Surgical treatment of primary and secondary lymphedema

INTRODUCTION — 

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.

Conservative multimodal therapy is the mainstay of management, with surgical treatment of primary and secondary lymphedema offered to selected patients. Surgical treatment can potentially benefit patients with any clinical stage of lymphedema. It can reduce limb girth, alleviate many symptoms, and improve mobility and quality of life.

Surgical treatments for lymphedema are categorized as physiologic or reductive techniques. Physiologic techniques repair or create alternative pathways for lymph to flow out of the affected limb. Reductive or "ablative" techniques surgically remove edematous and fibrotic soft tissues from a lymphedematous limb.

The indications, preoperative evaluation, and techniques for surgical treatment of primary and secondary lymphedema are reviewed. The etiology, clinical features, and nonsurgical treatment of lymphedema are reviewed separately. (See "Pathophysiology, classification, and causes of lymphedema" and "Clinical features, diagnosis, and staging of peripheral lymphedema" and "Management of peripheral lymphedema".)

OPERATIVE MANAGEMENT — 

There is no consensus regarding the role of surgery, the optimal surgical approach, or the timing of surgery in the treatment of primary or secondary lymphedema [1,2]. The quality of evidence evaluating outcomes following surgical treatment of lymphedema is overall low, which has inspired several ongoing randomized trials to provide more definitive recommendations [3-5]. The decision for surgery to treat lymphedema should be made on a case-by-case basis. The goals of surgical management are to alleviate pain and discomfort, retain or restore function, reduce the risk of infection, prevent disease progression, improve cosmesis, and limit deformity [6-8]. Earlier intervention prior to tissue fibrosis and severe adipose deposition is more effective for treating lower extremity lymphedema.

Reasons for referral — Operative management of primary and secondary lymphedema is typically reserved for patients under specific circumstances when conservative treatment cannot or has not achieved the desired goals. Conservative treatment (ie, complete decongestive therapy) is discussed separately. (See "Management of peripheral lymphedema", section on 'Approach to management'.)

General reasons that patients are referred for operative management of primary and secondary lymphedema include:

●Localized primary lesions (including microcystic and macroscopic lymphatic malformations) or massive localized lymphedema

●Lack of improvement (plateau) or progression of lymphedema despite adequate conservative treatment

●Recurrent cellulitis

●Leakage of lymph into body cavities, organs, or externally

●Limitation of function (eg, reduced mobility, contracture)

●Deformity or disfigurement

●Pain or other severe symptoms such as heaviness or tightness

●Diminished quality of life, including emotional and psychosocial distress

Indications for specific techniques are discussed below. (See 'Surgical techniques' below.)

Surgical options — Surgical treatments for lymphedema can be categorized as physiologic or reductive (or ablative) techniques. In some cases, physiologic procedures can be combined with reductive procedures to maximize results. Proper patient selection and careful surgical planning optimize potential physical benefits, improvements in quality of life, and reduce the risk of potential complications.

Physiologic techniques — Physiologic techniques repair normal or create alternative pathways to increase the capacity of the lymphatic system to transport lymph fluid out of the affected limb. These are the only potential options to halt, reverse, or minimize the degree of lymphedema progression. With these techniques, the fluid trapped in lymphedematous tissues drains into other lymphatic basins or into the venous circulation.

Physiologic procedures used to accomplish this goal include lymphatic bypass procedures (ie, lymphovenous bypass [LVB] or vascularized lymph node transplant [LNT]) and flap transposition procedures. Lymphatic bypass procedures are more commonly used.

Physiologic procedures may be used to treat patients with any of the following:

●Failure or plateau of nonoperative management

●Recurrent cellulitis or lymphangitis

●Desire to reduce the amount of daily time wearing compression garments

●Significantly impaired quality of life

Physiologic procedures should not be used for patients with medical comorbidities that preclude a safe surgical procedure or in patients who will not be compliant with postoperative lymphedema treatment including compression therapy. (See "Management of peripheral lymphedema".)

Physiologic procedures are used most often for patients with early-stage lymphedema (ie, International Society of Lymphology [ISL] stage I or II) (table 1) prior to the deposition of excess fat and extensive tissue fibrosis [2,9-11]. Patients with more advanced lymphedema have been treated with physiologic techniques; however, results have varied. Overall, patients with ISL stage I or II lymphedema do well with LVB while patients with stage III and IV lymphedema may do better with LNT.

A combination of LVB and LNT may provide better outcomes compared with either procedure alone, but this approach is more likely to be applied to patients with earlier-stage lymphedema because patients with ISL stage III or IV lymphedema often have no patent lymphatic vessels, and thus, would not be candidates for LVB. (See 'Combined procedures' below.)

Reductive techniques — Reductive techniques (also called ablative techniques) debulk the affected limb by removing fibrofatty tissue generated from sustained lymphatic fluid stasis. Reductive techniques include direct excision, liposuction, or a combination of these. Liposuction removes tissue using small cannulas inserted into the subcutaneous space. Direct excision often involves radical resection of the excess tissues. (See 'Direct excision' below and 'Liposuction' below.)

