My child suffers from lymphedema

Although it is rare in children, and probably underestimated, paediatric lymphedema can appear from a very young age⁽¹⁾.

Its prevalence is estimated at 1/100,000 before the age of 20⁽¹⁾. Childhood lymphedema, which is primary in most cases, is generally due to a constitutional malformation of the lymphatic system (abnormal lymph nodes, lack of lymphatic vessels, non-functionalitý of the lymphatic network...), causing a chronic accumulation of lymph in the subcutaneous tissue of one or more limbs. 
Childhood lymphedema can appear in utero (it can be detected by a foetal ultrasound scan), at birth, during the first years of life and up until adolescence. Boys are more frequently affected at birth, while girls are affected in adolescence⁽²⁾. Although a clinical examination is sufficient for diagnosis, the disease is generally confirmed by lymphoscintigraphy⁽³⁾. Lymphoscintigraphy, which can be performed from the first few months of life, provides information on lymphedema compared with other possible methods of exploration⁽³⁾ (lympho-MRI⁽⁴⁾, MRI⁽¹⁾, ultrasound⁽⁵⁾, etc.).

The different types of paediatric lymphedema

As in adults, paediatric lymphedema can be primary or, less frequently, secondary. Primary lymphedemas are sporadic (or isolated), more rarely hereditary (in 7% of cases)⁽⁶⁾, congenital (Milroy's disease) or not (Meige's disease), or form part of more complex genetic malformative syndromes. "In children, lymphedema may be accompanied by other vascular and/or organ malformations, such as Turner's, Klinefelter's, Noonan's, Klippel-Trénaunay's, Parkes-Weber's syndromes, yellow nails, trisomy 21, 13, 18, etc.", attests Dr Marlène Coupé⁽⁷⁾, a vascular physician. 
In cases of congenital primary lymphedema, lymphatic oedema appears from birth with a possible improvement phase around the age of two⁽⁶⁾. A distinction is made between Milroy's disease, which accounts for approximately 2% of primary lymphedemas(6) and is limited to the lower limbs⁽⁷⁾. 
Meige's disease is a non-congenital hereditary form. Lymphedema appears at puberty and is sometimes severe. Other anomalies are rarely associated (entropion, platinum cleft, facial oedema, serous effusion, laryngeal oedema)⁽⁷⁾.

The symptoms

Paediatric lymphedema rarely affects the upper limbs (4%), genitals (11%) or face (2.5%). The lower limbs are affected in 86% of cases, with bilateral involvement in 47% of cases. Lymphedema may also affect all four limbs and the face (7%)⁽⁶⁾. 
Signs include hyperpigmentation, increased skin folds on the toes and at the base of the toes, curved nails that grow upwards and a double row of eyelashes. Oedema predominates in the forefoot and Stemmer's sign, difficulty pinching the skin on the dorsal surface of the 2nd toe, is suggestive⁽⁷⁾. 
The cup sign can also be seen with orange peel skin⁽⁷⁾.

Treatments

Treatment is based on a combination of physiotherapy using manual lymphatic drainage and bandaging. Massages, lasting a minimum of 20 minutes, are given one to four times a week, depending on the severity of the lymphedema, the parents' availability and access to this treatment⁽¹⁾. Bandaging, designed to reduce the volume of lymphedema, is an essential part of decongestive physiotherapy. 
In children, a custom-made, low-compression stocking or sleeve is made and changed regularly, as it wears out or as the child grows. The device can be removed at night and put back on in the morning. Depending on the child's age and difficulty in accepting it, it can be applied at weekends only or during the week excluding weekends, with the ideal being daily use of the device⁽¹⁾. It is important to start these treatments very early, possibly after the child has learned to walk (at the age of two, for example, in the case of congenital lymphedema). The aim is to limit functional discomfort, reduce the volume of lymphedema and slow its progression to a more serious stage⁽¹⁾.

Precautions to take

In addition to the sometimes significant functional and psychological repercussions, erysipelas is the main complication of paediatric lymphedema⁽⁸⁾. 
It is advisable to :

• Monitor and treat the appearance of fungal infections, particularly between the toes; dry the lymphedemic limb and toes very carefully after washing.
• Avoid potential sources of injury, however small (scratches, insect bites, thorns, sharp objects, etc.) and disinfect any wounds quickly⁽⁹⁾. Skin care aimed at preventing infectious episodes must therefore be carried out: disinfection of all wounds, treatment of interdigital mycosis, etc. 
• Other precautions to reduce the risk of new oedema include avoiding sources of heat and being overweight⁽⁹⁾. 

If you have any questions, ask your doctor for advice.