Functioning in lymphedema from the patients' perspective using the International Classification of Functioning, Disability and health (ICF) as a reference.

PURPOSE: To identify and quantify meaningful concepts in lymphedema from the patients' perspectives using the International Classification of Functioning, Disability and Health (ICF). METHODS: Six focus group interviews in five different centers were organized, audiotaped, transcribed verbatim and analyzed. RESULTS: A total of 2681 relevant ICF linkings were performed with the focus group data, resulting in 130 different second-level categories. Of these 130 second-level categories, 41 (31.5%) categories were categorized as Body Functions, 20 (15.5%) as Body Structures, 41 (31.5%) as Activities and Participation, and 28 (21.5%) as Environmental Factors. Overall, the most important issues according to the patients were the use of hosiery and bandages, support and relationships, and the shape of structures related to movement. CONCLUSION: Based on their experiences with lymphedema, patients reported activity limitations and participation restrictions combined with impaired body functioning. Anatomical changes (Body Structures) were also often mentioned as a problem in daily life. Environmental factors may act as a barrier or facilitator for patient functioning. The ICF provides a valuable reference to identify concepts in statements from lymphedema patients. The results of this research will be used in the development of ICF Core Sets for lymphedema.

[Soft tissue infections]. / Wekedeleninfecties.

Soft tissue infections are common, but can be difficult to diagnose and manage. In this article, the classification of soft tissue infections is discussed, as well as the diagnostic possibilities and treatment options. Furthermore, the management of recurrent infections and necrotizing soft tissue infections are discussed. The added value of compression therapy is reviewed in more detail.

Reliability, concurrent validity, and clinical feasibility of measurement methods determining volume in patients with lower limb lymphedema and healthy controls.

PURPOSE: To investigate reliability, concurrent validity, and clinical feasibility of measurements assessing volume in patients with lower limb lymphedema (LLL) and healthy controls. MATERIALS AND METHODS: To investigate intra- and interrater reliability, 47 patients with LLL and 30 healthy controls were assessed three times by two assessors. To investigate between session reliability, 50 participants were reassessed two weeks later. Each assessment included measurements of the midline region (hip circumference; suprapubic volume), leg volume (perimeter every 4 cm; Perometer®), and foot volume (water displacement; figure-of-eight method). Concurrent validity was assessed with correlation coefficients. Measurements were timed and practical limitations were reviewed. Clinical trial registration number: NCT: 05269264. RESULTS: Measurements of the total volume of different regions showed weak to very high intraclass correlation coefficients (ICCs) (0.131-998). Absolute and relative volume differences had lower ICC values (0.360-0.976). A strong correlation was found between the total volumes of the same region. The Perometer® and figure-of-eight method were the fastest method for leg and foot volume, respectively. CONCLUSIONS: The assessed total volumes might be more valuable in assessing the evolution of volume in bilateral LLL than the calculated absolute and relative differences between both limbs. The Perometer® and figure-of-eight method were the most time efficient for leg and foot volume, respectively.Implications for rehabilitationLymphedema is a chronic condition for which a reliable and clinically feasible assessment of volume is essential for the diagnosis, treatment decisions, and the evaluation of the treatment.This study shows that the total leg/foot volumes were more reliable than the calculated absolute and relative differences between both limbs and could therefore more valuable to evaluate bilateral lower limb lymphedema.For the assessment of leg volume, the Perometer® was the most reliable and fastest method.For the evaluation of the foot volume, the figure-of-eight method was overall the best method.

The VASCERN PPL working group patient pathway for primary and paediatric lymphoedema.

Lymphoedema is caused by an imbalance between fluid production and transport by the lymphatic system. This imbalance can be either caused by reduced transport capacity of the lymphatic system or too much fluid production and leads to swelling associated with tissue changes (skin thickening, fat deposition). Its main common complication is the increased risk of developing cellulitis/erysipelas in the affected area, which can worsen the lymphatic function and can be the cause of raised morbidity of the patient if not treated correctly/urgently. The term primary lymphoedema covers a group of rare conditions caused by abnormal functioning and/or development of the lymphatic system. It covers a highly heterogeneous group of conditions. An accurate diagnosis of primary lymphoedema is crucial for the implementation of an optimal treatment plan and management, as well as to reduce the risk of worsening. Patient care is diverse across Europe, and national specialised centres and networks are not available everywhere. The European Reference Network on Rare Multisystemic Vascular Diseases (VASCERN) gathers the best expertise in Europe and provide accessible cross-border healthcare to patients with rare vascular diseases. There are six different working groups in VASCERN, which focus on arterial diseases, hereditary haemorrhagic telangiectasia, neurovascular diseases, lymphoedema and vascular anomalies. The working group Paediatric and Primary Lymphedema (PPL WG) gathers and shares knowledge and expertise in the diagnosis and management of adults and children with primary and paediatric lymphoedema. The members of PPL WG have worked together to produce this opinion statement reflecting strategies on how to approach patients with primary and paediatric lymphoedema. The objective of this patient pathway is to improve patient care by reducing the time to diagnosis, define the best management and follow-up strategies and avoid overuse of resources. Therefore, the patient pathway describes the clinical evaluation and investigations that lead to a clinical diagnosis, the genetic testing, differential diagnosis, the management and treatment options and the patient follow up at expert and local centres. Also, the importance of the patient group participation in the PPL WG is discussed.