While these techniques do not improve lymphatic function, they can reduce the weight and circumference of lymphedematous limbs, resulting in improved function. Reductive techniques can be curative in patients with localized primary lymphatic malformations (picture 1) but are palliative, not curative, for patients with secondary lymphedema. Nevertheless, reductive techniques can be very helpful as an adjunct and can be performed before or after a physiologic procedure to further improve surgical outcomes [12,13]. (See 'Combined procedures' below.)

Reductive techniques are best used for patients who have failed conservative measures or for patients who present with advanced lymphedema with fat deposition and tissue fibrosis (ie, ISL stage II or III (table 1)). Patients who are not candidates for, or who do not wish to undergo a physiologic procedure, may also benefit.

Preoperative evaluation — A thorough preoperative assessment is essential to determine the best surgical treatment. It is important to note that surgical treatment does not eliminate the need for ongoing nonsurgical therapy to maintain long-term benefits. Patients must be educated and willing to comply with the necessary preoperative and postoperative treatment protocols.

Lymphedema assessment — The surgeon should confirm the etiology and severity of lymphedema based on clinical evaluation (picture 2 and table 1) and limb volume measurements, or L-Dex scores from bioimpedance spectroscopy, as well as the effects of lymphedema on physical functioning and well-being using validated quality of life surveys. (See "Clinical features, diagnosis, and staging of peripheral lymphedema", section on 'Diagnosis'.)

In addition, imaging studies are necessary to evaluate the lymphatics, and to evaluate venous anatomy and function.

Lymphatic imaging — When planning microsurgical intervention, it is important to obtain or review the results of any previous (within a year) lymphatic imaging studies. Physiologic derangement of lymphatic drainage in the affected limb can be proven using indocyanine green (ICG) or magnetic resonance (MR) lymphangiography [14,15]. These also help locate functional lymphatic channels for potential anastomosis [16-18]. Of these, ICG is more commonly obtained. The availability of MR lymphangiography may be limited, and MR can be uncomfortable, and expensive.

ICG lymphangiography — ICG imaging can be used preoperatively to evaluate lymphatic flow and identify abnormalities. ICG is injected into the skin and imaged with a dynamic infrared fluorescence camera [17-19].

The severity of lymphedema can be graded depending on the severity of dermal backflow and diffusion pattern of ICG. In people with functioning lymphatics, a linear pattern will appear. Splash, stardust, and diffuse patterns indicate an increasing severity of lymphedema and increased levels of fibrosis in the lymphatic channels [20]. 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 (image 1). In those with functioning lymphatics, ICG imaging provides direct information to indicate the location of surgery. ICG patterns are similar for the upper and lower extremities.

One limitation of ICG lymphangiography is that only lymphatics within 2 cm of the skin's surface can be seen. To overcome this limitation, MR lymphangiography can also be performed, providing the surgeon with additional information on the deep lymphatic system to aid surgical planning [17]. (See 'MR lymphangiography' below.)

When considering a lymph node transplant, in addition to evaluating the affected extremity, 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 transplant procedure, the risk of developing donor-site lymphedema can be greatly reduced [17-19].

MR lymphangiography — MR lymphangiography is a modification of 3D volumetric contrast-enhanced MR imaging developed to depict the severity of lymphedema, the number and location of individual lymphatic channels, and the presence of dermal backflow.

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 intracutaneous injection of gadolinium-based MR contrast to obtain an MR venogram. The MR venogram is used to differentiate lymphatics from veins [16,21].

An advantage of MR lymphangiography is its ability to depict individual subdermal lymphatic channels, which is useful for selecting patients for physiologic procedures. Also, compared with lymphoscintigraphy, it does not involve ionizing radiation and the examination is quicker.

Vascular imaging studies — For patients who will undergo a physiologic procedure, venous imaging (eg, duplex ultrasound, MR venography) should be obtained to identify venous insufficiency, or venous outflow obstruction (ie, thrombosis, stenosis), which requires treatment prior to consideration for a physiologic procedure [22,23]. For venous thrombosis, lymphatic surgery is postponed until after the course of treatment is completed. For patients with venous stenosis, surgery can release scarring around the vein, or an endovascular procedure used to open the vein (eg, venous angioplasty). (See "Endovenous intervention for thoracic central venous obstruction" and "Endovenous intervention for iliocaval venous obstruction" and "Primary (spontaneous) upper extremity deep vein thrombosis".)

SURGICAL TECHNIQUES

Lymphatic bypass procedures — Primary or secondary forms of lymphedema can be treated using lymphatic bypass, although the effectiveness of these procedures in patients with primary lymphedema has been debated [24-26]. The basic principle is that lymphatic vessels distal to the lymphatic obstruction are anastomosed to a nearby vein such that lymphatic fluid is shunted to the venous system and bypasses the area of lymphatic obstruction. Intraoperatively, before making an incision, isosulfan blue dye or other dyes such as fluorescein or indocyanine green (ICG) can be injected into the subdermal tissues distal to the operative site to help identify the lymphatic vessels [27].

