The Lymphatic System is part of the circulatory system and it is indispensable for life. Morphological or functional changes in lymphatic vessels can contribute to disorders such as lymphedema, tumor metastasis, inflammation, and other pathological conditions. Lymphedema (LE) arises when there is a disruption of lymphatic flow (Organic Impairment), leading to the buildup of lymphatic fluid. Nontreatment and undertreatment of lymphedema are associated with an unfavorable prognosis of disease progression and severity, which are barriers to work and causes of social stigmatization, discrimination, and loss of Quality of Life (QoL).
Lymphedema is a progressive disease of the skin classified as a functional, immune, and lymphatic circulatory system disorder (Organic Lymphatic Vascular Disease). The lymphatics are an active and integrated component of the immune response, and in lymphedema, there is always an increased susceptibility to infection due to the compromised system. Read more about what is an Organic Impairment HERE.
It is clear that no patient or their lymphedema is the same and neither is its progress. Some patients with lymphedema have few symptoms, and can easily do different degrees of physical activity, while others feel the strain, experience increased swelling with minimum effort, or even suffer varying degrees of disability and severe complications. Read more about lymphedema and Infectious Cellulitis HERE.
In recent years, some lymphedema guidelines and protocols have been developed, to support health policy guidelines, that focus on the implementation of low-budget health management for the treatment of lymphoedema. To achieve this goal, a new treatment concept called “Decongestive Lymphatic Therapy (DLT)” was developed to principally limit or exclude Manual Lymphatic Drainage (MLD) conducted by therapists at the specialist level.
In many countries lymphedema care services are scarce, and various strategies are been used to justify and implement low-cost alternative treatments, including downplaying conservative treatment (CDT) or undermining the importance of Manual Lymphatic Drainage (MLD). The resulting management approach emphasizes the provision of treatment with MLD only for "Very Severe" lymphedema (elephantiasis) and complex cases, thus excluding intervention for patients with mild, moderate, and severe lymphedema.
Even though the terms “Complete Decongestive Physiotherapy" (CDT) and “Decongestive Lymphatic Therapy" (DLT) are used synonymously in some medical literature, they are not equivalent and don't represent the same treatment management focus. Read more about the main differences between “CDT” and “DLT” HERE.
Paradoxically, some treatment guidelines, while stating that Manual Lymphatic Drainage (MLD) should only be conducted by therapists at the specialist level, simultaneously detract from MLD and recommend as a good and valid alternative, self-management options for the intensive volume reduction phase, even though this has little scientific evidence.
Concerning the objective of discrediting Manual Lymphatic Drainage (MLD), there are confusing research studies that focus on demonstrating its unnecessary inclusion as an essential component of treatment, basing the reports only on the treatment results in terms of volumetric reduction measures, but not taking into account the key functions of MLD. Read more about the principal functions of Manual Lymphatic Drainage HERE.
These studies and trials do not mention the possible serious and irreversible side effects that may arise in the short or long term, if Manual Lymphatic Drainage (MLD) is not included during the application of compression therapy in the process of reducing the volume of the oedematous limb, such as the possibility of lymph displacement to previously unaffected areas.
Many lymphedema-related healthcare management studies are only cost-saving non-investment strategy tools. It should be borne in mind that the annual health expenditure for the treatment and maintenance of lymphedema adds far less in comparison to the cost of treatment for many chronic diseases, and even so, many national health systems do not treat or are undertreating lymphedema.
Early detection and preventive treatment (Preventive Medicine) can reduce overall costs by keeping affected limbs at minimal volumes and other co-morbidities and complications before they become chronic.
- Manual Lymphatic Drainage (MLD) is part of the conservative treatment known as Complex Decongestive Therapy (CDT). The five components of the Initial Phase I are skin care, manual lymphatic drainage (MLD), multilayer compression bandaging, decongestive exercises, and patient education.
- It must be taken into account that the isolated application of individual components is not recommended and, that treatment without Manual Lymphatic Drainage (MLD) does not represent Complex Decongestive Therapy (CDT).
- There is a big difference between the terms "Complex Decongestive Physiotherapy" (CDT) and "Decongestive Lymphatic Therapy" (DLT).