Dogmas and controversies in compression therapy: report of an International Compression Club (ICC) meeting, Brussels, May 2011.

The International Compression Club (ICC) is a partnership between academics, clinicians and industry focused upon understanding the role of compression in the management of different clinical conditions. The ICC meet regularly and from these meetings have produced a series of eight consensus publications upon topics ranging from evidence-based compression to compression trials for arm lymphoedema. All of the current consensus documents can be accessed on the ICC website (http://www.icc-compressionclub.com/index.php). In May 2011, the ICC met in Brussels during the European Wound Management Association (EWMA) annual conference. With almost 50 members in attendance, the day-long ICC meeting challenged a series of dogmas and myths that exist when considering compression therapies. In preparation for a discussion on beliefs surrounding compression, a forum was established on the ICC website where presenters were able to display a summary of their thoughts upon each dogma to be discussed during the meeting. Members of the ICC could then provide comments on each topic thereby widening the discussion to the entire membership of the ICC rather than simply those who were attending the EWMA conference. This article presents an extended report of the issues that were discussed, with each dogma covered in a separate section. The ICC discussed 12 'dogmas' with areas 1 through 7 dedicated to materials and application techniques used to apply compression with the remaining topics (8 through 12) related to the indications for using compression.

Consensus recommendations on lymphedema in Phelan-McDermid syndrome.

Phelan-McDermid syndrome (PMS) is a neurodevelopmental disorder caused by deletions 22q13.3 or pathogenic variants in the SHANK3 gene. Lymphedema can be a clinical feature in 10-25% of individuals with PMS due to a deletion 22q13.3, but is not observed in those with a SHANK3 variant. This paper forms a part of the European consensus guideline for PMS and focuses on what is known regarding lymphedema in PMS in order to present clinical recommendations. The mechanism causing lymphedema in PMS is unknown. Lymphedema can be suggested by pitting oedema of the extremities or, in later stages, non-pitting swelling. It can occur already at a young age and be progressive if untreated, impacting daily functioning. Lymphedema can be treated using existing general multidisciplinary management guidelines, taking the functioning of the individual with PMS into account. Furthermore, well-known risk factors for the development of lymphedema as lack of physical activities and weight gain/obesity should be addressed. Diagnosis and treatment are best performed in a multidisciplinary centre of expertise.

A care substitution service in the Netherlands: impact on referral, cost, and patient satisfaction.

BACKGROUND: In care substitution services, medical specialists offer brief consultations to provide general practitioners (GPs) with advice on diagnosis, treatment, or hospital referral. When GPs serve as gatekeepers to secondary care, these regional services could reduce pressures on healthcare systems. The aim is to determine the impact of implementing a care substitution service for dermatology, orthopaedics, and cardiology on the hospital referral rate, health care costs, and patient satisfaction. METHODS: A before-after study was used to evaluate hospital referral rates and health care costs during a follow-up period of 1 year. The study population comprised patients with eligible International Classification of Primary Care codes for referral to the care substitution service (only dermatology, orthopaedic, cardiology indications), as pre-defined by GPs and medical specialists. We compared referral rates before and after implementation by χ2 tests and evaluated patient preference by qualitative analysis. RESULTS: In total, 4,930 patients were included, 2,408 before and 2,522 after implementation. The care substitution service decreased hospital referrals during the follow-up period from 15 to 11%. The referral rate decreased most for dermatology (from 15 to 9%), resulting in a cost reduction of €10.59 per patient, while the other two specialisms experienced smaller reductions in referral rates. Patients reported being satisfied, mainly because of the null cost, improved organisation, improved care, and positive experience of the consultation. CONCLUSIONS: The care substitution service showed promise for specialisms that require fewer hospital facilities, as exemplified by dermatology.