Candidates for lymphatic bypass — Lymphatic bypass procedures are used in the following settings:

●Nonoperative management that is not achieving the desired goals

●Recurrent cellulitis or lymphangitis

●Dissatisfaction with compression garments

●Significantly impaired quality of life

Relative contraindications to lymphatic bypass procedures include:

●Extensive tissue fibrosis

●Late-stage lymphedema changes

●Venous hypertension

●Recurrent cancer in the ipsilateral extremity or metastatic disease

●Anticipated noncompliance with postoperative care protocols

●Body mass index >35

●Active smoking

Methods — There is no consensus on the type of lymphatic bypass procedure used; decisions are made based on surgeon preference and experience. Physiologic procedures have been shown to be effective for reducing limb volume and size. In a systematic review that evaluated patients undergoing lymphovenous bypass (LVB), lymph node transplant (LNT), or a combination, the overall pooled change in excess circumference (1002 patients) was -35.6 percent [28]. The overall pooled change in excess volume (587 patients) was -32.7 percent. In an earlier systematic review, 91.2 percent of patients overall reported a subjective improvement [29]. At follow-up, 65 percent of patients discontinued compression garments. The absolute circumference reduction in extremities was as follows:

●All – 3.31 cm (95% CI 2.58-4.04; 11 studies, 138 patients)

●Upper extremity – 2.73 cm (95% CI 1.63-3.83; six studies, 79 patients)

●Lower extremity – 3.52 cm (95% CI 2.28-4.75; five studies, 52 patients)

Lymphovenous bypass — LVB (also known as lymphovenous anastomosis) restores adequate lymphatic drainage by bypassing diseased lymphatics and providing direct drainage into the venous system. The patient must have partially functional lymphatic vessels to create an effective anastomosis.

This microsurgical technique anastomoses distal subdermal lymphatic vessels to adjacent venules less than 0.8 mm in diameter [25,27,30]. The number and location of anastomoses in each patient varies based on the presence of functional and accessible vessels [31,32]. The distal subdermal lymphatics are less affected by lymphedema and are more readily available for a bypass procedure than deeper lymphatic channels. The pressure in the subdermal venules is also lower than in the deeper, larger veins, and there is less venous backflow in the subdermal vessels, which, at least in theory, should result in a permanent improvement of lymphedema.

LVB is most effective for patients with International Society of Lymphology (ISL) stages I to IIa (table 1), when the lymphatic vessels are still able to transfer lymph fluid [33]. Lymphatic imaging should be performed to determine their status. The use of both preoperative and intraoperative lymphatic mapping is necessary to achieve superior results following LVB [33]. LVB may be a treatment option for patients who exhibit a linear, splash, or stardust ICG pattern according to Cheng's Grading System [34]. (See 'Preoperative evaluation' above.)

While there are no guidelines for the recommended number of LVBs, a greater number of anastomoses could reduce interstitial fluid levels [35]. Patients without active functional lymphatic channels are not candidates for LVB. Another contraindication to LVB is noncorrectable venous hypertension.

Extremity lymphedema treated with LVB — Patient satisfaction following LVB for lower extremity lymphedema has been reported to be high. Most of the outcome data for physiologic techniques are from retrospective reviews [28,29]. Some prospective studies have also been reported [10,36,37].

●Effectiveness – The response to LVB is variable, ranging from a complete response to none. Improvements may include a decrease in limb size and weight, softer and improved skin texture, better-fitting clothing and improved appearance, and overall improved quality of life. The variability of outcomes among studies is likely from several factors including lack of standardization of changes in extremity measurements. Few studies report the use of complementary techniques (eg, volume measurements and bioimpedance or lymphoscintigraphy) to corroborate postoperative measurements. Studies also have differing lengths of follow-up, variable use of postoperative compression garments and/or physical therapy, and the use of nonstandardized or nonvalidated questionnaires for subjective analysis. Other caveats include mixed series of patients, either based upon etiology (eg, primary congenital conditions, or secondary lymphedema following nodal resections, trauma, or filariasis); location of lymphedema (eg, upper or lower extremity); and/or variable criteria for patient selection, selection of procedures, the timing of intervention, and identification of suitable lymphatic vessels for bypass surgery.

Despite these limitations, systematic reviews report improvements in limb volumes, with some individuals experiencing marked reductions [2,28,29,38]. LVB also reduces pain symptoms. In one study, in addition to a reduction in limb size, the average visual analog scale pain scores decreased from 5.3 preoperatively to 1.8 postoperatively [14].

