Differential diagnosis, investigation, and current treatment of lower limb lymphedema
TL;DR: The common differential diagnosis in Western patients with lower limb swelling is secondary lymphedema, venous disease, lipedema, and adverse reaction to ipsilateral limb surgery.
Abstract: Hypothesis The causes and management of lower limb lymphedema in the Western population are different from those in the developing world. Objective To look at the differential diagnosis, methods of investigation, and available treatments for lower limb lymphedema in the West. Data Source A PubMed search was conducted for the years 1980-2002 with the keyword "lymphedema." English language and human subject abstracts only were analyzed, and only those articles dealing with lower limb lymphedema were further reviewed. Other articles were extracted from cross-referencing. Results Four hundred twenty-five review articles pertaining to lymphedema were initially examined. This review summarizes the findings of relevant articles along with our own practice regarding the management of lymphedema. Conclusions The common differential diagnosis in Western patients with lower limb swelling is secondary lymphedema, venous disease, lipedema, and adverse reaction to ipsilateral limb surgery. Lymphedema can be confirmed by a lymphoscintigram, computed tomography, magnetic resonance imaging, or ultrasound. The lymphatic anatomy is demonstrated with lymphoscintigraphy, which is particularly indicated if surgical intervention is being considered. The treatment of choice for lymphedema is multidisciplinary. In the first instance, combined physical therapy should be commenced (complete decongestive therapy), with surgery reserved for a small number of cases.
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TL;DR: The splash pattern is the earliest finding on indocyanine green lymphography of asymptomatic limbs of secondary lower extremity lymphedema patients, indicating that the leg dermal backflow stage allows early diagnosis of secondaryLower Extremity lyMPhedema even in a subclinical stage.
Abstract: BACKGROUND Early diagnosis and treatment are as important for management of secondary lymphedema following cancer treatment as in primary cancer treatment. Indocyanine green lymphography is the modality of choice for routine follow-up evaluation of patients at high risk of developing lymphedema after cancer therapy. METHODS Fifty-six limbs of 28 so-called unilateral secondary lower extremity lymphedema patients who underwent indocyanine green lymphography were compared with dermal backflow patterns of indocyanine green lymphography on 28 asymptomatic limbs and assessed using leg dermal backflow stage. RESULTS Of 28 asymptomatic limbs of secondary lower extremity lymphedema patients, the dermal backflow patterns were detected in 19 limbs but were absent in nine limbs. Significant differences were seen between asymptomatic limbs with dermal backflow patterns (n=19) and limbs without them (n=9): age, 51.4±15.3 years versus 34.8±12.7 years (p=0.007); body weight, 75.1±7.9 kg versus 50.1±5.3 kg (p=0.012); body mass index, 23.1±4.2 versus 19.7±1.8 (p=0.005); leg dermal backflow stage of asymptomatic limb, 1.2±0.4 versus 0.0±0.0 (p<0.001); and leg dermal backflow stage of symptomatic limb, 3.5±0.6 versus 2.8±0.8 (p=0.033). CONCLUSIONS The splash pattern is the earliest finding on indocyanine green lymphography of asymptomatic limbs of secondary lower extremity lymphedema patients. The leg dermal backflow stage allows early diagnosis of secondary lower extremity lymphedema even in a subclinical stage. The concept of subclinical lymphedema could play an important role in early diagnosis and prevention of lymphedema after cancer treatment. CLINICAL QUESTION/LEVEL OF EVIDENCE Diagnostic, V.
245 citations
TL;DR: This progressive chronic disease has serious implications on patients' quality of life and is often misdiagnosed because it mimics other conditions of extremity swelling.
Abstract: Lymphedema is a localized form of tissue swelling resulting from excessive retention of lymphatic fluid in the interstitial compartment and caused by impaired lymphatic drainage. Lymphedema is classified as primary or secondary. Primary lymphedema is caused by developmental lymphatic vascular anomalies. Secondary lymphedema is acquired and arises as a result of an underlying systemic disease, trauma, or surgery. We performed PubMed and Google Scholar searches of the English-language literature (1966-2017) using the terms lymphedema, cancer-related lymphedema, and lymphatic complications. Relevant publications were manually reviewed for additional resources. This progressive chronic disease has serious implications on patients' quality of life. It is often misdiagnosed because it mimics other conditions of extremity swelling. There is no definitive cure for lymphedema. However, with proper diagnosis and management, its progression and potential complications may be limited.