[Diagnosis and treatment of primary and secondary lymphedema]. / Diagnostiek en behandeling van primair en secundair lymfoedeem.

Chronic edema is caused by lymphatic impairment due to either overload of interstitial fluid or anatomic/functional lymphatic failure in any part of the body. Lymphatics play a crucial role in interstitial homeostasis, immune surveillance and fat absorption in the gut. Lymphedema is a clinical sign with swelling and not a diagnosis. In the latter stages inflammatory processes lead to fat formation and fibrosis. Untreated lymphedema has an increased risk for erysipelas, physical dysfunction and tends to worsen. Obesity and reduced physical activity are the main influenceable risk factors after oncological treatments in patients with impaired lymphatics. Prior to a multidisciplinary treatment program, a proper diagnosis with health profile is made. Treatment exists of two phases: an initial treatment phase and a maintenance phase in which the focus is on compression, self-management, exercise, minimizing risk factors and creating a healthy lifestyle. Clinimetrics are important for monitoring in both phases.

Improvement of Limb Volumes After Bariatric Surgery in Nine End-Stage Primary, Secondary, and Obesity-Induced Lymphedema Patients: A Multiple Case Report.

Background: Lymphedema (LE) is a chronic condition of swelling due to lymphatic impairment and is characterized by edema and fibro-adipose tissue deposition. LE may be caused by an anomalous development of the lymphatic system, known as primary LE, or may develop secondary to traumatic, infectious, or other external events. Knowledge is increasing about the plural and bidirectional relationship between LE and obesity. The rate of obesity is increasing worldwide, and bariatric surgery offers the most effective and durable treatment, as this surgery exhibits positive effects on many obesity-related diseases. We explored whether bariatric surgery could improve leg volumes in morbidly obese LE patients. Methods and Results: Between 2013 and 2019, 829 patients were hospitalized in our Center of Expertise for Lymphovascular Medicine for intensive treatment of their LE. Nine patients with end-stage primary, secondary, or obesity-induced LE underwent a bariatric procedure related to their morbid obesity. Data concerning age, gender, medical diagnosis, LE stage, type of bariatric treatment, body weight, body mass index (BMI), and limb volumes were retrospectively collected from the patient files. At the individual patient level, body weight, BMI, leg volumes, and their percent reduction between presurgery and postsurgery were calculated. At the group level, paired sample t-tests were conducted to compare the mean body weight, BMI, and volumes of both legs between postsurgery and presurgery. The data demonstrate a significant decrease in body weight, BMI, and leg volumes in morbidly obese end-stage primary, secondary, and obesity-induced LE patients following bariatric surgery. Conclusions: Our multiple case study indicates that bariatric surgery provides a good indication for concomitant treatment of morbid obesity and LE.

Early Referral for Breast-Cancer-Related Lymphedema: Do We Follow the Evidence? A Two-Year Prospective Multicenter Cohort Study.

The early detection of breast-cancer-related lymphedema and referral for therapy has the potential to reduce lymphedema-related morbidity. Although research shows the benefits, a gap is observed between evidence and daily practice. We aimed to determine whether the early detection of lymphedema and referral for treatment is adequate following the current guidelines. Women with primary breast cancer treated with breast-conserving therapy or ablative treatment were included. Demographic-, general health-, tumor-, and treatment-related data were recorded. Bilateral arm volume measurements were performed preoperatively and 3, 6, 12, and 24 months post-surgery. A 5% or greater Relative Volume Change was considered the cutoff point for lymphedema and as an indication for therapy referral. After 24 months post-surgery, the main outcomes show that among the patients with early signs of lymphedema, based on a Relative Volume Change ≥5%, a nonreferral for therapy was noted in 83%. Additionally, we observed a significant improvement of the mean Relative Volume Change at 24 months within this group, which might implicate that nonreferral was an adequate choice and that watchful waiting is appropriate when lymphedema is detected within the first year post-surgery.

The ICC Compression Questionnaire: A Comprehensive Tool to Evaluate Compression Materials or Devices Applied in Subjects with Lymphedema or Chronic Venous Disease.