LVB can effectively treat lymphedema in the upper or lower extremity. In a systematic review, the excess percent reduction in circumference was 48.9 percent (95% CI 40.7-57.2; seven studies, 80 patients) [29]. In a prospective study of 100 consecutive patients undergoing LVB, 96 percent of patients with upper extremity lymphedema had symptomatic improvement (lighter, softer, less painful arms) and 74 percent had a quantitative reduction in excess volume [39]. The mean volume reduction after LVB decreased with increasing lymphedema stage. Other studies have also reported this correlation.

•In one review, reported limb volume reductions were: 78.5 percent for stage II patients, 54.8 percent for stage III patients, and 47.4 percent for stage IV patients [40].

•In another study in which LVB was performed in 89 upper extremities and 11 lower extremities, the mean volume differential reductions were significantly better for stage I or II lymphedema compared with stage III or IV lymphedema (61 versus 17 percent at six months) [39].

Progression of lymphedema postoperatively is uncommon. In long-term studies (more than three years of follow-up), improvements with bypass procedures were maintained even when compression garments were discontinued [23,25,30].

●Reduced cellulitis frequency – In addition to improvements in lymphedema symptoms, LVB can result in a significant reduction in the frequency of infections, specifically cellulitis [9,23,25,28,36,40-42].

•In a systematic review, the overall pooled change in the number of cutaneous infection episodes per year was -1.9 (95% CI -1.4 to -2.3) [28].

•In a large retrospective review that included approximately 1800 patients undergoing a microsurgical LVB procedure, the incidence of cellulitis was decreased by 87 percent following LVB [23].

•A later trial randomly assigned patients with secondary lower extremity lymphedema who underwent at least three months of nonoperative decongestive therapy to LVB and complete decongestive therapy or complete decongestive therapy alone. The primary outcome of cellulitis frequency over six months was significantly reduced in the LVB group (-0.57 versus -0.21; 95% CI -0.35, 95% CI -0.62 to -0.09) [36]. Thigh area hardness was also reduced. However, limb circumference and pain were similar for both groups, suggesting no additional benefit of LVB in these metrics.

●Patient satisfaction – Patient satisfaction following LVB for lower extremity lymphedema has been associated with improved quality of life [24,43-45]. In a study evaluating the correlation of limb volume reduction following LVB and quality of life, volume reductions for unilateral lymphedema and bilateral lymphedema corresponded with a 23 and 14 percent improvement, respectively, in patient-reported quality of life using the Lymphedema Quality of Life Questionnaire (LYMQOL) [45].

Primary lymphedema and other conditions — When LVB was first described, it was suggested that patients with primary lymphedema were not candidates for this procedure. Studies have since shown that LVB can effectively treat patients with primary lymphedema. In most patients with primary lower extremity lymphedema, surgeons have been able to find healthy lymphatics suitable for anastomosis. Reduction in lower extremity limb volume after LVB is similar for secondary and primary lymphedema [26].

Several case reports and small series suggest that lymphatic bypass procedures may also be effective in reducing primary and secondary genital lymphedema [42,46,47]. Prospective studies are needed to evaluate the efficacy of lymphatic bypass procedures in this setting.

LVB 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 some series. In one small retrospective review, lymphatic leakage ceased after a mean of six days after pelvis or groin LVB [48]. For lower extremity lymphedema, LVB also decreased pain scores. In a small study, the average visual analog scale scores decreased from 5.3 preoperatively to 1.8 postoperatively [14]. The average change in limb circumference in their sample was -4.7 percent.

Complications of LVB — The rate of complications following lymphatic bypass procedures is low around 6 percent [9,25-27,32,49]. Most complications are minor, such as delayed wound healing and lymphatic fistula, and typically improve spontaneously. Other complications include infection, lymphatic fistula, partial skin ulceration, and wound dehiscence. In a systematic review, surgical site infection occurred in 4.7 percent, lymph leak in 7.7 percent, re-exploration was needed for flap congestion in 2.7 percent, and other procedures were performed in 22.6 percent [29].

Vascularized lymph node transplant — Vascularized LNT (also called vascularized lymph node transfer) is a physiologic technique that involves a free transfer of healthy lymph nodes en bloc from one nodal basin to the site of obstruction [41,50,51]. The recipient site can be the site of the prior lymph node excision or a nonanatomic site. The nodes are transplanted using microsurgical techniques with arterial and venous anastomosis at the recipient site; there is usually no lymphatic anastomosis with this approach.

Indications for LNT include any of the following: repeated episodes of cellulitis (but not acute cellulitis), inadequate improvement with complete decongestive therapy, rapidly progressive lymphedema, and significantly impaired quality of life [52,53]. Proper patient selection and careful surgical planning decrease the risk of potential complications and optimize potential benefits.

The mechanisms regulating lymphatic repair and improving lymphatic flow following LNT remain largely unknown [41,54]. Proposed mechanisms include the presence of pre-existing lymphovenous communication within the transplanted lymph nodes and via efferent lymphatic vessels from the lymph nodes. Some authors have also suggested that a "lymphatic pump" is created when lymph nodes are transplanted nonanatomically.

