [13]. Patients are advised to maintain good hygiene and to keep the affected limb properly moisturized [6]. Patients should seek medical attention if they observe any abnormalities on their affected limb or suspect that an infection may be present. (See "Clinical staging and conservative management of peripheral lymphedema", section on 'General measures'.)

Compression therapy — External compression is used with the aim of decreasing interstitial fluid production and reducing excess lymph fluid in the affected extremity. Different methods of compression include multilayer short stretch compression bandages, compression garments, and pneumatic compression devices. The benefits of compression therapy are largely dependent on patient compliance [13]. (See "Clinical staging and conservative management of peripheral lymphedema", section on 'Compression therapy'.)

Compression bandaging provides the greatest volume reduction in the early stages of lymphedema. For lower limb lymphedema, a minimum of two layers of bandages is most effective [6].

Compression garments are custom-made elastic stockings that are used in the maintenance phase of lymphedema treatment to effectively prevent fluid reaccumulation [46,47]. New garments must be created when there is a decrease in limb volume in order to maintain these improvements.

Compression garments are also an important component of surgical interventions for lymphedema. Compression therapy is used preoperatively to maximize limb volume reduction and improve surgical outcomes. Many patients are also required to continue compression therapy following surgical intervention to maintain long-term improvements.

IPC is another form of compression therapy that applies pressure to the affected limb during an inflation and deflation process. This method is widely used in the treatment of lymphedema and plays an important role in the movement of lymph fluid [13]. Following use of an external pump, patients are still required to wear compression garments due to the risk of excess fluid recurrence.

Decongestive therapy — Complete decongestive therapy (CDT), also known as complex physical therapy (CPT), is the standard approach for the initial treatment of lymphedema. (See "Clinical staging and conservative management of peripheral lymphedema", section on 'Complete decongestive therapy'.)

The first phase of treatment uses a multimodality approach under the care of a lymphedema specialist to reduce extremity volume. This intensive phase includes manual lymphatic decompression (MLD), compression garment use, exercise, and skin care. MLD is a lymphatic massage technique that enhances filling of the cutaneous lymphatics and improves dilation and contraction of the lymphatic vessels [47]. Compression bandaging/garments are applied immediately following MLD, preceded by completion of a series of exercises to increase lymphatic flow [6]. MLD is an important component for achieving successful results with complete decongestive therapy. However, MLD alone is not recommended to attempt to achieve volume reduction [13]. (See "Clinical staging and conservative management of peripheral lymphedema", section on 'Manual lymphatic drainage'.)

Compression therapies and manual lymphatic drainage are generally contraindicated in the setting of active infection. Infection is typically diagnosed by the presence of increased temperature, erythema, and pain in the affected extremity. Cellulitis/lymphangitis should be treated with antibiotics appropriate for common skin flora (particularly Streptococcus species). Patients with signs of systemic sepsis (eg, fever, elevated white count, hemodynamic instability, altered mental status) may require admission to the hospital for parenteral antibiotics and limb elevation. (See "Cellulitis and skin abscess in adults: Treatment" and "Lymphangitis".)

After reducing the volume of the affected lower extremity, the second phase of CDT begins and is focused on volume maintenance. During phase 2, patients continue to wear compression garments and practice a healthy skin care regimen. Patients are also advised to continue exercise and perform MLD, as necessary [6,13,44,48].

Numerous studies have supported the effectiveness of CDT for lower extremity lymphedema compared with physical exercise or compression therapy alone. CDT achieves an average volume reduction between 31 and 73 percent in patients with lower extremity lymphedema [45,49-52]. However, the effects of CDT are not permanent and require lifelong continuation of phase 2 therapy to prevent recurrence. For patients who are relying on conservative therapies, self-care, daily use of compression garments, and daily exercise are all required for the successful long-term management of lymphedema [13].

SURGERY FOR LOWER EXTREMITY LYMPHEDEMA — Advancements in the field of microsurgery have improved options and the reliability of surgical treatment for lymphedema. Microsurgical intervention using lymphovenous anastomosis (LVA) or vascularized lymph node transfer (LNT) are the only potential options to halt, reverse, or minimize the degree of progression. Surgical treatment has been found to benefit patients with all clinical stages of lymphedema [53,54].

Overall, patients with International Society of Lymphology (ISL) stage I or II (table 1) lymphedema of the lower extremities do well with LVA while patients with stage III and IV lymphedema do better with LNT. However, patients with ISL stage III or IV lymphedema are often not candidates for LVA because there are no patent lymphatic vessels. A combination of LVA and LNT may offer improved results compared with either procedure alone, but this approach would most likely be applied to patients with earlier stages of disease because patients with more advanced stages of lymphedema often do not have any patent lymphatic channels to perform a combined procedure. (See 'Lymphaticovenous anastomosis' below and 'Free lymph node transfer' below and 'Combined procedures' below.)

A thorough preoperative assessment is essential when determining the most effective surgical treatment plan for each individual patient. It is important to note that these procedures do not eliminate the need for ongoing conservative care to maintain benefits long term. Patients must also be educated and willing to comply with the necessary pre- and postoperative protocol (eg, lymphedema therapy, compression garment use).

Indications — Indications for the surgical treatment of lower extremity lymphedema are listed below and discussed more fully separately. Earlier intervention prior to tissue fibrosis and severe adipose deposition is more effective for treating lower extremity lymphedema. (See "Surgical treatment of primary and secondary lymphedema", section on 'Indications for surgery'.)

Indications include:

Lack of improvement or progression in spite of conservative measures (see 'Conservative care' above)

Nonoperative management that has reached a plateau

Recurrent cellulitis

Limitation of function (eg, mobility, contracture)

Leakage of lymph into body cavities, organs, or externally

Deformity or disfigurement

Pain

Diminished quality of life, including emotional or psychosocial distress

Preoperative lymphatic imaging — When planning microsurgical intervention, it is beneficial to review the results of indocyanine green (ICG) or magnetic resonance (MR) lymphography to help locate functional lymphatic channels for potential anastomosis [55-57]. Physiologic derangement of lymphatic drainage in the affected limb can be proven using ICG or MR lymphangiography [58,59].