Background: Compression therapy is an important part of the treatment of patients with lymphedema or chronic venous disease. However, there is no validated questionnaire evaluating the effect of compression and its acceptance by the patient. Therefore, the aims of this study were to construct a questionnaire evaluating the effect of compression and its acceptance by the patient, that is, the Dutch ICC Compression Questionnaire (ICC-CQ), to investigate its reliability and validity, and to translate it into English. Methods and Results: Eleven experts in applying compression and 51 Dutch patients with experience of using compression were involved in the construction process. One part of the ICC-CQ has to be completed by the patient and evaluates seven domains. The other part has to be completed by the health care provider and comprises three domains. Reliability and validity of the final version was investigated in a new group of 79 Dutch-speaking patients with lymphedema or chronic venous disease, wearing compression garments (N = 52) or bandages (N = 27). Except for one domain, the Intraclass Correlation Coefficients for test-rest/interrater reliability ranged from 0.55 to 0.93. Cronbach's alpha for internal consistency ranged from 0.71 to 0.97. Eighty-nine percent of the patients fully understood the questionnaire indicating good face validity, and 87% found it complete indicating good content validity. Construct validity was considered good since 10 out of 11 hypotheses were accepted. Conclusion: The ICC-CQ is the first reliable and valid questionnaire evaluating different kinds of compression and the experience by patients with lymphedema or chronic venous disease.

Paediatric lymphoedema: An audit of patients seen by the paediatric and primary lymphoedema group of vascular European Reference Network (VASCERN).

Little is known about the overall prevalence of lymphoedema in children and the types of paediatric lymphoedema seen by specialist centres. Therefore, this study was aimed to provide a profile of children with primary or secondary lymphoedema seen by the expert centres of the paediatric and primary lymphoedema working group (PPL-WG) of VASCERN and to compare the profile between the different countries. A retrospective review of all children (aged up to 18 years) seen for the first time by the expert centres over one year (2019) was carried out. Lymphoedema-, patient- and genetics-related data was collected and described for the whole group and compared between the different European countries/UK. In 2019, a total of 181 new children were seen by eight expert centres. For primary lymphoedema, the phenotype was based on the St George's classification of lymphatic anomalies. The percentages diagnosed according to each category were: 7.2% for syndromic lymphoedema, 2.8% for systemic/visceral involvement, 30.4% for congenital, 35.9% for late-onset lymphoedema and 19.3% for vascular/lymphatic malformations. 4.4% had secondary lymphoedema. Nearly 10% of all children had had at least one episode of cellulitis. The median delay from onset of symptoms to being seen by an expert centre was 2.4 years. In 44.4% of the children with primary lymphoedema a genetic test was performed, of which 35.8% resulted in a molecular diagnosis. Across the different centres, there was a wide variety in distribution of the different categories of paediatric lymphoedema diagnosed and the frequency of genetic testing. In conclusion, this paper has demonstrated that there is a large delay between the onset of paediatric lymphoedema and the first visit in the expert centres and that an episode of cellulitis is a relatively common complication. Diagnostic variation across the centres may reflect different referral criteria. Access to genetic testing was limited in some centres. It is recommended that these issues are addressed in the future work of the PPL-WG to improve the referral to the expert centres and the consistency in service provision for paediatric lymphoedema in Europe.

Prospective, randomized, controlled trial comparing a new two-component compression system with inelastic multicomponent compression bandages in the treatment of leg lymphedema.

BACKGROUND: New, less-bulky, short-stretch compression bandages could be a valuable alternative in the management of lymphedema of the leg. OBJECTIVE: To compare the effectiveness of a two-component compression (2CC) system in the treatment of leg lymphedema with that of the traditional treatment with conventional inelastic multicomponent compression bandages (IMC). METHODS: Thirty hospitalized patients with moderate to severe unilateral lymphedema (stage II-III) of the leg were included. Patients were divided in two groups; one (n=15) received a 2CC, and the other (n=15) received IMC. Primary outcome was volume reduction of the affected leg; secondary outcome was loss of interface pressure. RESULTS: Median leg volumes before bandaging were 4,150 mL (2CC) and 4,360 mL (IMC). Median volume reduction after 2 hours was 120 mL (2.9%) with the 2CC system and 80 mL (1.8%) with IMC (p>.05). After 24 hours, volume reduction was 8.4% and 4.4% respectively (p>.05). Interface pressure dropped significantly within 2 hours of bandage application in both groups. CONCLUSION: Our results indicate that the 2CC system forms a suitable alternative to IMC in the conventional treatment of moderate to severe lymphedema.

Primary lymphoedema.