Potential donor sites for lymph nodes include the groin, axilla, omentum, intestinal mesentery, supraclavicular, and submental regions [55-57]. 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 [20,58].

Extremity lymphedema treated with LNT — Systematic reviews support a beneficial role of LNT with decreased limb volume and improved quality of life for most carefully selected patients [2,20,28,29,38,59]. In one of these, the excess percent circumference reduction was 48.5 percent (95% CI 35.3-61.6; four studies, 51 patients) [29].

Prospective studies and retrospective reviews have reported outcomes following LNT alone or in combination with LVB [10,11,37,60,61].

●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. Similar to reports for LVB, reported total volume reductions are greater in patients with lower preoperative excess volumes [60].

●A review of 134 patients who underwent LNT or LNT and simultaneous LVB for primary or secondary lymphedema reported that 90.2 percent had decreased limb volume, 97.1 percent had decreased L-Dex scores, and 96.2 percent had improved quality-of-life scores using a validated lymphedema questionnaire [10]. When analyzed one and two years postoperatively, this study reported a reduction of 34.7 and 45.7 percent in limb volume, respectively. Similar improvements were noted for L-Dex measurements and quality-of-life outcomes.

●A review of 274 patients who underwent LNT with or without LVB or LVB alone reported similar outcomes [37]. In this study, nearly 90 percent of patients with upper extremity lymphedema and 60 percent of patients with lower extremity lymphedema had decreased limb volume after surgery. At one and two years after surgery, upper limb volume decreased by 25.7 and 47.4 percent, respectively. Approximately 86 percent of patients with upper extremity lymphedema also had improvements in quality-of-life outcomes as measured by a validated questionnaire. Outcomes for lower extremity lymphedema were more modest with limb volume reduction decreases of 14.5 and 34.8 percent at one and two years.

As with LVB, another notable improvement following LNT is a decrease in the frequency of infections, a major health and financial burden associated with lymphedema. The annual incidence of infection episodes has been reported to significantly decrease following LNT for both upper and lower extremity lymphedema [11,59,61]. In a study of 35 patients undergoing LNT for lower extremity lymphedema due to treatment of gynecological cancer, all patients had significant reductions in limb circumference as well as a reduction in the incidence of cellulitis [62]. Similarly, another study reported an 85 percent reduction in the rates of recurrent infections in 89 patients treated with vascularized lymph node transplantation [11]. Health-related quality of life scores may 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.

Complications of LNT — A potentially serious complication of LNT is the development of lymphedema associated with the donor site [41,51,54,63]. The use of mesenteric lymph nodes (eg, omentum, intestinal mesentery lymph nodes) obviates this problem since harvesting these lymph nodes does not result in lymphedema. However, this benefit must be balanced with potential intra-abdominal complications (eg, hernia, small bowel obstruction, pancreatic injury). The risk of developing donor-site lymphedema has been minimized with the development of reverse lymphatic mapping. This technique injects ICG fluorescence lymphography and/or a radioactive tracer into the limb adjacent to the donor site, allowing a surgeon to identify which lymph nodes drain the extremity at the donor site and to avoid harvesting these nodes and select an alternative donor site. In a review of 60 LNTs performed in a four-year period using mapping techniques, significant improvement occurred in more than 50 percent of their patients, with no cases of donor site lymphedema [63].

The risk of other complications following an LNT is low but may include loss of flap, donor site lymphedema, seroma, lymphocele, infection, and wound healing complications [20].

Direct excision — Direct excision has successfully treated many patients with lymphedema and may be the only, and possibly best, treatment option for patients with severe lymphedema who have impaired physical function, recurrent infections, skin ulcerations, pain, and poor quality of life [52,63-65].

These procedures are particularly useful in areas where liposuction is not possible (eg, scrotal lymphedema, large medial thigh masses). However, rates of effectiveness vary, morbidity can be significant, and lesions may recur.

A variety of direct excision procedures have been described for the treatment of extremity and genital lymphedema [32,33]. Direct excision was first described in 1912 as the "Charles procedure," which involved circumferential excision that often worsened lymphedema and is rarely used today [64,65].

Localized excision of focal areas of severe lymphedema is commonly performed. Direct excision involves excision of the affected tissue above the deep fascia with preservation of the overlying dermis or with removal of the dermis. The resulting defects are covered either with tissue flaps or with skin grafts. (See "Overview of flaps for soft tissue reconstruction".)

Multiple modifications have been made to this procedure to improve outcomes. Examples include the use of negative-pressure dressing after 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 [52,64,66].

Candidates for direct excision — Direct excision is generally reserved for patients with very severe disease who have not responded to other treatments. Indications for direct excision include:

●Advanced-stage lymphedema that does not improve with conservative treatment (most commonly primary lymphedema)

●Recurrent episodes of cellulitis

●Severe disfigurement or dysfunction

●Large pedunculated masses with relatively narrow bases of attachment

Direct excision is particularly useful for patients with massive, localized lymphedema. These masses can weigh up to 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 [67-69].