ICG lymphangiography — ICG lymphangiography may be used preoperatively as well as intraoperatively to visualize lymphatic flow and identify any abnormalities. During this test, ICG is injected into the skin and imaged with a dynamic infrared florescence camera [39,56,57]. The severity of lymphedema can be graded depending on the diffusion pattern of ICG. In "normal" lymphatic systems, a linear pattern will appear. Patterns for the lower extremity are similar with those of the upper extremity (image 1). Splash, stardust, and diffuse patterns indicate an increasing severity of lymphedema and increased levels of fibrosis in the lymphatic channels [48].

One limitation of ICG lymphangiography is that only lymphatics within 2 cm of the skin's surface can be seen. To overcome this limitation, it has been recommended that MR lymphangiography also be performed, improving surgical planning by providing a surgeon with imaging of both the superficial and deep lymphatic systems [56].

The results of ICG lymphangiography can help determine an appropriate surgical intervention. It can help the surgeon identify potential sites for LVA surgery. According to Cheng's Grading System of Lymphedema and Available Options for Management, LVA may be a treatment option for patients who exhibit a linear, splash, or stardust ICG pattern (table 3 and picture 2) [60]. While there is no guideline for recommended number of LVAs, a greater number of anastomoses could be beneficial for reducing interstitial fluid levels [61]. Patients without active functional lymphatic channels would not be appropriate candidates for LVA.

When considering a lymph node transfer surgery, ICG lymphangiography can be used to locate the lymph nodes that drain the limb at a donor site. By identifying and excluding these from harvest at the time of a lymph node transfer procedure, the risk of developing donor site lymphedema can be greatly reduced [39,56,57].

MR lymphangiography — MR lymphangiography is a modification of 3D volumetric contrast-enhanced MR imaging that has been developed to depict the severity of lymphedema, the number and location of individual lymphatic channels, and the presence of dermal backflow. The imaging of individual subdermal lymphatic channels is useful for selecting which patients may be candidates for microsurgical lymphedema treatment.

Compared with lymphoscintigraphy, MR lymphangiography can depict individual lymphatic channels, does not involve ionizing radiation, and requires a shorter examination time. However, it has limited availability, high cost, is uncomfortable (dye injection), and there is a potential for damage to the lymphatics as a result of dye injection.

MR lymphangiography consists of two primary sequences: a 3D heavily T2-weighted sequence to depict the severity and extent of the lymphedema, and a high-resolution fat-suppressed 3D spoiled gradient-echo sequence performed after an intracutaneous injection of gadolinium-based MR contrast to obtain an MR venogram. The MR venogram is used to differentiate lymphatics from veins (figure 4 and image 2) [55,62].

Techniques and effectiveness — Surgical treatments for lymphedema can be categorized as physiologic or reductive techniques. Physiologic techniques repair normal or create alternate pathways for lymph fluid to properly flow out of the affected limb. Reductive or "ablative" techniques surgically remove the edematous and fibrotic soft tissues in a lymphedematous limb [54]. Proper patient selection and careful surgical planning decrease the risk of potential complications, optimize potential physical benefits, and achieve an increased quality of life [63,64].

Physiologic techniques — Physiologic techniques improve lymphatic drainage by surgically creating bypasses and/or transferring lymph nodes. A systematic review identified four studies involving nearly 2000 reconstructive procedures for lower extremity edema [53]. The reported volume reductions were between 42 and 59 percent. (See "Surgical treatment of primary and secondary lymphedema", section on 'Physiologic techniques'.)

Physiologic procedures may be indicated for the following:

Failure of nonoperative management

Nonoperative management that has reached a plateau

Recurrent cellulitis or lymphangitis

Dissatisfaction with compression garments or impaired quality of life

Contraindications for physiologic procedures include:

Medical comorbidities precluding a safe surgical procedure

Patient noncompliance with compression therapy or postoperative care plans

Lymphaticovenous anastomosis — LVA is a microsurgical procedure that bypasses diseased lymphatics and restores adequate lymphatic drainage via direct drainage into the venous system. (See "Surgical treatment of primary and secondary lymphedema", section on 'Lymphaticovenular anastomosis'.)

During this procedure, the distal lymphatics are anastomosed to small superficial veins, creating a "bypass" for the lymphatic fluid into the venous system (ie, subdermal lymphaticovenular bypass [65]). Patients must have partially functional lymphatic vessels in order to create an effective anastomosis. The number and location of anastomoses varies based on the presence of functional and accessible vessels in each patient [66,67]. Both preoperative and intraoperative lymphatic mapping is necessary to achieve superior results following LVA [54]. ICG imaging provides the necessary lymphatic mapping to aid in identifying candidates for LVA and can also provide intraoperative imaging. ICG differentiates between patients who still have some degree of functioning lymphatics, evidenced by proximally progressing lines on the extremity, and those who do not, evidenced by a diffuse pattern. In those with functioning lymphatics, ICG imaging also provides direct data on the patient to indicate the location of surgery. (See 'ICG lymphangiography' above.)

LVA effectively treats lymphedema in the lower extremity. LVA is most effective for ISL stages I to IIa or Campisi stages Ib to IIIb (table 1), at which time the lymphatic vessels are still able to transfer lymph fluid [54]. The mean volume reduction after LVA continuously decreases as lymphedema stage increases. A study supporting this correlation reported limb volume reductions as 78.5 percent for stage II patients, 54.8 percent for stage III patients, and 47.4 percent for stage IV patients [68]. In a prospective study that included patients with lower extremity lymphedema, symptom improvement was reported by 96 percent of patients and quantitative improvement occurred in 74 percent of patients undergoing LVA [69]. The authors also noted a significant improvement in identification of lymphatics through the use of preoperative ICG imaging. LVA was also may also be useful for treating lymphatic disease secondary to lymph vessel injuries of the pelvis and groin, with rapid resolution and excellent outcomes reported. In one small retrospective review, lymphatic leakage ceased after a mean of six days after pelvis or groin LVA [70].

For lower extremity lymphedema, LVA also decreased pain scores. In a small study, the average visual analog scale scores decreased from 5.3 preoperatively to 1.8 postoperatively [58]. The average change in limb circumference in their sample was -4.7 percent.