Lymphoedema is the swelling of one or several parts of the body owing to lymph accumulation in the extracellular space. It is often chronic, worsens if untreated, predisposes to infections and causes an important reduction in quality of life. Primary lymphoedema (PLE) is thought to result from abnormal development and/or functioning of the lymphatic system, can present in isolation or as part of a syndrome, and can be present at birth or develop later in life. Mutations in numerous genes involved in the initial formation of lymphatic vessels (including valves) as well as in the growth and expansion of the lymphatic system and associated pathways have been identified in syndromic and non-syndromic forms of PLE. Thus, the current hypothesis is that most cases of PLE have a genetic origin, although a causative mutation is identified in only about one-third of affected individuals. Diagnosis relies on clinical presentation, imaging of the structure and functionality of the lymphatics, and in genetic analyses. Management aims at reducing or preventing swelling by compression therapy (with manual drainage, exercise and compressive garments) and, in carefully selected cases, by various surgical techniques. Individuals with PLE often have a reduced quality of life owing to the psychosocial and lifelong management burden associated with their chronic condition. Improved understanding of the underlying genetic origins of PLE will translate into more accurate diagnosis and prognosis and personalized treatment.

Shaving Technique and Compression Therapy for Elephantiasis Nostras Verrucosa (Lymphostatic Verrucosis) of Forefeet and Toes in End-Stage Primary Lymphedema: A 5 Year Follow-Up Study in 28 Patients and a Review of the Literature.

BACKGROUND: Longstanding lymphedema can lead, especially when there is recurrence of erysipelas, to irreversible elephantiasis nostras verrucosa (ENV). This predisposes to new episodes of erysipelas, leading to further damage of the lymphatics and deterioration of the lymphedema as a whole. We report the results of 28 patients with primary lymphedema and surgical removal ENV of the forefoot and toes treated between 2006 and 2014. METHOD: Retrospective descriptive 5 year follow-up study of 28 patients with various diagnosis of primary lymphedema. Wound healing time, number of erysipelas, body mass index (BMI), recurrence of EVN and types of compression were documented during follow-up. RESULTS: After preoperative multidisciplinary work up, operation of the toes with shaving and excision was performed within a conservative treatment program. During the follow up, the number of erysipelas attacks decreased dramatically (mean 17.6 vs. 0.6). Before treatment, no toecaps were used; and in follow up, it was a part of treatment. Recurrence of ENV was not observed. Compared to the literature with often BMI > 35, the mean BMI in our group was 30.0 (overweight). In 12 patients, we concurrently performed circumferential suction-assisted lipectomy for end-stage lymphedema of the leg. CONCLUSION: Although lymphedema patients are treated with garments during the maintenance phase, compression of the toes is often too challenging. Surgical removal of the verrucosis of toes is an effective therapeutic modality as part of an integrated lymphedema treatment program to restore the shape of the toes and enable the wearing of toecaps. This technique can also be effective for ENV of origins other than primary lymphedema. Although ENV is a generally accepted term, it can have undesirable connotations. We suggest using a more inclusive name such as lymphostatic verrucosis, because long-lasting lymphatic impairment is involved in all ENV and the term verrucosis is above discussion.

Effect of Nonelastic Compression With an Adjustable Wrap After Total Knee Arthroplasty.

Swelling is a common phenomenon after total knee arthroplasty, with potential for negative impact on the rehabilitation process and final outcome. The aim of this study was to investigate the effectiveness of a new compression protocol with a self-adjustable, nonelastic compression wrap for the knee region. This study was conducted as a prospective comparative study. Total leg volume and the circumference of the knee at three levels were compared between groups. The results of our study suggest that the application of the new compression protocol has no effect on swelling in the acute postoperative phase (0-2 days) but reduces swelling at Day 14 within the subacute phase. The observed positive effect of the compression protocol could be of clinical importance in the subacute phase as well as for a subgroup of patients suffering from aberrant quadriceps weakness concomitant with knee swelling.

Compression therapy in breast cancer-related lymphedema: A randomized, controlled comparative study of relation between volume and interface pressure changes.