Outcomes of excision — The effectiveness of direct excision varies with the location and extensiveness of the involved tissues.

The largest single-institution observational study further illustrates the effectiveness of excision and reconstruction to reduce the bulk of lymphedematous tissue in patients with primary lymphedema [70]. Quality of life was assessed using the SF-36 questionnaire.

●In the retrospective component of the study, 78 patients underwent 174 extremity procedures, and 49 (63 percent) noted a decrease in limb size. Overall, quality of life improved for 23 patients, and physical activity improved for 26. Thirteen patients (12 male and 1 female) underwent 32 excision and reconstructive procedures of the genitalia with overall mixed results. Quality of life was improved in eight patients, sexual function improved in five, physical activity improved in seven. All patients had a reduction in the size of the genitalia.

●The prospective component included 10 patients with extremity lymphedema; nine reported a decrease in limb size. Quality of life improved in seven patients and physical activity improved for four. Eight patients experienced a reduction in the size of the genitalia. Quality of life was improved for seven patients, physical activity was improved for six, and sexual function was improved for four.

Quality of life improvement was significantly higher for patients undergoing genitalia reduction compared with those undergoing surgery for lymphedematous extremities (71 versus 34 percent). The perception that the operative procedure was worthwhile for the reduction of lymphedematous tissue of the extremities and/or genitalia was significantly higher in the patients evaluated prospectively compared with patients evaluated retrospectively (83 versus 56 percent). The difference in perception may be due to poor recall or a reduced quality of life with time.

In a retrospective review of 38 patients with lower extremity lymphedema (primary or secondary) treated by skin and subcutaneous excision, the decrease in lymphedema and improved function and contour were long-lasting in 30 patients [64]. The average length of follow-up was 14 years (range 3 to 17 years). Five patients had progression of lymphedema following the procedure, and the sixth patient had progression of lymphedema associated with weight gain.

The recurrence rate after macroscopically resecting a localized lesion ranges from 15 to 40 percent [71]. Recurrence is hypothesized to result from regrowth and re-expansion of microscopic, unresected lymphatic channels. Cutaneous vesicles may occur within the surgical scar and can be managed with additional surgical resections and/or sclerotherapy.

Complications of excision — While effective, direct excision results in substantial morbidity. Complications include wound dehiscence, graft loss, pain, infection, scarring, and lymphatic fistula.

●In the extremities other complications include flap loss, which may require additional surgery and contour irregularities or other suboptimal cosmetic results, poor functional results, and distal (eg, foot) edema. Severe wound healing complications may, in some cases, worsen the extremity necessitating amputation [72,73].

●In the genitalia, complications include hemorrhage, urethral injury, infection, painful erection, sexual dysfunction, decreased sensation, and scarring in the suture line [74,75].

Liposuction — Liposuction is a well-established surgical technique with low complication rates. Lymphedema liposuction involves the removal of fat and fibrosis using a suction technique [32,35]. Liposuction is contraindicated in patients requiring systemic anticoagulation.

While liposuction does not improve lymphatic function, it can provide immediate and complete reduction of excess volume in affected limbs. The procedure is relatively simple to perform and has a low rate of complications (eg, minor infection, paresthesias). Liposuction successfully removes the accumulation of adipose tissue that typically presents during late-stage lymphedema [32,35,76]. Even when signs of fibrosis are present, power-assisted liposuction can facilitate the breakdown of fibrotic tissue [62]. Compliance with lifelong compression therapy can prevent recurrence [32,77,78].

Cadaver and imaging studies suggest that liposuction does not disrupt lymphatic vessels (if performed parallel to the limb), and therefore, treating lymphedema with liposuction does not further impair the limb's already reduced lymphatic transport capacity [79-84].

Patients with primary or secondary nonpitting lower extremity lymphedema may be candidates for liposuction. Liposuction alone provides only temporary and palliative relief of symptoms for mild cases of lymphedema [85].

Candidates for liposuction — Candidates for liposuction include [80,86]:

●Stage II or III, nonpitting lymphedema that has failed conservative management (more than three months of treatment)

●Localized fat deposits

●Symptomatic complaints of heaviness, strain, or pain

●Functional impairment

●Recurrent infection

Contraindications to liposuction include:

●Insufficient or inadequate conservative management or noncompliance

●Lymphangiosarcoma of the affected extremity

●Open wounds in the lymphedematous extremity

●Active smoking

Prior to surgery, treatment with compression garments and massage must be implemented to control lymphatic fluid until minimal. Before proceeding with surgery, the patient must express their understanding and motivation to comply with lifelong postoperative compression therapy [20,32,77,87].

In general, circumferential liposuction for lymphedema is safe with few reported postoperative complications. When complications do occur, they tend to be minor and include occasional paresthesias and minor issues with wound healing. The use of tourniquets and tumescent technique significantly decreases blood loss, and patients rarely require transfusions.