Patient satisfaction following LVA for lower extremity lymphedema is high, with the most common improvements including a decrease in limb size, decreased weight of limb, improved quality of life, softer and improved texture of skin, and better-fitting clothing. In a study evaluating the correlation of limb volume reduction following LVA and quality of life, median volume reductions of 11 percent for unilateral lymphedema and 8 percent for bilateral lymphedema corresponded with a 23 and 14 percent improvement, respectively, in patient-reported quality of life using Lymphoedema Quality of Life Questionnaire (LYMQOL) [71]. A significant reduction in the frequency of infections, specifically cellulitis, has also been reported after LVA [68,72].

When LVA was first described, it was suggested that patients with primary lymphedema were not candidates for this procedure. Studies have since shown that LVA can also effectively treat patients with primary lymphedema. In most primary lower extremity lymphedema cases that have been studied, it has been possible for surgeons to find healthy lymphatics suitable for anastomosis. Reduction in lower extremity limb volume after LVA is similar for secondary and primary lymphedema [73].

Complications following LVA are low, with rates around 5.9 percent, including infection, lymphatic fistula, partial skin ulceration, and wound dehiscence [67]. Contraindications for this procedure include venous incompetence, venous hypertension, and a lack of functional lymphatics on ICG imaging. (See "Surgical treatment of primary and secondary lymphedema", section on 'Physiologic techniques'.)

Free lymph node transfer — Free or vascularized LNT is another physiologic microsurgical technique used for the treatment of lymphedema [74]. LNT involves a free tissue transfer of lymph nodes taken from one area of the body and transferred to the area of lymph node deficiency, to improve drainage of the affected limb. There are two proposed mechanisms for improved lymphatic flow with LNT: lymphovenous communication preexisting within the transferred lymph nodes and via efferent lymphatic vessels from the lymph nodes [61]. (See 'Preoperative lymphatic imaging' above and "Surgical treatment of primary and secondary lymphedema", section on 'Vascularized lymph node transplant'.)

The recommended indication for LNT is stage 2 or higher lymphedema (ISL) (table 1) with repeated episodes of cellulitis (ie, but no acute cellulitis) and failure to improve from complete decongestive therapy for six months [75,76]. Known surgical removal of lymph nodes and/or previous radiation therapy in an affected limb or a history of trauma can all be treated with LNT.

Potential lymph node donor sites include the groin, axilla, supraclavicular, and submental regions, and some advocate using omental lymph nodes or mesenteric lymph nodes to eliminate secondary lymphedema related to the donor site [65]. For lower extremity lymphedema, the groin, popliteal fossa, and ankle have all been described as potential recipient sites. The groin is the most common recipient site but often requires extensive lysis or excision of scar tissue in patients with a history of previous groin surgery and radiation (figure 5) [48,77].

The risk of complications following an LNT is low but may include flap loss, donor site lymphedema, seroma, lymphocele, infection, and wound healing complications [48]. Donor site lymphedema has been minimized with the use of reverse lymphatic mapping (RLM). This technique uses ICG fluorescence lymphography and/or radioactive tracer injection into the limb adjacent to the donor site, allowing a surgeon to identify which lymph nodes at the donor site drain the extremity to avoid harvesting them, or to select an alternative donor site. Using this technique, one review of 60 LNTs reported significant improvement in more than 50 percent of their patients with no cases of donor site lymphedema [78].

LNT is associated with high rates of improvement. Reductions in lower extremity volume and limb circumference occur as early as three months after LNT, with continued, significant improvements often being observed up to 12 months after surgery [48,54,77,79-82]. In one of the largest studies, 98 percent of patients reported experiencing some degree of improvement following LNT [83]. Larger total volume reductions have been reported in patients with lower preoperative excess volumes [79]. In a study of 35 patients undergoing submental vascularized LNT to the lower extremity for lymphedema due to treatment of gynecological cancer, all patients had significant reductions in limb circumference as well as reduction in the incidence of cellulitis [84].

Health-related quality of life scores also improve significantly following LNT for lower extremity lymphedema. At three months postoperatively, improvements in mood have been noted, and by six to nine months postoperatively, patients have reported improvements in symptoms, appearance, and function [80]. Another notable improvement following LNT is a decrease in the frequency of infections, which is a major health and financial burden associated with lymphedema. The reduction of this burden may also contribute to patient-reported improvements in quality of life [80,81].

Reductive techniques — Reductive techniques surgically debulk the affected limb using direct excision and/or liposuction. Patients who are not candidates for, do not see much improvement from, or do not wish to undergo a physiologic procedure may benefit from these techniques. While they do not improve lymphatic function, they can reduce the circumference and weight of lymphedematous limbs, resulting in improved function [85]. A retrospective review of surgical reduction using skin and subcutaneous tissue excision reported that 70 to 79 percent of lower extremity patients experienced a significant reduction in extremity size, improved function, and contour following surgery [86]. Reductive techniques can also be performed before or after a physiologic procedure to further improve surgical outcomes. (See "Surgical treatment of primary and secondary lymphedema", section on 'Reductive techniques' and 'Combined procedures' below.)

Direct excision — Direct excision involves complete excision of the tissue above the deep fascia with preservation of the overlying dermis, or with removal of the dermis, using skin grafting for wound closure [54,67]. Direct excision was first described in 1912 as the "Charles procedure" and involved circumferential excision, which often resulted in worsened lymphedema distal to the excision. While the Charles procedure is rarely used today, excision of localized areas of severe lymphedema is commonly performed.

Direct excision has successfully treated many cases of severe lower extremity lymphedema and may be the only, and possibly best, treatment option for patients with severe lower extremity lymphedema suffering from impaired physical function, recurrent infections, skin ulcerations, pain, and poor quality of life [75,87-89]. Indications for excision include advanced-stage lymphedema, lymphedema that does not improve from conservative treatment, recurrent episodes of cellulitis, and severe disfigurement or dysfunction. It is particularly useful for patients with massive localized lymphedema (MLL). These masses can weigh upwards of 30 pounds on each leg and can severely limit mobility, quality of life, and the ability to undergo complete decongestive therapy and compression therapy; excision can provide immediate improvement [29,90,91].

Excision can be effective in reducing extremity size, improving quality of life, and improving function. Potential complications include wound dehiscence, contour irregularities, scarring, infection, and skin graft failure that may require additional surgery [44]. Multiple modifications have been made to this procedure to improve outcomes. Examples include the use of negative-pressure dressing after an initial debulking surgery to delay skin grafting and a staged excision of skin and subcutaneous tissue to minimize morbidity or to address specific pockets of tissue [75,88,89].

