Objective Medical compression stockings are a standard, non-invasive treatment option for all venous and lymphatic diseases. The aim of this consensus document is to provide up-to-date recommendations and evidence grading on the indications for treatment, based on evidence accumulated during the past decade, under the auspices of the International Compression Club. Methods A systematic literature review was conducted and, using PRISMA guidelines, 51 relevant publications were selected for an evidence-based analysis of an initial 2407 unrefined results. Key search terms included: 'acute', CEAP', 'chronic', 'compression stockings', 'compression therapy', 'lymph', 'lymphatic disease', 'vein' and 'venous disease'. Evidence extracted from the publications was graded initially by the panel members individually and then refined at the consensus meeting. Results Based on the current evidence, 25 recommendations for chronic and acute venous disorders were made. Of these, 24 recommendations were graded as: Grade 1A (n = 4), 1B (n = 13), 1C (n = 2), 2B (n = 4) and 2C (n = 1). The panel members found moderately robust evidence for medical compression stockings in patients with venous symptoms and prevention and treatment of venous oedema. Robust evidence was found for prevention and treatment of venous leg ulcers. Recommendations for stocking-use after great saphenous vein interventions were limited to the first post-interventional week. No randomised clinical trials are available that document a prophylactic effect of medical compression stockings on the progression of chronic venous disease (CVD). In acute deep vein thrombosis, immediate compression is recommended to reduce pain and swelling. Despite conflicting results from a recent study to prevent post-thrombotic syndrome, medical compression stockings are still recommended. In thromboprophylaxis, the role of stockings in addition to anticoagulation is limited. For the maintenance phase of lymphoedema management, compression stockings are the most important intervention. Conclusion The beneficial value of applying compression stockings in the treatment of venous and lymphatic disease is supported by this document, with 19/25 recommendations rated as Grade 1 evidence. For recommendations rated with Grade 2 level of evidence, further studies are needed.

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Indications for medical compression stockings in venous and lymphatic disorders: An evidence-based consensus statement:

The current document is intended to define a consensus on the diagnostic guidelines for patients with lymphedema, based upon literature­based evidence, both clinical and investigative. General diagnostic guidelines include systemic evaluation; assessment of venous function; duplex ultrasonography; and lymphoscintigraphy. Proper diagnosis should allow appropriate clinical and laboratory staging of the disease for the assessment of progression of the condition and its response to treatment. Diagnosis should include an assessment of the infections in the early and latent stages. Lymphedema is assessed by the stage of disease (0­III) and WHO guidelines for International Classification of Functioning, Disability and Health and Quality of Life issues can be used as a reference for the impact of this complex condition on social, emotional, and physical function. It is the authors’ intent that this document stimulate further inquiry and discussion regarding all aspects of lymphedema diagnosis.

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IUA-ISVI consensus for diagnosis guideline of chronic lymphedema of the limbs.

Complex Decongestive Lymphatic Therapy With or Without Vodder II Manual Lymph Drainage in More Severe Chronic Postmastectomy Upper Limb Lymphedema: A Randomized Noninferiority Prospective Study

BACKGROUND Lymphedema can be classified as either primary or secondary. In the present study, the authors investigated the efficacy of lymphaticovenous anastomosis for correcting primary lower limb lymphedema and to determine its indications and contraindications. METHODS The authors retrospectively examined patients with primary lower limb lymphedema who underwent lymphaticovenous anastomosis between April of 2009 and September of 2013. Anastomosis efficacy was evaluated using lower limb circumference measurements at five anatomical locations. Lymphedema staging was determined using modified leg dermal backflow stage. The authors added two categories to the standard leg dermal backflow staging system: no backflow and distal backflow. Lymphaticovenous anastomosis was performed under local anesthesia, except in pediatric cases. RESULTS The authors evaluated 62 patients (79 lower limbs). Lower limb circumference increased after lymphaticovenous anastomosis in patients with an onset age of 1 year or later and before age 11 years, but it significantly decreased in patients with an onset age older than 11 years. The presence of lymphedema for a longer period did not negatively impact lymphaticovenous anastomosis efficacy. In particular, lymphaticovenous anastomosis was effective in the leg dermal backflow stage 2 and no backflow group. CONCLUSIONS For patients developing lymphedema before 11 years of age, the indications should be considered carefully. However, lymphaticovenous anastomosis was effective in patients developing lymphedema after the age of 11 years. Regardless, due to its low level of invasiveness, lymphaticovenous anastomosis may be considered for patients who are refractory to conservative treatment, even if they have early-onset lymphedema. CLINICAL QUESTION/LEVEL OF EVIDENCE Therapeutic, IV.

Indication of Lymphaticovenous Anastomosis for Lower Limb Primary Lymphedema.

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.

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

Primary lymphedema can be managed effectively as a form of chronic lymphedema by a sequenced and targeted treatment and management program based around a combination of Decongestive Lymphatic Therapy (DLT) with compression therapy, when the latter is desired as an adjunct to DLT. Treatment in the maintenance phase should include compression garments, self-management, including self-massage, meticulous personal hygiene and skin care, in addition to lymphtransport-promoting excercises and activities, and, if desired, pneumatic compression therapy applied in the home. When conservative treatment fails, or gives sub-optimal outcomes, the management of primary lymphedema can be improved, where appropriate, with the proper addition of surgical interventions, either reconstructive or ablative. These two surgical therapies can be more effective when fully integrated with manual lymphatic drainage (MLD)-based DLT postoperatively. Compliance with a long-term commitment to MLD/DLT and particularly compression postoperatively is a critical factor in determining the success of any new treatment strategy involving either reconstructive or palliative surgery. The future of management of primary lymphedema has never been brighter with the new prospect of gene-and perhaps stem-cell oriented management.

N Bunke

Papers

Diagnosis and treatment of primary lymphedema. Consensus document of the International Union of Phlebology (IUP)-2013.