OBJECTIVE: Short stretch bandages are very effective in the initial management of arm lymphedema. However, no studies to date have measured the pressure required to achieve specific amounts of volume reduction. The purpose of this study was to determine whether there is a difference between low and high-pressure bandaging in terms of therapeutically intended volume reduction of the compressed arm. METHODS: Experimental, randomized and comparative study with two study-groups consisting of high and low initial interface pressure bandages. Thirty-six hospitalized patients in Nij Smellinghe hospital suffering from moderate to severe unilateral breast cancer-related lymphedema not responsive to outpatient treatment were included. Bilateral arm volume was measured by inverse water volumetry before, after two hours and after 24 hours of bandaging. The amount of edema was calculated by subtracting the volume of the diseased arm from that of the contralateral side. Sub-bandage pressure was measured after bandage application and two hours later. Bandages were then re-applied and the pressure was measured again. Twenty-four hours later, the pressure measurement was repeated and bandages were removed for final volumetry. Patients were randomized into two groups: group A received low pressure bandages (20-30 mm Hg) and group B received high pressure bandages (44-58 mm Hg). The main outcome measures were reduction of arm volume and edema volume in the affected arm in both study groups. Secondary outcome parameters were changes in sub-bandage pressure and patient comfort. RESULTS: Median arm volume reduction after two and 24 hours was 104.5 mL (95% confidence interval [CI], 51.2-184.2) (-2.5%) (P < .0001) and 217 mL (95% CI, 143.9-280.2) (-5.2%) (P < .01) for group A and 56.5 mL (95% CI, -2.7-123.1) (n.s.) and 167.5 mL (95% CI, 105.2-316.1) (-4.2%) (P < .01) for group B, respectively. There was no statistically significant difference between the volume changes in group A and group B. After 24 hours, edema decreased by median percentage of 9.2% in group A and 4.8% in group B (n.s.). Bandages in group A were better tolerated. The sub-bandage pressure drop in the first two hours was between 41% and 48% in both treatment groups at both measuring sites. After 24 hours, the pressure drop was between 55% and 63%. No proximal swelling above the bandage was observed. CONCLUSIONS: Inelastic, multi-layer, multi-component compression bandages with lower pressure (20-30 mm Hg) are better tolerated and achieve the same amount of arm volume reduction as bandages applied with higher pressure (44-58 mm Hg) in the first 24 hours.

Controlled, comparative study of relation between volume changes and interface pressure under short-stretch bandages in leg lymphedema patients.

BACKGROUND: Pressure loss under short-stretch bandages (SSBs) is a well-known phenomenon and is thought to be related to bandage relaxation and poor quality of application. AIM: The aim was to demonstrate that therapeutically intended volume reduction of the compressed leg is the most important cause for the loss of bandage pressure. DESIGN: This was an experimental, controlled comparative study. METHODS: In 20 patients suffering from lymphedema of the lower extremities and in 9 healthy persons, lower leg volume was measured by water displacement. After application of a pressure measurement device (Kikuhime) at the B1 region, SSBs were applied on one leg according to a standard protocol. Volume and pressure measurements were performed at 0, 2, and 24 hours in supine as well as standing positions. RESULTS: A significant reduction of leg volume is already achieved 2 hours after bandage application, both in lymphedema patients and in normal controls. A further volume decrease of the lymphedematous legs is observed in the following 24 hours after application of a new bandage (-290 mL). The volume reduction is associated with a significant loss of bandage pressure from initial values over 60 mmHg by 37 and 48% in controls and lymphedema patients, respectively. CONCLUSIONS: Inelastic, multilayer, multicomponent compression bandages lead to an immediate reduction of leg volume, both in lymphedematous and in normal legs. This therapeutically intended volume reduction seems to be the main reason for the fast decrease of the subbandage pressure. This study was supported by Lohmann & Rauscher, Ltd. Germany.

The Dutch lymphedema guidelines based on the International Classification of Functioning, Disability, and Health and the chronic care model.

Lymphedema is a chronic and progressive condition due to an imbalance between lymphatic filtration and transport capacity. A relative overload of fluid is caused by lymphatic impairment (afterload impairment); an absolute overload is caused by increased filtration (preload raised). Lymphedema can result in considerable disability and loss of quality of life. The Dutch Society of Dermatology organized a task force to create guidelines using the International Classification of Functioning, Disability, and Health. As lymphedema is a chronic condition, a new approach was chosen according to the chronic care model. In the development of the guidelines, the following topics of lymphedema care were proposed: (1) lymphedema detection; (2) clinimetric instruments; and (3) rational treatment modalities. A systematic review of the literature published up to June 2013 was conducted. Statements were based on scientific evidence and experience. The guidelines propose recommendations based on an interdisciplinary approach to lymphedema using a continuum of care from prevention to initial treatment phase, maintenance phase, and follow-up. An integrated treatment program can be conservative or surgical. A therapeutic program depends on stage and origin of lymphedema; International Classification of Functioning, Disability, and Health-based limitations; needs of the patient; ability to perform self-management; and ability to reduce patient-related risk factors, such as overweight and lack of exercise.