Liposuction — Liposuction is a well-established surgical technique with a low complication rate. Lymphedema liposuction involves the removal of excessive adipose tissue that typically presents during late-stage lymphedema using a minimally invasive technique [61,67]. Liposuction does not improve lymphatic function but can reduce excess volume in affected limbs. Even when signs of fibrosis are present, power-assisted liposuction can facilitate the breakdown of the tissue, particularly in lower extremity lymphedema [35]. For patients who have been appropriately selected for liposuction, recurrence can be prevented with postoperative compression therapy [67,92,93].

Patients with stage II or III lymphedema (primary or secondary) who have failed conservative treatment may be candidates. Contraindications to liposuction include inadequate prior conservative management and noncompliance with compression. Prior to surgery, treatment with compression garments and massage must be implemented to control lymphatic fluid until minimal or no pitting is present. Before proceeding with surgery, patients must express their understanding and motivation to comply with lifelong postoperative compression therapy [48,67,92,94].

Liposuction was first applied in a therapeutic setting to treat patients with upper extremity lymphedema, with high success rate for long-term reduction in excess arm volume [78,94]. Studies focused on the use of liposuction for lower extremity lymphedema have shown similar promising outcomes [92,95]. Maximal reduction in leg volume can usually be achieved around six months after surgery. In a large series of lower extremity lymphedema patients treated with liposuction, an average reduction in excess limb volume of 79 percent was reported at two years after surgery [96]. Volume reduction after liposuction may be even greater for patients with secondary lymphedema (101 percent excess limb reduction). Combined with compression therapy, liposuction achieves long-lasting reductions in lower extremity limb volume [93-100]. In one study of 69 patients (72 legs), excess reduction in leg volume over a five-year period was as high as 90 percent [92].

In addition to improvements in limb volume, liposuction improves quality of life. Significant subjective improvements in functional impairment, heaviness of limb, anxiety, perceived degree of swelling, self-consciousness, and impact on emotions have all been reported [92,93,96]. In a prospective study of 55 patients, average function scores improved from 7.4 preoperatively to 28.0 postoperatively using the Patient Specific Functional Scale (score ranges from "0" [not able to perform activities at all] to "30" [able to perform activities perfectly]) [93]. Patients with a history of cellulitis have a significant reduction in the number of annual episodes following lower extremity liposuction [92,96].

Combined procedures — Procedures that combine two physiologic techniques or combine physiologic and reductive techniques may offer additional benefits. Liposuction is a common adjunctive treatment option due to its low complication rate and its notable improvements in limb volume. It has been suggested that by combining microsurgery with liposuction, the need for ongoing compression therapy may be alleviated [39,67,93]. Liposuction may also be used as a secondary procedure following a primary physiologic surgery. Studies have shown that as many as 16 percent of LVA patients and 31.6 percent of LNT patients will benefit from an additional liposuction procedure postoperatively [67].

Combined physiologic procedures using LVA and LNT have been suggested to maximize patient outcomes as these two approaches work via different mechanisms. While these procedures are complex and require a surgeon with expertise in microsurgery, they have been found to decrease morbidity, resulting in greater improvements in quality of life and functional recovery [48,101].

In some patients, it may be appropriate to combine a physiologic and a reductive technique. Combining these procedures may be the most effective approach for treating severe lower extremity lymphedema. An evaluation of combined suction assisted protein lipectomy (SAPL) and LVA demonstrated a reduction in excess lower limb volume from 20.2 to 2.7 percent without damaging associated lymphatic vessels [102,103]. While reductive techniques such as liposuction can remove fatty and fibrotic tissue in the affected limb, an LNT and/or LVA procedure can actually improve lymphatic function.

Postoperative compression — To maximize the reduction in limb volume after surgery and prevent lymphedema recurrence following surgery, continued lymphedema therapy and compression therapy are essential. The combined treatment approach of surgical and nonsurgical techniques has proven to be effective in the treatment of primary and secondary lymphedema. Following a physiologic procedure, patients may eventually be able to discontinue compression garment use. However, the use of compression garment use is usually a lifelong requirement following reductive techniques to prevent recurrence [11,54,104].

Following LVA, the recommended timing to begin using compression garments postoperatively varies based on a surgeon's preference. Garment usage may begin immediately after surgery but is often not implemented until four weeks after the procedure. Compression garments are used for at least six months postoperatively [104,105]. However, if there is significant improvement in patients, the use of compression garments may be discontinued over time. Approximately 56.3 percent of patients are able to discontinue use after an LVA procedure [104].

Following LNT, compression garments may be worn immediately. It is often recommended to continue their use for at least two to six months after surgery [81]. Following LNT, as many as 78 percent of patients are able to discontinue compression therapy [104].

Following reductive procedures, ongoing compression therapy (24 hours/day) is essential for maintaining or further reducing limb volume and preventing recurrence. Compression garments are applied immediately following surgery and are most important during the first three months after surgery, when the most notable changes in volume occur. After initiating compression therapy, limbs should be measured during each follow-up visit and garments should be adjusted in response to changes in limb volume [92,106].

SOCIETY GUIDELINE LINKS — Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Lymphedema".)

INFORMATION FOR PATIENTS — UpToDate offers two types of patient education materials, "The Basics" and "Beyond the Basics." The Basics patient education pieces are written in plain language, at the 5th to 6th grade reading level, and they answer the four or five key questions a patient might have about a given condition. These articles are best for patients who want a general overview and who prefer short, easy-to-read materials. Beyond the Basics patient education pieces are longer, more sophisticated, and more detailed. These articles are written at the 10th to 12th grade reading level and are best for patients who want in-depth information and are comfortable with some medical jargon.

Here are the patient education articles that are relevant to this topic. We encourage you to print or e-mail these topics to your patients. (You can also locate patient education articles on a variety of subjects by searching on "patient info" and the keyword(s) of interest.)