Upper extremity liposuction — Lymphedema of the upper arm following breast cancer surgery is associated with a 73 percent increase in adipose tissue by volumetrics; thus, liposuction is an alternative procedure for treating upper extremity lymphedema [76,78]. When treating upper extremity lymphedema, liposuction should be used in conjunction with compression garments, and in conjunction with physiologic procedures [88]. Patients treated with liposuction can have substantial (>90 percent) long-term decrease in limb volume and improved quality of life provided compression garments are worn at all times [78-81,87-92].

The following prospective studies illustrate the results of liposuction for patients with upper arm lymphedema following axillary dissection for breast cancer:

●A review of 37 patients with unilateral nonpitting stage II lymphedema treated with conservative management for two to four days followed by circumferential suction-assisted liposuction and continued use of compression therapy found the mean reduction in upper arm volumes was 118 percent 12 months after the procedure [90].

●A prospective case-matched study (n = 28) found that patients treated with liposuction with continued use of controlled compression therapy had significantly decreased lymphedema compared with patients undergoing controlled compression therapy alone after one year (mean difference = 1000 mL) [89].

●Liposuction combined with physiologic techniques (myocutaneous flap transfer and lymph fascia grafting) in 11 patients with severe lymphedema of the upper extremity resulted in an approximate 10 percent decrease in lymphedema after three years [88]. The mean number of episodes of cellulitis decreased after the surgical procedure compared with preoperative episodes (0.7 versus 6.5 episodes per year).

Lower extremity liposuction — While liposuction has also been reported in a more limited number of patients, the technique also appears to be effective for the treatment of lower extremity lymphedema. Evidence for the efficacy of liposuction for lower extremity lymphedema has been less convincing compared with outcomes for the upper extremity and comprised primarily of small series and case reports. Combined with compression therapy, liposuction achieves long-lasting reductions in lower extremity limb volume [77-79,82,83,85,87,93-98]. Maximal reduction in limb volume can usually be achieved around six months after surgery.

In one large series of patients with lower extremity lymphedema treated with liposuction, the average reduction in excess limb volume was 79 percent two years after surgery [97]. In another review of 69 patients (72 legs), excess reduction in leg volume over a five-year period was as high as 90 percent [77]. Other studies have also reported improved cosmetic and functional results with advanced liposuction devices [94,95,99,100].

In addition to improvements in limb volume, liposuction may improve 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 [77,78,97]. In a prospective study of 55 patients, average function scores improved from 7.4 preoperatively to 28 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]) [78].

Patients with a history of cellulitis also have a significant reduction in the number of annual episodes following lower extremity liposuction [77,97].

Combined procedures — For some patients, it may be appropriate to combine techniques, which may be a more effective surgical approach for treating severe lymphedema. Procedures that combine two physiologic techniques or combine a physiologic and a reductive technique may offer additional benefits. While reductive techniques such as liposuction can remove fatty and fibrotic tissue in the affected limb, lymph node bypass (LVB) or lymph node transfer (LNT) improves lymphatic function.

●Combined physiologic procedures – Combined physiologic procedures using LVB 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 result in greater improvements in quality of life and functional recovery and are associated with decreased morbidity [20,101].

●Combined physiologic and reductive procedures – Physiologic techniques may also be combined with reductive techniques to maximize limb volume reduction. Liposuction is a common adjunctive treatment option due to its low complication rate. It has been suggested that by combining microsurgery with liposuction, the need for continual compression therapy may be alleviated [19,32,78]. Liposuction can be used as a secondary procedure following a primary physiologic surgery. Studies have shown that as many as 16 percent of LVB patients and 31.6 percent of LNT patients benefit from an additional liposuction procedure [32]. An evaluation of combined liposuction and LVB reduced excess lower limb volume from 20.2 to 2.7 with no evidence of lymphatic damage [102,103].

POSTOPERATIVE MANAGEMENT — 

Patients are admitted for one to five days after surgery for postoperative pain management and wound care [23,27,104]. The limbs are elevated for 24 hours for more limited procedures and up to one week for more extensive procedures [23,27,79].

Antibiotics are typically used for one week postoperatively; however, a more prolonged duration of prophylactic antibiotics may be used (eg, four weeks or longer) [23,26,104]. The skin is managed with water-based, gentle moisturizing creams to prevent skin drying or cracking.

To maximize the reduction in limb volume after surgery and prevent lymphedema recurrence following surgery, patients must continue conservative lymphedema therapy. The combined treatment approach of surgical and nonsurgical management has proven effective in the treatment of both primary and secondary lymphedema.

Compression garments are worn postoperatively to prevent the recurrence of lymphedema following physiologic and reductive procedures [79]. There is no consensus on the start time, length of time to continue wearing garments, or even the type of garment [23,26,27,30]. Typically, however, compression garments are started one week after surgery and used for 6 to 12 months. Most patients continue to wear garments indefinitely, although in some patients undergoing physiologic procedures, these may eventually be able to be discontinued with close follow-up [23]. Following reductive techniques, using compression garments is a lifelong requirement to prevent recurrence [29,33,105]. Compression garments are adjusted as necessary to maintain a tight fit. The garment fit is reassessed every four to six months to ensure that adequate compression is maintained.