221 citations
TL;DR: Lower extremity lymphedema is an important medical issue which causes morbidity and is frequently seen by dermatologists, and treatment is based on rerouting the lymph fluid through remaining functional lymph vessels.
Abstract: Lower extremity lymphedema is an important medical issue which causes morbidity and is frequently seen by dermatologists. The subject has not been adequately addressed in dermatologic literature for many years. Primary lymphedema is caused by an inherent malfunction of the lymph-carrying channel, in which no direct outside cause can be found. Secondary lymphedema is caused by an outside force, such as tumors, scar tissue after radiation, or removal of lymph nodes, which results in dysfunction of the lymph-carrying channels. Treatment is based on rerouting the lymph fluid through remaining functional lymph vessels. This is accomplished through elevation, exercises, compression garments/devices, manual lymph drainage, and treatment is combined with good skin care practices.
186 citations
Cites background from "Differential diagnosis, investigati..."
...The Kaposi-Stemmer sign is a clinical sign indicative of lymphedema, in which an examiner is unable to pinch a fold of skin at the base of the second toe on the dorsal aspect of the foot (Fig 4).(2,4,5) Ultimately, the skin is at risk for breakdown and subsequent infection....
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...Causes of lymphedema.(4,5) J AM ACAD DERMATOL 2 Kerchner, Fleischer, and Yosipovitch ARTICLE IN PRESS...
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...Lymphedema occurs when there is (1) an inherent defect within the lymph-carrying conduits, termed primary lymphedema, or (2) whenever acquired damage arises, termed secondary lymphedema (eg, pressure from tumors, scar tissue after radiation, surgical removal of lymph nodes) (Fig 1).(1,3,5) The primary causes can be further categorized according to the age at which they first had clinical manifestations: congenital causes (age \1 year), lymphedema praecox (age 1-35), and lymphedema tarda (age [35 years)....
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...radiotherapy, and surgery is reserved for those with isolated disease.(2,5,10-13)...
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...If there is significant edema, a postoperative tibial or popliteal vein thrombosis should be considered.(5,8) DIAGNOSIS Whenever there is doubt regarding the clinical diagnosis, diagnostic confirmation can be accomplished with isotopic lymphoscintigraphy (considered the method of choice) or, if necessary, with radiocontrast lymphangiography....
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TL;DR: The goal was to write a brief, focused review that would answer questions about the management of leg edema and organized the information to make it rapidly accessible to busy clinicians.
Abstract: A common challenge for primary care physicians is to determine the cause and find an effective treatment for leg edema of unclear etiology. We were unable to find existing practice guidelines that address this problem in a comprehensive manner. This article provides clinically oriented recommendations for the management of leg edema in adults. We searched on-line resources, textbooks, and MEDLINE (using the MeSH term, "edema") to find clinically relevant articles on leg edema. We then expanded the search by reviewing articles cited in the initial sources. Our goal was to write a brief, focused review that would answer questions about the management of leg edema. We organized the information to make it rapidly accessible to busy clinicians. The most common cause of leg edema in older adults is venous insufficiency. The most common cause in women between menarche and menopause is idiopathic edema, formerly known as "cyclic" edema. A common but under-recognized cause of edema is pulmonary hypertension, which is often associated with sleep apnea. Venous insufficiency is treated with leg elevation, compressive stockings, and sometimes diuretics. The initial treatment of idiopathic edema is spironolactone. Patients who have findings consistent with sleep apnea, such as daytime somnolence, loud [corrected] snoring, or neck circumference >17 inches, should be evaluated for pulmonary hypertension with an echocardiogram. If time is limited, the physician must decide whether the evaluation can be delayed until a later appointment (eg, an asymptomatic patient with chronic bilateral edema) or must be completed at the current visit (eg, a patient with dyspnea or a patient with acute edema [<72 hours]). If the evaluation should be conducted at the current visit, the algorithm shown in Figure 1 could be used as a guide. If the full evaluation could wait for a subsequent visit, the patient should be examined briefly to rule out an obvious systemic cause and basic laboratory tests should be ordered for later review (complete blood count, urinalysis, electrolytes, creatinine, blood sugar, thyroid stimulating hormone, and albumin).