Basics topics (see "Patient education: Peripheral lymphedema after cancer treatment (The Basics)")

Beyond the Basics topics (see "Patient education: Lymphedema after cancer surgery (Beyond the Basics)")

SUMMARY AND RECOMMENDATIONS

Lymphedema occurs when the load exceeds the transport capacity of the lymphatic system, which leads to the accumulation of protein-rich fluid (lymph) and fibroadipose tissue in the interstitium. Lymphedema in the lower extremity can result from injury, infection, or congenital abnormalities of the lymphatic system. Symptoms of lymphedema include limb swelling (including focal changes), skin changes, discomfort, and restricted range of motion. (See 'Classification and anatomy' above and 'Lower extremity clinical features' above.)

Conservative therapy is important and should be the initial treatment for patients with lower extremity lymphedema. Conservative treatment consists of a multimodality regimen that includes general measures for care, physiotherapy (eg, simple lymphatic drainage, manual lymphatic drainage, complete decongestive therapy), and compression therapy (compression bandaging, compression garments, intermittent pneumatic compression), the type and the intensity of which depends upon the clinical stage (table 1). (See 'Conservative care' above and "Clinical staging and conservative management of peripheral lymphedema", section on 'Conservative treatment by severity'.)

For patients with mild lymphedema (International Society of Lymphology [ISL] stage I), we suggest physiotherapy (simple lymphatic drainage, a commonly taught self-help maneuver) and compression garments, rather than more intensive therapy (Grade 2B). Manual lymphatic drainage (MLD) is a massage-like technique that is typically performed by specially trained physical therapists, but a self-help maneuver (simple lymphatic drainage) has also been used for mild cases. Light pressure is used to mobilize edema fluid from distal to proximal areas.

For patients with moderate-to-severe lymphedema (ISL stage II to III) and no contraindications, we suggest intensive physiotherapy, usually in the form of complete decongestive therapy (CDT), rather than less intense therapy (Grade 2B). CDT refers to a two-phase (treatment phase, maintenance phase) multicomponent technique that is designed to reduce the degree of lymphedema and to maintain the health of the skin and supporting structures.

Patients with severe lymphedema (ISL stage III) may also benefit from intermittent pneumatic compression (IPC; also called sequential pneumatic compression) in addition to complete decongestive therapy. IPC devices use a plastic stocking that is intermittently inflated over the affected limb. Most pneumatic compression pumps sequentially inflate a series of chambers in a distal-to-proximal direction.

Surgical intervention is effective in the treatment of lower extremity lymphedema in those with appropriate indications. Available surgical treatments have few contraindications, low risks, and good outcomes. Improvements after surgery include reduced limb volume, reduced rates of infection/cellulitis, improved physical function, ability to return to everyday activities, and improved quality of life. (See 'Indications' above and 'Techniques and effectiveness' above.)

Physiologic procedures (lymphaticovenous anastomosis, lymph node transfer) improve lymphatic drainage by surgically creating alternate pathways for fluid egress.

Reductive techniques (direct excision, liposuction) remove excess tissue to reduce the size and weight of an affected limb. This can also improve range of motion and restore patient mobility.

Surgical intervention often involves a combined (physiologic and reductive surgery) or staged approach.

