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

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

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

Hereditary lymphoedema 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 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 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. 

WHAT IS THE GOLD STANDARD TREATMENT FOR PEDIATRIC AND PRIMARY LYMPHOEDEMA

After Clinical evaluation and imaging tests to confirm the diagnosis of Lymphoedema, 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 to 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. Read more about what is the best treatment for lymphoedema HERE.

The ideal setting and approach for treating lymphoedema 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, which should work together in the assessment and management of all aspects of lymphoedema. Read more about what kind of doctor treats lymphoedema HERE.

There is no cure for lymphoedema, but as well as other chronic diseases that need life-long treatment or medication to control the condition, in the majority of cases lymphoedema 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 life. With proper diagnosis and management, the progression and potential complications of lymphoedema may be limited in many cases. Read more about which country has the best lymphoedema treatment coverage HERE.

Patients should be especially careful when choosing a Multidisciplinary Centre of Reference/Excellence for lymphedema, as not all counties 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 key difference between the two main treatment protocols HERE.

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

REFERENCES

(Pinch on the texts to read the following Research Articles)

SCIENTIFIC SOCIETIES

• GERMAN SCIENTIFIC SOCIETY GUIDELINE: Diagnostics and Therapy of Lymphoedema.
• THE INTERNATIONAL SOCIETY OF LYMPHOLOGY: Consensus Document.
• THE PRIMARY LYMPHEDEMA FRENCH NATIONAL DIAGNOSIS AND CARE PROTOCOL (PNDS; Protocole National de Diagnostic et de Sions)

PEDIATRIC AND PRIMARY LYMPHEDEMA

• NORD: Rare Disease Database. Lymphedema
• AN APPROACH TO FAMILIAL LYMPHOEDEMA
• PRIMARY LYMPHEDEMA
• PEDIATRIC CHILDREN LYMPHEDEMA: A Retrospective Chart Review of 86 Cases
• LYMPHATIC FILARIASIS: An Infection of Childhood

Articles that may also interest you: 
(Click on the texts)




• FOCUS ON THE TERM ELEPHANTIASIS AND ITS IMPLICATIONS FOR LYMPHOEDEMA 




• WHAT ARE THE RECOMMENDATIONS TO AVOID INFECTIOUS CELLULITIS AND LYMPHANGITIS IN LYMPHEDEMA 




• WHAT IS THE RISK OF DERMATOLYMPHANGIOADENITIS (INFECTIOUS CELLULITIS IN LYMPHEDEMA 




• WHAT IS THE BEST TREATMENT OPTION FOR ELEPHANTIASIS




• WHY IS ELEPHANTIASIS A FORGOTTEN AND NEGLECTED DISEASE




• IS THERE DISTRESS ASSOCIATED WITH UNDER-TREATMENT OF LYMPHEDEMA - PSYCHOLOGICAL CONSEQUENCES




• WHY IS THE WORLD NOT TREATING A TREATABLE DISEASE LIKE LYMPHEDEMA. 




• WHAT IS THE BEST TREATMENT OPTION FOR LYMPHEDEMA




• IS LYMPHEDEMA DISABLING - INABILITY TO WORK AND DISABILITY RECOGNITION 




• INTERNATIONAL AWARENESS CAMPAIGN TO PROMOTE CORRECT TREATMENT FOR LYMPHEDEMA




• ELEPHANTIASIS IS NOT ONLY LYMPHATIC FILARIASIS 




• CAMPAIGN TO PREVENT CHILD ELEPHANTIASIS DUE TO PRIMARY AND SECONDARY LYMPHEDEMA 




• BEWARE OF NEW TERMINOLOGY DESCRIBING LYMPHEDEMA TREATMENT




• WHAT KIND OF DOCTOR TREATS LYMPHEDEMA/LYMPHOEDEMA - WHICH MEDICAL SPECIALITIES ARE RELATED TO LYMPHEDEMA







• FAST TRACK SOCIAL SECURITY DISABILITY RECOGNITION FOR CHILDREN WITH LYMPHEDEMA




• INTERMITTENT PNEUMATIC COMPRESSION (IPC) PUMP DEVICES. SERIOUS SIDE EFFECTS POR LYMPHEDEMA




• BEST PRACTICE PROTOCOL GUIDE FOR LYMPHOEDEMA (in Galician)




• A LYMPHEDEMA STORY - DOCTOR KENNETH GERSHENGORN AND THE MAUREEN DIGAN CLINICAL CASE




• WHY IS A BUTTERFLY THE SYMBOL FOR LYMPHEDEMA




• LYMPHEDEMA - INTERNATIONAL DISEASE CODING AND CLASSIFICATION




• WHAT DOES INTERMITTENT PNEUMATIC COMPRESSION PUMP THERAPY (IPC) DO, AND DOES NOT DO FOR LYMPHEDEMA. 




• WHAT IS PHLEBOLYMPHEDEMA.




• IS LYMPHEDEMA PAINFUL?




• WHICH COUNTRY PROVIDES THE BEST LYMPHOEDEMA TREATMENT COVERAGE 




• WHAT IS THE BEST TREATMENT OPTION FOR PAEDIATRIC AND PRIMARY LYMPHOEDEMA




• SPANISH LYMPHEDEMA ASSOCIATIONS – SPANISH NATIONAL FEDERATION OF LYMPHEDEMA ASSOCIATIONS








LYMPHEDEMA INCIDENCE 
AND PREVALENCE
(click on the texts)