- The term "Compression Therapy” is used to describe the compression part of lymphedema therapy, whether it is bandaging or a custom-fitted garment. Edema volume reduction is principally achieved by compression, and wrapping with a low-stretch bandage is the major component of the intensive volume reduction phase.
- The volume reduction produced by compression is mostly due to a reduction in tissue water content, with proportionately less protein removed. The volume reduction produced by compression is mainly due to a reduction in the water content of the tissues, with a proportionally lower elimination of proteins. Isolated application of compression is not recommended because of the risk of draining líquid and not proteins, reducing volume but increasing the concentration of proteins with the consequent risk of generating Fibrosis.
- Isolated compression therapy of the extremities, particularly in those with lymphedema also affecting the quadrants of the trunk, may increase swelling at the root of the extremity and may even lead to Genital Lymphedema.
- Manual Lymphatic Drainage (MLD) should always be applied before the compression phase and is not intended to be a stand-alone treatment option. MLD as a stand-alone treatment is used when treating body parts that are not conducive to sustained compression (e.g., face, breast, genitalia, and trunk).
- The main functions of Manual Lymphatic Drainage (MLD) are aimed at lymph vessel and lymph node drainage; rerouting of lymph into nonobstructed lymphatics; development of accessory lymph collectors; improvement of lymphatic contractility; and prevention of the displacement of lymph fluid to adjunct areas when lymphatic edema is submitted to compression therapy (bandages/compression pumps) for volumetric reduction. It must be kept in mind that the primary role of MLD is "NOT" for volumetric reduction.
- Preventive Medicine indicates lymphedema treatment in the early stages of the disease, but even in lymphedema stages II and III, adequate treatment with Complete Decongestive Therapy (CDT) is successful in the majority of cases, without having to resort to aggressive surgeries.
IMPORTANT NOTICE
Lymphedema is a serious chronic and progressive disease due to lymphatic dysfunction (Organic Lymphatic Vascular Disease). The clinical treatment of lymphedema is neither a cosmetic nor an aesthetic treatment. The treatment of lymphedema is to control its progression and alleviate the symptoms related to dysfunction of the lymphatic circulatory system.
To prevent and avoid serious complications associated with treatment, such as the possible displacement of edema to previously unaffected areas when compression therapy is applied for volume reduction, patients should use highly specialized and experienced therapists.
Professional qualification and instruction delivered remotely online are not the same as live hands-on practical instruction in the clinical training and certification of lymphedema therapists, similar to all other rehabilitation, medical, and surgical training programs, and especially for developing the necessary manual skills to treat a disease as complex as lymphedema.
Patients should also take special care when choosing a Multidisciplinary Expert Center of Reference for Lymphedema, as not all countries and centers provide the same treatment options. The best choice is a center of reference that provides Complete Decongestive Therapy (CDT), which is recognized as the “Gold Standard” treatment for lymphedema.
- Read more about what is the best treatment option for Lymphedema HERE.
- Read about what are the main differences between the two principal lymphedema treatment protocols HERE.
REFERENCES
(Click on the texts to read the research articles)
SCIENTIFIC SOCIETIES
PATHOPHYSIOLOGY
- lymphedema.
- Biology of Lymphedema
- Lymph vessels: the forgotten second circulation in health and disease
- Lymphatic Vessel Network Structure and Physiology
- Lymphatic System Flows
- Organ-specific lymphatic vasculature: From development to pathophysiology.
- The lymphatic vascular system: much more than just a sewer.
- The unresolved pathophysiology of lymphedema.
- Lymphedema: A Practical Approach and Clinical Update.
- Coagulation in Lymphatic System.
- Regulation of immune function by the lymphatic system in lymphedema.
- Oxidative stress in chronic lymphoedema.
- Regulatory T Cells Mediate Local Immunosuppression in Lymphedema.
- Hemostatic properties of the lymph: relationships with occlusion and thrombosis.
- Secondary lymphedema: Pathogenesis
- Gastrointestinal Lymphatics in Health and Disease
- Intestinal lymphangiectasia in adults
- Role of the lymphatic vasculature in cardiovascular medicine
- The lymphatic vasculature in disease
- Lymphoscintigraphic abnormalities in the contralateral lower limbs of patients with unilateral lymphedema.