164 citations
Cites background from "Differential diagnosis, investigati..."
...skin on the dorsum of the foot at the base of the second toe is a sign of lymphedema.(15,22)...
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...However, the distinction cannot always be made because chronic venous insufficiency can lead to secondary lymphedema with abnormally delayed lymph drainage on lymphoscintigram.(15,34)...
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TL;DR: ICG fluorescence lymphography has the potential to become an alternative lymphatic imaging technique to assess lymph function and was compared to dynamic lymphoscintigraphy in subjects without leg oedema.
157 citations
References
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710 citations
TL;DR: The diagnostic features, the pathophysiology and the available therapies for lymphedema are presented, which include isotopic lymphoscintigraphy, indirect and direct lymphography, magnetic resonance imaging, computed tomography and ultrasonography.
Abstract: This review presents the diagnostic features, the pathophysiology and the available therapies for lymphedema. This disease is often able to be diagnosed by its characteristic clinic- cal presentation, yet, in some cases, ancillary tests might be necessary to establish the diag- nosis, particularly in the early stages of the disease and in edemas of mixed etiology. These diagnostic modalities are also useful in clinical studies. Available modalities include isotopic lymphoscintigraphy, indirect and direct lymphography, magnetic resonance imaging, computed tomography and ultrasonography. Lymphedema may be primary or secondary to the presence of other diseases and/or to the consequences of sur- gery. Primary lymphedema may occur at any phase of life but it most commonly appears at puberty. Secondary lymphedema is encountered more often. The most prevalent worldwide cause of lymphedema is filariasis, which is particularly common in south-east Asia. In the USA, postsurgical lymphedema of the extremity prevails. Complications of chronic limb lymphedema include recurrent cellulitis and lymphangiosarcoma. Most patients are treated conservatively, by means of various forms of compression therapy, including complex physical therapy, pneumatic pumps and compressive garments. Volume reducing surgery is performed rarely. Lymphatic microsurgery is still in an experimental stage, although a few centers consistently report favorable outcomes.
381 citations
"Differential diagnosis, investigati..." refers background in this paper
...It is most common in the lower limb (80% of cases) but can also occur in the arms, face, trunk, and external genitalia.(1) Lymphedema is an important differential diagnosis in lower limb swelling, with various investigation and treatment options available....
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TL;DR: These patients typically report a significant recovery from their previous cosmetic and functional impairments, and also from the psychosocial limitations they experienced from a physical stigma they felt was often trivialized by the medical and payor communities.
Abstract: Objective To define the immediate and long-term volumetric reduction following complete decongestive physiotherapy (CDP) for lymphedema. Design Prospective study of consecutively treated patients. Setting Freestanding outpatient referral centers. Patients Two hundred ninety-nine patients referred for evaluation of lymphedema of the upper (2% primary, 98% secondary) or lower (61.3% primary, 38.7% secondary) extremities were treated with CDP for an average duration of 15.7 days. Lymphedema reduction was measured following completion of treatment and at 6- and 12-month follow-up visits. Intervention Complete decongestive physiotherapy is a 2-phase noninvasive therapeutic regimen. The first phase consists of manual lymphatic massage, multilayered inelastic compression bandaging, remedial exercises, and meticulous skin care. Phase 2 focuses on self-care by means of daytime elastic sleeve or stocking compression, nocturnal wrapping, and continued exercises. Main Outcome Measures Average limb volumes in milliliters were calculated prior to treatment, at the end of phase 1, and at 6- to 12-month intervals during phase 2 to assess percent volume reduction. Results Lymphedema reduction averaged 59.1% after upper-extremity CDP and 67.7% after lower-extremity treatment. With an average follow-up of 9 months, this improvement was maintained in compliant patients (86%) at 90% of the initial reduction for upper extremities and lower extremities. Noncompliant patients lost a part (33%) of their initial reduction. The incidence of infections decreased from 1.10 infections per patient per year to 0.65 infections per patient per year after a complete course of CDP. Conclusions Complete decongestive physiotherapy is a highly effective treatment for both primary and secondary lymphedema. The initial reductions in volume achieved are maintained in the majority of the treated patients. These patients typically report a significant recovery from their previous cosmetic and functional impairments, and also from the psychosocial limitations they experienced from a physical stigma they felt was often trivialized by the medical and payor communities.
