domingo, 6 de septiembre de 2020

WHAT IS THE BEST TREATMENT OPTION FOR PAEDIATRIC AND PRIMARY LYMPHEDEMA - Organic Lymphatic Vascular Disease - Milroy Disease – Meige Disease - Lymphoedema Tarda - Congenital Hereditary Lymphedema – Quality of life & Disability - Children with Lymphedema - Lymphostatic Elephantiasis/Lymphatic Filariasis/Podoconiosis - Best Practices Management Guideline - Patient Care Pathway

Primary lymphedema can be hereditary or sporadic. Hereditary lymphedema, also known as Primary Congenital Lymphedema, is a genetic disorder affecting the lymphatic system and is inherited as an autosomal dominant trait. There are three forms of hereditary lymphedema:

  • Lymphedema that appears at birth (MILROY DISEASE)
  • Lymphedema that appears at puberty/adolescence (LYMPHOEDEMA PRAECOX or MEIGE DISEASE)
  • Lymphedema that appears after the age of 35 (LYMPHOEDEMA TARDA)

Hereditary lymphedema may be associated with several genetic multisystem disorders. Sporadic lymphoedema is referred to when there is no obvious cause or family history (the condition is not inherited).

Lymphoedema is a progressive disease of the skin, classified as a functional immune, and lymphatic circulatory 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 immune system. Read more about what is an Organic Impairment HERE

Lymphedema arises when there is a disruption of lymphatic flow (Organic Impairment), leading to the buildup of lymphatic fluid. 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.

Lymphoedema is characterized by chronic progressive inflammation and swelling (edema), which may be present in the trunk, face, genitalia, hands, arms, legs, feet, and also internal organs. Lymphatic dysfunction (Organic Lymphatic Vessel Disease) is due to obstruction, malformation, or underdevelopment of the lymphatic vessels, which causes the accumulation of protein-rich fluid (lymph) in the soft layers of tissue under the epidermis. 


After Clinical evaluation and imaging tests to confirm the diagnosis of Lymphedema, COMPLETE DECONGESTIVE THERAPY (CDT) is recommended as the first-line Gold Standard treatment, consisting of intensive and long-term management phases. Conservative therapy in childhood needs to be suitably adapted to the respective age and involves the direct participation of the parents. 

The initial Intensive Phase aims to reduce swelling and normalize the tissue pressure and consists of Manual Lymph Drainage (MLD) in combination with multilayered compression bandaging, meticulous skin care, exercise, and the use of well-fitted compression garments. CDT and MLD should be conducted by lymphedema therapists with training at the specialist level. After the volume of the limb is stabilized, the patient enters the Maintenance Phase in which she/he continues with skin care, compression garments, and exercises. In Phase II, the components of CDT are applied according to the individual edema findings, and depending on the course of the disease, Phase I may have to be repeated, or in the case of intercurrent diseases.

The ideal setting and approach for treating lymphedema are in a Multidisciplinary Unit, where the special needs of each patient are addressed. The team should be composed of a lymphedema therapist, dermatologist, dietician, internist, pediatrician, and vascular and/or plastic surgeon, who should work together in the assessment and management of all aspects of lymphedema. Read more about what kind of doctor treats lymphedema HERE.

There is no cure for lymphedema, but as well as other chronic diseases that need life-long treatment or medication to control the condition, in the majority of cases lymphedema also needs continued treatment for maintenance and control of progression. Patients require regular revisions and therapy, so as to improve their physiological response to the disease and ultimately, also enhance their quality of lifeWith proper diagnosis and management, the progression and potential complications of lymphedema may be limited in many cases. Read more about which country has the best lymphedema treatment coverage HERE.

Patients and their parents/caregivers should also be counseled on the importance of adhering to lymphedema basic-care recommendations, to help prevent the progression of the disease and possible complications. 


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 principal functions of Manual Lymphatic Drainage HERE.
  • Read about what are the main differences between the two principal lymphedema treatment protocols HERE. 
  • Read about what are the strategies for the implementation of low-cost treatment options for Lymphedema HERE.


(Click on the texts to read the research articles)











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