  1. Anatomy of the Human Body, 20th ed, Gray H (Ed), Lea & Febiger, Philadelphia 1918.
  2. Cesmebasi A, Baker A, Du Plessis M, et al. The surgical anatomy of the inguinal lymphatics. Am Surg 2015; 81:365.
  3. Rockson SG. Anatomy and structural physiology of the lymphatic system. In: Principles and Practice of Lymphedema Surgery, Cheng MH, Chang DW, Patel KM (Eds), Elsevier, Edinburgh 2016. p.25.
  4. Chapter 1: Anatomy of the Vulva. Acta Radiologica 1961; 55:16.
  5. Greene AK. Epidemiology and morbidity of lymphedema. In: Lymphedema Presentation, Diagnosis, and Treatment, Greene AK, Slavin SA, Brorson H (Eds), Springer, Cham 2015. p.33.
  6. Kerchner K, Fleischer A, Yosipovitch G. Lower extremity lymphedema update: pathophysiology, diagnosis, and treatment guidelines. J Am Acad Dermatol 2008; 59:324.
  7. Jones GE, Mansour S. An approach to familial lymphoedema. Clin Med (Lond) 2017; 17:552.
  8. Dean SM, Starr J. An unusual case of familial lymphedema. Ann Vasc Surg 2014; 28:1314.e1.
  9. Choi I, Lee S, Hong YK. The new era of the lymphatic system: no longer secondary to the blood vascular system. Cold Spring Harb Perspect Med 2012; 2:a006445.
  10. Nitti M, Hespe GE, Cuzzone D, et al. Definition, incidence and pathophysiology of lymphedema. In: Principles and Practice of Lymphedema Surgery, Cheng MH, Chang DW, Patel KM (Eds), Elsevier, Edinburgh 2016. p.40.
  11. Cormier JN, Askew RL, Mungovan KS, et al. Lymphedema beyond breast cancer: a systematic review and meta-analysis of cancer-related secondary lymphedema. Cancer 2010; 116:5138.
  12. Kuroda K, Yamamoto Y, Yanagisawa M, et al. Risk factors and a prediction model for lower limb lymphedema following lymphadenectomy in gynecologic cancer: a hospital-based retrospective cohort study. BMC Womens Health 2017; 17:50.
  13. Bakar Y, Tuğral A. Lower Extremity Lymphedema Management after Gynecologic Cancer Surgery: A Review of Current Management Strategies. Ann Vasc Surg 2017; 44:442.
  14. Hayes SC, Janda M, Ward LC, et al. Lymphedema following gynecological cancer: Results from a prospective, longitudinal cohort study on prevalence, incidence and risk factors. Gynecol Oncol 2017; 146:623.
  15. Colicchia M, Sharma V, Abdollah F, et al. Therapeutic Value of Standard Versus Extended Pelvic Lymph Node Dissection During Radical Prostatectomy for High-Risk Prostate Cancer. Curr Urol Rep 2017; 18:51.
  16. Keegan KA, Cookson MS. Complications of pelvic lymph node dissection for prostate cancer. Curr Urol Rep 2011; 12:203.
  17. Hyngstrom JR, Chiang YJ, Cromwell KD, et al. Prospective assessment of lymphedema incidence and lymphedema-associated symptoms following lymph node surgery for melanoma. Melanoma Res 2013; 23:290.
  18. Dean SM, Kaffenberger BH, Lustberg ME. Kaposi sarcoma: An unconventional cause of lower extremity lymphedema. Vasc Med 2017; 22:544.
  19. Allen PJ, Gillespie DL, Redfield RR, Gomez ER. Lower extremity lymphedema caused by acquired immune deficiency syndrome-related Kaposi's sarcoma: case report and review of the literature. J Vasc Surg 1995; 22:178.
  20. Santos M, Vilasboas V, Mendes L, et al. Lymphangiectatic Kaposi's sarcoma in a patient with AIDS. An Bras Dermatol 2013; 88:276.
  21. Babu S, Nutman TB. Immunopathogenesis of lymphatic filarial disease. Semin Immunopathol 2012; 34:847.
  22. U.S. Department of Health & Human Services. Parasites - Lymphatic Filariasis: Epidemiology & Risk Factors. https://www.cdc.gov/parasites/lymphaticfilariasis/epi.html (Accessed on April 02, 2019).
  23. Farrow W. Phlebolymphedema-a common underdiagnosed and undertreated problem in the wound care clinic. J Am Col Certif Wound Spec 2010; 2:14.
  24. Endicott K, Laredo J, Lee BB. Diagnosis and management of primary phlebolymphedema. In: Lymphedema, Lee BB, Rockson SG, Bergan J (Eds), Springer International Publishing, Cham 2018. p.913.
  25. Mehrara BJ, Greene AK. Lymphedema and obesity: is there a link? Plast Reconstr Surg 2014; 134:154e.
  26. Greene AK. Obesity-induced lymphedema. In: Lymphedema Presentation, Diagnosis, and Treatment, Greene AK, Slavin SA, Brorson H (Eds), Springer, Cham 2015. p.97.
  27. Savetsky IL, Torrisi JS, Cuzzone DA, et al. Obesity increases inflammation and impairs lymphatic function in a mouse model of lymphedema. Am J Physiol Heart Circ Physiol 2014; 307:H165.
  28. Chopra K, Tadisina KK, Brewer M, et al. Massive localized lymphedema revisited: a quickly rising complication of the obesity epidemic. Ann Plast Surg 2015; 74:126.
  29. Evans RJ, Scilley C. Massive localized lymphedema: A case series and literature review. Can J Plast Surg 2011; 19:e30.
  30. Beesley V, Janda M, Eakin E, et al. Lymphedema after gynecological cancer treatment : prevalence, correlates, and supportive care needs. Cancer 2007; 109:2607.
  31. Pereira de Godoy AC, Ocampos Troitino R, de Fátima Guerreiro Godoy M, Pereira de Godoy JM. Lymph Drainage of Posttraumatic Edema of Lower Limbs. Case Rep Orthop 2018; 2018:7236372.
  32. Szczesny G, Olszewski WL, Deszczyński J. [Post-traumatic lymphatic and venous drainage changes in persistent edema of lower extremities]. Chir Narzadow Ruchu Ortop Pol 2000; 65:315.
  33. Lessiani G, Iodice P, Nicolucci E, Gentili M. Lymphatic edema of the lower limbs after orthopedic surgery: results of a randomized, open-label clinical trial with a new extended-release preparation. J Biol Regul Homeost Agents 2015; 29:805.
  34. Kayıran O, De La Cruz C, Tane K, Soran A. Lymphedema: From diagnosis to treatment. Turk J Surg 2017; 33:51.
  35. Executive Committee. The Diagnosis and Treatment of Peripheral Lymphedema: 2016 Consensus Document of the International Society of Lymphology. Lymphology 2016; 49:170.
  36. Dean SM. Images in vascular medicine. 'Ski-jump' toenails--a phenotypic manifestation of primary lymphedema. Vasc Med 2015; 20:268.
  37. Tidhar D, Armer JM, Bernas M, et al. Clinical evaluation of lymphedema. In: Principles and Practice of Lymphedema Surgery, Cheng MH, Chang DW, Patel KM (Eds), Elsevier, Edinburgh 2016. p.51.
  38. Weiss J, Daniel T. VALIDATION OF THE LYMPHEDEMA LIFE IMPACT SCALE (LLIS): A CONDITION-SPECIFIC MEASUREMENT TOOL FOR PERSONS WITH LYMPHEDEMA. Lymphology 2015; 48:128.