- The Prevalence of Lower Limb and Genital Lymphedema after Prostate Cancer Treatment: A Systematic Review
- Peripheral Edema
- The lymphatic system and the skin. Classification, clinical aspects, and histology.
- Lymphedema and cutaneous diseases.
- Lymphedema and subclinical lymphostasis (microlymphedema) facilitate cutaneous infection, inflammatory dermatoses, and neoplasia: A locus minoris resistentiae.
- Serum Immune Proteins in Limb Lymphedema Reflecting Tissue Processes Caused by Lymph Stasis and Chronic Dermato-lymphangio-adenitis (Cellulitis).
- Lymphedema and subclinical lymphostasis (microlymphedema) facilitate cutaneous infection, inflammatory dermatoses, and neoplasia: A locus minoris resistentiae.
- Lymphatic Flow: A Potential Target in Sepsis.
- Infectious complications of lymphedema.
- Acute inflammatory exacerbations in lymphoedema.
- Inflammatory Manifestations of lymphedema.
- Cellulitis.
- Clinical features, microbiological epidemiology, and recommendations for the management of cellulitis in extremity lymphedema.
- Challenges of cellulitis in a lymphedematous extremity.
- Diagnosis and management of cellulitis.
- Cellulitis risk factors for patients with primary or secondary lymphedema.
- Oedema as a risk factor for multiple episodes of cellulitis/erysipelas of the lower leg.
- Prevention of dermatolymphangioadenitis by combined physiotherapy.
- Erysipelas: a common potentially dangerous infection.
- Malignant tumors as complications of lymphedema.
- Lymphedematous areas: Privileged sites for tumors, infections, and immune disorders.
- Lymphedema-related angiogenic tumors and other malignancies.
- Lymphedema: an immunologically vulnerable site for the development of neoplasms.
- Lymph stasis promotes tumor growth
- A retrospective analysis of Stewart-Treves syndrome in the context of chronic lymphedema.
- Stewart-Treves Syndrome
- Congenital lymphedema complicated by pain and psychological distress: case report
- Lymphatic Pain in Breast Cancer Survivors
- Does Manual Lymphatic Drainage Have Any Effect on Pain Threshold and Tolerance of Different Body Parts?
- Lymphedema therapy reduces the volume of edema and pain in patients with breast cancer
- Quality of life in patients with primary and secondary lymphedema in the community
TREATMENT
- Treatment of limbs lymphedema.
- Nonoperative treatment of lymphedema.
- Lymphedema: From diagnosis to treatment.
- Lymphedema-clinical picture and therapy.
- Physiotherapeutic rehabilitation of lymphedema: state-of-the-art.
- Diagnosis and management of lymphatic vascular disease
- Effective treatment of lymphedema of the extremities.
- Lymphoscintigraphic aspects of the effects of manual lymphatic drainage.
- Intensive Treatment of Lower-Limb Lymphedema and Variations in Volume.
- Effectiveness and safety of Complete Decongestive Therapy of Phase I.
- Therapeutic Efficacy of Complex Decongestive Therapy in the Treatment of Elephantiasis of the Lower Extremities.
- Effects of Phase I complex decongestive physiotherapy on physical functions and depression levels in breast cancer-related lymph edema.
- Does lymphoedema bandaging reduce the risk of toe ulceration?
- Study of 700 referrals to a Lymphedema Program.
- Worldwide assessment of healthcare personnel dealing with lymphoedema.
- Földi M, Földi E. Földi’s textbook of lymphology for physicians and Lymphoedema therapists.
- Fluid Shifts Induced by Physical Therapy in Lower Limb Lymphedema Patients
- Visualization of Accessory Lymphatic Pathways, before and after Manual Drainage, in Secondary Upper Limb Lymphedema Using Indocyanine Green Lymphography
- The risk of genital edema after external pump compression for lower limb lymphedema.
- The management of genital lymphoedema
- The management of deep vein thrombosis in lymphoedema: a review.
- Lymphedema-associated comorbidities and treatment gap.