312 citations
TL;DR: This study aimed to compare the effect of multilayer bandaging as an initial phase of lymphedema treatment followed by elastic hosiery versus hOSiery alone.
Abstract: BACKGROUND
Multilayered, low stretch bandages (MLB) combined with exercises, skin care, and manual lymphatic drainage therapy are recommended as an intensive phase of treatment for lymphedema patients. The relative efficacy of each of the components of this comprehensive treatment program have not been determined. This study aimed to compare the effect of multilayer bandaging as an initial phase of lymphedema treatment followed by elastic hosiery versus hosiery alone.
METHODS
A randomized, controlled, parallel-group trial was undertaken in the setting of the Lymphedema Clinic, The Royal Marsden Hospital, London. Ninety women with unilateral lymphedema (of the upper or lower limbs) were enrolled in the study. The interventions consisted of 18 days of multilayer bandaging followed by elastic hosiery or hosiery alone, each for a total period of 24 weeks. The main outcome measure was the percentage reduction in excess limb volume.
RESULTS
The reduction in limb volume by MLB followed by hosiery was approximately double that from hosiery alone and was sustained over the 24-week period. The mean overall percentage reduction at 24 weeks was 31% (n = 32) for MLB versus 15.8% (n = 46) for hosiery alone, for a mean difference of 15.2% (95% confidence interval, 6.2–24.2) (P = 0.001).
CONCLUSIONS
Multilayer bandaging as an initial phase of treatment for lymphedema patients, followed by hosiery, achieves greater and more sustained limb volume reduction than hosiery alone. Cancer 2000;88:2832–7. © 2000 American Cancer Society.
239 citations
"Differential diagnosis, investigati..." refers background or methods in this paper
...Treatment using this technique in 90 female patients with either upper or lower limb lymphedema was significantly more effective than hosiery alone.(71) In lipedema, no difference was made by compressive stockings....
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...Multilayer bandaging is another form of compression and has been shown to be effective in both upper and lower limb lymphedema.(71) This form of compression consists of an inner layer of tubular stockinette followed by foam and padding to protect the joint flexures and to even out the contours of the limb so that the pressure is evenly distributed....
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TL;DR: 5,6-Benzo-[alpha]-pyrone results in slow but safe reduction of lymphedema of the extremities; limb mobility also improved; and side effects disappeared after the first month of therapy.
Abstract: Background Benzopyrones can reduce the volume of high-protein edema fluid by stimulating proteolysis. These compounds provide a method for removing excess protein and its consequent edema and reduce its clinical sequelae, such as chronic inflammation and secondary infections. Methods We conducted a randomized, double-blind, placebo-controlled, crossover trial of 5,6-benzo-[alpha]-pyrone in 31 patients with postmastectomy lymphedema of the arm and 21 patients with lymphedema of the leg of various causes. This agent is also known as 56 BaP, 1,2-benzopyrone, and coumarin, although it has no anticoagulant activity. The patients received 400 mg of the active drug or placebo, each for six months. Results During the placebo period, lymphedema often worsened, especially in the arms. Measurements of limb volume showed that the active drug reduced the mean amount of edema fluid in the arms from 46 percent above normal to 26 percent above normal (P<0.001) and the amount in the legs from 25 percent to 17 percent abov...
236 citations
"Differential diagnosis, investigati..." refers background in this paper
...This group of drugs has also been shown to be effective in the treatment of lymphedema by reducing edema fluid, increasing softness of the limbs, and decreasing elevated skin temperature.(88) More importantly, there were markedly fewer instances of secondary infection, and there was improvement in the symptoms, such as reduction in the bursting pain and feeling of hardness, tightness, heaviness, swelling, and an increase in mobility....
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