  39. NLN Medical Advisory Committee. The diagnosis and the treatment of lymphedema. Position statement of the national lymphedema network. http://www.lymphnet.org/pdfDocs/nlntreatment.pdf (Accessed on April 01, 2019).
  40. Executive Committee of the International Society of Lymphology. The diagnosis and treatment of peripheral lymphedema: 2020 Consensus Document of the International Society of Lymphology. Lymphology 2020; 53:3.
  41. O'Donnell TF Jr, Rasmussen JC, Sevick-Muraca EM. New diagnostic modalities in the evaluation of lymphedema. J Vasc Surg Venous Lymphat Disord 2017; 5:261.
  42. Hadjis NS, Carr DH, Banks L, Pflug JJ. The role of CT in the diagnosis of primary lymphedema of the lower limb. AJR Am J Roentgenol 1985; 144:361.
  43. Case TC, Witte CL, Witte MH, et al. Magnetic resonance imaging in human lymphedema: comparison with lymphangioscintigraphy. Magn Reson Imaging 1992; 10:549.
  44. Tiwari A, Cheng KS, Button M, et al. Differential diagnosis, investigation, and current treatment of lower limb lymphedema. Arch Surg 2003; 138:152.
  45. Liao SF, Li SH, Huang HY. The efficacy of complex decongestive physiotherapy (CDP) and predictive factors of response to CDP in lower limb lymphedema (LLL) after pelvic cancer treatment. Gynecol Oncol 2012; 125:712.
  46. Yasuhara H, Shigematsu H, Muto T. A study of the advantages of elastic stockings for leg lymphedema. Int Angiol 1996; 15:272.
  47. Tiwari P, Coriddi M, Salani R, Povoski SP. Breast and gynecologic cancer-related extremity lymphedema: a review of diagnostic modalities and management options. World J Surg Oncol 2013; 11:237.
  48. Chang DW, Masia J, Garza R 3rd, et al. Lymphedema: Surgical and Medical Therapy. Plast Reconstr Surg 2016; 138:209S.
  49. Kim SJ, Park YD. Effects of complex decongestive physiotherapy on the oedema and the quality of life of lower unilateral lymphoedema following treatment for gynecological cancer. Eur J Cancer Care (Engl) 2008; 17:463.
  50. Ko DS, Lerner R, Klose G, Cosimi AB. Effective treatment of lymphedema of the extremities. Arch Surg 1998; 133:452.
  51. Szuba A, Cooke JP, Yousuf S, Rockson SG. Decongestive lymphatic therapy for patients with cancer-related or primary lymphedema. Am J Med 2000; 109:296.
  52. Yamamoto R, Yamamoto T. Effectiveness of the treatment-phase of two-phase complex decongestive physiotherapy for the treatment of extremity lymphedema. Int J Clin Oncol 2007; 12:463.
  53. Cormier JN, Rourke L, Crosby M, et al. The surgical treatment of lymphedema: a systematic review of the contemporary literature (2004-2010). Ann Surg Oncol 2012; 19:642.
  54. Carl HM, Walia G, Bello R, et al. Systematic Review of the Surgical Treatment of Extremity Lymphedema. J Reconstr Microsurg 2017; 33:412.
  55. Mitsumori LM, McDonald ES, Wilson GJ, et al. MR lymphangiography: How i do it. J Magn Reson Imaging 2015; 42:1465.
  56. Masià J, Pons G, Rodríguez-Bauzà E. Barcelona Lymphedema Algorithm for Surgical Treatment in Breast Cancer-Related Lymphedema. J Reconstr Microsurg 2016; 32:329.
  57. Kung TA, Champaneria MC, Maki JH, Neligan PC. Current Concepts in the Surgical Management of Lymphedema. Plast Reconstr Surg 2017; 139:1003e.
  58. Mihara M, Hara H, Zhou HP, et al. Lymphaticovenous Anastomosis Releases the Lower Extremity Lymphedema-associated Pain. Plast Reconstr Surg Glob Open 2017; 5:e1205.
  59. Suami H, Chang D, Skoracki R, et AL. Using indocyanine green fluorescent lymphography to demonstrate lymphatic architecture. J Lymphoedema 2012; 7:25.
  60. Constantinescu T, Huang JJ, Chang MH. Tracking outcomes following lymphedema treatment. In: Principles and Practice of Lymphedema Surgery, Cheng MH, Chang DW, Patel KM (Eds), Elsevier, Edinburgh 2016. p.203.
  61. Suami H. Controversies in the treatment of lymphedema. In: Principles and Practice of Lymphedema Surgery, Cheng MH, Chang DW, Patel KM (Eds), Elsevier, Edinburgh 2016. p.17.
  62. Mitsumori LM, McDonald ES, Neligan PC, Maki JH. Peripheral Magnetic Resonance Lymphangiography: Techniques and Applications. Tech Vasc Interv Radiol 2016; 19:262.
  63. Carter J, Huang HQ, Armer J, et al. GOG 244 - The Lymphedema and Gynecologic cancer (LeG) study: The impact of lower-extremity lymphedema on quality of life, psychological adjustment, physical disability, and function. Gynecol Oncol 2021; 160:244.
  64. Schiltz D, Kiermeier N, Müller K, et al. Quality of life evaluation and lack of correlation with volumetric results after lymphovenous anastomoses in lymphedema therapy of the lower extremity. J Vasc Surg Venous Lymphat Disord 2022; 10:436.
  65. Garza R 3rd, Skoracki R, Hock K, Povoski SP. A comprehensive overview on the surgical management of secondary lymphedema of the upper and lower extremities related to prior oncologic therapies. BMC Cancer 2017; 17:468.
  66. Lee GK, Perrault DP, Bouz A, et AL. Surgical treatment modalities for lymphedema. J Aesthet Reconstr Surg 2016; 2:13.
  67. Gallagher K, Marulanda K, Gray S. Surgical Intervention for Lymphedema. Surg Oncol Clin N Am 2018; 27:195.
  68. Gennaro P, Gabriele G, Salini C, et al. Our supramicrosurgical experience of lymphaticovenular anastomosis in lymphoedema patients to prevent cellulitis. Eur Rev Med Pharmacol Sci 2017; 21:674.
  69. Chang DW, Suami H, Skoracki R. A prospective analysis of 100 consecutive lymphovenous bypass cases for treatment of extremity lymphedema. Plast Reconstr Surg 2013; 132:1305.
  70. Kadota H, Shimamoto R, Fukushima S, et al. Lymphaticovenular anastomosis for lymph vessel injury in the pelvis and groin. Microsurgery 2021; 41:421.
  71. Phillips GSA, Gore S, Ramsden A, Furniss D. Lymphaticovenular anastomosis in the treatment of secondary lymphoedema of the legs after cancer treatment. J Plast Reconstr Aesthet Surg 2019; 72:1184.
  72. Demirtas Y, Ozturk N, Yapici O, Topalan M. Supermicrosurgical lymphaticovenular anastomosis and lymphaticovenous implantation for treatment of unilateral lower extremity lymphedema. Microsurgery 2009; 29:609.
  73. Demirtas Y, Ozturk N, Yapici O, Topalan M. Comparison of primary and secondary lower-extremity lymphedema treated with supermicrosurgical lymphaticovenous anastomosis and lymphaticovenous implantation. J Reconstr Microsurg 2010; 26:137.