- A study of the advantages of elastic stockings for leg lymphedema.
- Indications for medical compression stockings in venous and lymphatic disorders: An evidence-based consensus statement.
- Medical compression stockings for chronic venous diseases and lymphedema: Scientific evidence and results of a patient survey on quality of care.
- Impact of Compression Therapy on Cellulitis (ICTOC) in adults with chronic edema: a randomized controlled trial protocol.
- Compression Therapy Is Cost-Saving in the Prevention of Lower Limb Recurrent Cellulitis in Patients with Chronic Edema.
- Occupational leg edema-use of compression stockings.
DISABILITY
- Disability and lymphedema.
- Lymphedema and employability.
- Worse and worse off: the impact of lymphedema on work and career after breast cancer
- Functioning in lymphedema from the patient's perspective using the International Classification of Functioning, Disability and Health (ICF).
- Unilateral upper extremity lymphedema deteriorates the postural stability in breast cancer survivors
- Postural Stability in Patients with Lower Limb Lymphedema
- Disability, psychological distress and quality of life in breast cancer survivors with arm lymphedema
DISFIGUREMENT
- Adjusting to disfigurement: processes involved in dealing with being visibly different
- Quality of Life in Cancer Patients with Disfigurement due to Cancer and its Treatments
- Quality-of-life and body image impairments in patients with lymphedema
- Association of lower extremity lymphedema with pelvic floor functions, sleep quality, kinesiophobia, body image in patients with gynecological cancers
QUALITY OF LIFE
- The impact of lower limb chronic oedema on patients' quality of life
- Quality of life in patients with primary and secondary lymphedema in the community
- Evaluating the effect of upper-body morbidity on quality of life following breast cancer treatment.
- Functionality and quality of life of patients with unilateral lymphedema of a lower limb: a cross-sectional study
- The effect of complete decongestive therapy on the quality of life of patients with peripheral lymphedema
PSYCHOSOCIAL IMPACT
- Psychosocial Impact of Lymphedema.
- Associations between chronic disease, age and physical and mental health status
- A network analysis of psychological flexibility, coping, and stigma in dermatology patients
- 'Abandoned by medicine'? A qualitative study of women's experiences with lymphoedema secondary to cancer, and the implications for care
- People are neglected, not diseases.
- Chronic edema/lymphoedema: under-recognized and under‐treated.
- Lymphatic Medicine: Paradoxically and unnecessarily ignored.
- Medical education: a deficiency or a disgrace.
PEDIATRIC LYMPHEDEMA
- NORD: Rare Disease Database - Lymphedema
- Medical management of lymphedema.
- Primary lymphedema in childhood.
- An approach to familiar lymphedema
- Pediatric Children Lymphedema
- Primary Lymphedema French National Diagnosis and Care Protocol (PNDS).
- Lymphatic filariasis: an infection of childhood.
- Lymphatic filariasis in children: clinical features, infection burdens and future prospects for elimination.
- Podoconiosis: Clinical spectrum and microscopic presentations.
- Podoconiosis: A Possible Cause of Lymphedema in Micronesia.
- A cross-sectional study to evaluate depression and quality of life among patients with lymphoedema due to podoconiosis, lymphatic filariasis, and leprosy.
- The impact of acute adenolymphangitis in podoconiosis on caregivers.
- Neglected tropical diseases and disability-what is the link?
- Locomotor disability in bancroftian filarial lymphoedema patients.
- Neglected patients with a neglected disease?
- Neglected tropical diseases and mental health: a perspective on comorbidity.
- People are neglected, not diseases.
- Addressing Inequity: Neglected Tropical Diseases and Human Rights.
- Neglected Tropical Diseases, Conflict, and the Right To Health.
- Peripheral Edema
- Phlebolymphedema
- Understanding Chronic Venous Disease: A Critical Overview of Its Pathophysiology and Medical Management
- Role of the lymphatic vasculature in cardiovascular medicine
- Communication between lymphatic and venous systems
- Phlebopathies and occupation
- Phlebopathies and workers
- Evidence of health risks associated with prolonged standing at work and intervention effectiveness
- Leg edema formation and venous blood flow velocity during a simulated long-haul flight