●Physiologic procedures

•Lymphovenous bypass – Following lymphovenous bypass (LVB), the recommended timing to begin compression garments postoperatively varies based on a surgeon's preference. Garment use 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 following surgery [29,106]. However, if there is significant improvement, the use of compression garments may be discontinued over time. About 56.3 percent of patients are able to discontinue compression garment use after an LVB procedure [29].

•Lymph node transplant – Following proximal lymph node transplant (LNT), compression garments may be worn immediately. With distal transfer, immediate compression over the microsurgical anastomosis is contraindicated. Patients are often recommended to continue use for at least two to six months after surgery [59]. As many as 78 percent of patients are reported to be able to discontinue compression therapy following an LNT [29].

●Reductive procedures

•Following reductive procedures, ongoing compression therapy (24 hours/day) is essential for maintaining or further reducing limb volume and preventing recurrence. Fluid reaccumulation can recur rapidly without postoperative compression therapy [89]. 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 correspondence to changes in limb volume [77,107].

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 5^th to 6^th 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 10^th to 12^th 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: Lymphedema (The Basics)")

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

SUMMARY AND RECOMMENDATIONS

●Lymphedema – Management of primary and secondary lymphedema is primarily nonsurgical. However, advances in surgical techniques have shown significant promise for many patients with this disease. The decision to perform an operative procedure to treat lymphedema should be made on a case-by-case basis. (See 'Introduction' above.)

●Lymphedema surgery – Surgery can effectively treat lymphedema in selected patients. Available surgical treatments have few contraindications, low risks, and good outcomes have been reported. Surgical options include physiologic techniques (lymphovenous bypass [LVB], lymph node transplant [LNT]), reductive techniques (liposuction, direct excision), or a combination of both. (See 'Surgical options' above.)

●Preoperative evaluation – The preoperative evaluation should include confirmation of the etiology and severity of lymphedema. Either indocyanine green (ICG) imaging or MR lymphangiography can be used to assess lymphatic flow and the location of lymphatic injury to help select the best treatment. Physiologic techniques require the presence of functional lymphatic channels for potential anastomosis. In select cases, venous duplex ultrasound or MR imaging/MR venography is obtained to assess for valvular competence and venous obstruction (eg, thrombosis, stenosis). (See 'Preoperative evaluation' above.)

●Physiologic techniques – Physiologic procedures (ie, LVB, LNT) create new channels that increase the capacity of the lymphatic system to transport lymph fluid. The basic principle is that the lymphatic vessels distal to the lymphatic obstruction are anastomosed to healthy lymphatic vessels or veins proximal to the obstruction. Physiologic procedures are used for patients with lymphedema prior to the development of extensive tissue fibrosis (eg, International Society of Lymphology [ISL] stage I, II, or early stage III) (table 1). (See 'Physiologic techniques' above.)

•Candidates – Candidates for lymphatic bypass or vascularized LNT are those with:

-Nonoperative management not achieving the desired goals

-Recurrent cellulitis or lymphangitis

-Desire to reduce the amount of daily time wearing compression garments

-Significantly impaired quality of life

•Contraindications – Relative contraindications to lymphatic bypass or vascularized LNT include:

-Extensive tissue fibrosis

-Late-stage lymphedema changes

-Recurrent cancer in the ipsilateral extremity or metastatic disease

-Anticipated noncompliance with postoperative care protocols

-Body mass index >35

-Active smoking

●Reductive techniques – Reductive techniques (also called ablative techniques; eg, direct excision, liposuction) remove excess tissue that has been generated from sustained lymphatic fluid stasis to reduce the size and weight of an affected limb. These procedures can also improve the range of motion and restore patient mobility. We use reductive techniques for patients with more advanced lymphedema after fat deposition and tissue fibrosis have occurred (eg, late-stage ISL II or ISL III). (See 'Reductive techniques' above.)

●Combined techniques – Combined techniques can provide additional benefits for selected patients. This can be a combination of two physiologic techniques or a physiologic and a reductive technique. This approach is more likely to be applied to patients with earlier-stage lymphedema because patients with ISL stage III or IV lymphedema often have no patent lymphatic vessels, and thus, would not be candidates for a physiologic procedure. Combined physiologic procedures may provide better outcomes compared with either procedure alone because the two approaches work via different mechanisms. A physiologic technique combined with liposuction also reduces excess limb volume and may reduce the need for continued compression therapy. (See 'Combined procedures' above.)

●Outcomes – Most carefully selected patients who undergo surgery experience reduced limb volume, reduced rates of infection/cellulitis, improved physical functioning, and improved quality of life. Outcomes in the upper extremity tend to be better than those of the lower extremity. Randomized trials are underway to further clarify the role of lymphatic surgery and the selection of patients for the treatment of lymphedema.