  74. Raju A, Chang DW. Vascularized lymph node transfer for treatment of lymphedema: a comprehensive literature review. Ann Surg 2015; 261:1013.
  75. Teng E, Chang DW. Overview of surgical techniques. In: Principles and Practice of Lymphedema Surgery, Cheng MH, Chang DW, Patel KM (Eds), Elsevier, Edinburgh 2016. p.87.
  76. Grünherz L, Hulla H, Uyulmaz S, et al. Patient-reported outcomes following lymph reconstructive surgery in lower limb lymphedema: A systematic review of literature. J Vasc Surg Venous Lymphat Disord 2021; 9:811.
  77. Scaglioni MF, Arvanitakis M, Chen YC, et al. Comprehensive review of vascularized lymph node transfers for lymphedema: Outcomes and complications. Microsurgery 2018; 38:222.
  78. Dayan JH, Smith ML. Microsurgical procedures: Minimizing donor site morbidity following vascularized lymph node transfer. In: Principles and Practice of Lymphedema Surgery, Cheng MH, Chang DW, Patel KM (Eds), Elsevier, Edinburgh 2016. p.167.
  79. Batista BN, Germain M, Faria JC, Becker C. Lymph node flap transfer for patients with secondary lower limb lymphedema. Microsurgery 2017; 37:29.
  80. Patel KM, Lin CY, Cheng MH. A Prospective Evaluation of Lymphedema-Specific Quality-of-Life Outcomes Following Vascularized Lymph Node Transfer. Ann Surg Oncol 2015; 22:2424.
  81. Ozturk CN, Ozturk C, Glasgow M, et al. Free vascularized lymph node transfer for treatment of lymphedema: A systematic evidence based review. J Plast Reconstr Aesthet Surg 2016; 69:1234.
  82. Akita S, Mitsukawa N, Kuriyama M, et al. Comparison of vascularized supraclavicular lymph node transfer and lymphaticovenular anastomosis for advanced stage lower extremity lymphedema. Ann Plast Surg 2015; 74:573.
  83. Becker C, Vasile JV, Levine JL, et al. Microlymphatic surgery for the treatment of iatrogenic lymphedema. Clin Plast Surg 2012; 39:385.
  84. Gustafsson J, Chu SY, Chan WH, Cheng MH. Correlation between Quantity of Transferred Lymph Nodes and Outcome in Vascularized Submental Lymph Node Flap Transfer for Lower Limb Lymphedema. Plast Reconstr Surg 2018; 142:1056.
  85. Weiswasser J, Ashinoff RL, Schneider LF. Vascular considerations in rehabilitative surgery. In: Rehabilitative Surgery: A Comprehensive Text for an Emerging Field, Elkwood AI, Kaufman M, Schneider LF (Eds), Springer, Cham, Switzerland 2017. p.345.
  86. Ciudad P, Manrique OJ, Bustos SS, et al. Comparisons in long-term clinical outcomes among patients with upper or lower extremity lymphedema treated with diverse vascularized lymph node transfer. Microsurgery 2020; 40:130.
  87. Karri V, Yang MC, Lee IJ, et al. Optimizing outcome of charles procedure for chronic lower extremity lymphoedema. Ann Plast Surg 2011; 66:393.
  88. van der Walt JC, Perks TJ, Zeeman BJ, et al. Modified Charles procedure using negative pressure dressings for primary lymphedema: a functional assessment. Ann Plast Surg 2009; 62:669.
  89. Miller TA, Wyatt LE, Rudkin GH. Staged skin and subcutaneous excision for lymphedema: a favorable report of long-term results. Plast Reconstr Surg 1998; 102:1486.
  90. Jabbar F, Hammoudeh ZS, Bachusz R, et al. The diagnostic and surgical challenges of massive localized lymphedema. Am J Surg 2015; 209:584.
  91. Porrino J, Walsh J. Massive localized lymphedema of the thigh mimicking liposarcoma. Radiol Case Rep 2016; 11:391.
  92. Stewart CJ, Munnoch DA. Liposuction as an effective treatment for lower extremity lymphoedema: A single surgeon's experience over nine years. J Plast Reconstr Aesthet Surg 2018; 71:239.
  93. Boyages J, Kastanias K, Koelmeyer LA, et al. Liposuction for Advanced Lymphedema: A Multidisciplinary Approach for Complete Reduction of Arm and Leg Swelling. Ann Surg Oncol 2015; 22 Suppl 3:S1263.
  94. Brorson H. Liposuction in Lymphedema Treatment. J Reconstr Microsurg 2016; 32:56.
  95. Brorson H, Ohlin K, Olsson G, et al. Controlled compression and liposuction treatment for lower extremity lymphedema. Lymphology 2008; 41:52.
  96. Lamprou DA, Voesten HG, Damstra RJ, Wikkeling OR. Circumferential suction-assisted lipectomy in the treatment of primary and secondary end-stage lymphoedema of the leg. Br J Surg 2017; 104:84.
  97. Micha JP, Nguyen DH, Goldstein BH. Successful management of persistent lower extremity lymphedema with suction-assisted lipectomy. Gynecol Oncol Rep 2018; 23:13.
  98. Espinosa-de-Los-Monteros A, Hinojosa CA, Abarca L, Iglesias M. Compression therapy and liposuction of lower legs for bilateral hereditary primary lymphedema praecox. J Vasc Surg 2009; 49:222.
  99. Eryilmaz T, Kaya B, Ozmen S, Kandal S. Suction-assisted lipectomy for treatment of lower-extremity lymphedema. Aesthetic Plast Surg 2009; 33:671.
  100. Greene AK, Slavin SA, Borud L. Treatment of lower extremity lymphedema with suction-assisted lipectomy. Plast Reconstr Surg 2006; 118:118e.
  101. Cheng MH, Chang DW, Patel KM. Future perspectives in lymphatic microsurgery. In: Principles and Practice of Lymphedema Surgery, Cheng MH, Chang DW, Patel KM (Eds), Elsevier, Edinburgh 2016. p.216.
  102. Ramachandran S, Chew KY, Tan BK, Kuo YR. Current operative management and therapeutic algorithm of lymphedema in the lower extremities. Asian J Surg 2021; 44:46.
  103. Campisi CC, Ryan M, Boccardo F, Campisi C. Fibro-Lipo-Lymph-Aspiration With a Lymph Vessel Sparing Procedure to Treat Advanced Lymphedema After Multiple Lymphatic-Venous Anastomoses: The Complete Treatment Protocol. Ann Plast Surg 2017; 78:184.
  104. Basta MN, Gao LL, Wu LC. Operative treatment of peripheral lymphedema: a systematic meta-analysis of the efficacy and safety of lymphovenous microsurgery and tissue transplantation. Plast Reconstr Surg 2014; 133:905.
  105. Yamashita S, Chang DW, Koshima I. Microsurgical procedures lymphovenous anastomosis techniques. In: Principles and Practice of Lymphedema Surgery, Cheng MH, Chang DW, Patel KM (Eds), Elsevier, Edinburgh 2016. p.173.
  106. Brorson H. Excisional procedures liposuction. In: Principles and Practice of Lymphedema Surgery, Cheng MH, Chang DW, Patel KM (Eds), Elsevier, Edinburgh 2016. p.107.
Topic 17013 Version 6.0

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