Written by  and originally published here. Click to read part one or part two of this article.

Ongoing measurement and monitoring of Primary and Secondary Lymphedema in Children

As with lymphedema in an adult, lymphedema in children needs to be actively monitored to ensure it is being effectively managed. In adults, lymphedema can be monitored effectively through subjective and objective measures.

Subjective measures of lymphedema include skin firmness and patient reports about how the affected area feels (such as “heaviness” or “tightness”). Objective measures include physical measurement of the area or limb, preferably in comparison to an unaffected area or limb (when possible), as well as hands-on evaluation of the tissue quality for the presence and nature of the swelling, any areas of density or firmness indicating fibrosis, and so on.

Objective measures of lymphedema in children are difficult because children are actively growing, however relative changes in volume compared to an unaffected side of the body (if possible) can be helpful. In many cases, the monitoring of lymphedema in children needs to rely on subjective metrics such as the patient’s experience of their lymphedema, as well as changes in the firmness of the swelling.

Common health complications in children with Primary or Secondary Lymphedema

Unmanaged lymphedema will progress through the stages of lymphedema (see Table 1 above), creating irreversible skin and tissue changes, and associated symptoms. In addition to these changes, children with lymphedema are at risk of secondary complications.

SECONDARY COMPLICATIONS OF LYMPHEDEMA

I) Infection

The lymphatic system plays a key role in monitoring and responding to infection. So it is not surprising that the accumulation of stagnant lymph fluid during lymphedema progression predisposes the patient to infection.

Figure 1: Cellulitis in the left leg of a four-year-old child with lymphedema. Image adapted from Schook C.C., 2011 (ref6).

As a result, infection of the skin and underlying tissues, called cellulitis, is a common complication (for an example, please see Figure 1). For more information on the risk factors for developing cellulitis, see our post: “Risk Factors for Cellulitis in Patients with Lymphedema“.

In three recent studies of primary lymphedema in children, 12.5%-18% of the children were found to have had cellulitis, with many having recurring infection (ref3,5,6). These rates appear to be lower than that reported in adults, suggesting that children may be less prone to developing an infection (ref 3).

Infection within the lymph vessels, called lymphangitis can also occur, but more rarely.

II) Other complications

Figure 2: Upslanting toenails in a child with lymphedema. Image adapted from Vidal F., 2016 (ref5).
  1. Psychological issues including self-consciousness with respect to the swelling, or fear and avoidance of physical activities.
  2. Upslanting toenails were reported in one study to be a frequent complication for children with lymphedema (ref5,6). Approximately one-third of children with leg lymphedema exhibited it. Upslanted toenails can increase the likelihood of pulling off a nail when putting on clothes or compression garments, and along with ingrown toenails, can increase the risk of infection. For an example of upslanting toenails, see Figure 2.
  3. Skin changes including hardening and the formation of small bumps.
  4. Orthopaedic issues such as difficulty with walking in advanced cases of leg lymphedema.
  5. Genital and urinary symptoms for cases of genital lymphedema, such as painful or difficult urination and urethral inflammation.
  6. Lymphorrhea, which is leakage of lymph fluid through openings in the skin, can increase the risk of infection due to the skin breach and the presence of the nutrient-rich lymph fluid acting as a food source for bacteria.
  7. Ulceration. Swelling causes the skin to become stretched and more fragile, increasing the risk of skin ulcers.

Treatment of Primary and Secondary Lymphedema in Children

The difference in the treatment of Lymphedema in children and adults:

The treatment of lymphedema in children is essentially the same as that for adults, with three key modifications necessary to ensure good outcomes (ref10):

  1. Parents and caregivers need to be fully educated on and intimately involved in monitoring and managing the symptoms of lymphedema and secondary complications. They should also encourage the child to practice proper self-management techniques, which they will need to learn as a life skill.
  2. Parents and caregivers need to actively encourage and support child participation in normal physical activities.
  3. Parents and caregivers need to provide strong emotional and psychological support to their child and seek professional help as necessary.

Lymphedema treatment approaches

I) Why can’t we simply elevate a limb to drain out the excess fluid?

In early lymphedema (stage 1) swelling can be partially or fully eliminated by elevating a limb or may be found to be reduced in the morning after sleep. At this stage, the lymphatic system is retaining enough function to sufficiently clear lymph fluid when the total limb fluid load is reduced through the help of gravity and inactivity.

However, if lymphedema remains untreated, the swelling will continue to progress. Eventually, elevation and sleep will have no effect on the swelling. Why is this?

To understand why we need to understand the difference between the cardiovascular system and lymphatic system. The cardiovascular system uses the pumping action of the heart (with assistance from muscle activity and gravity) to push blood through the body.

Instead of having one large heart to do the pumping, the lymphatic system relies primarily on the pumping action of many small fluid chambers that make up the lymphatic vessels. These lymphatic vessels then connect into larger vessels at fluid basins called lymph nodes.

Rather than fluid simply “draining” out of the tissues, the lymphatic system actively, and continuously, sucks up lymphatic fluid.

We rely on the lymphatic system because the venous system alone is incapable of collecting all of this excess fluid that would otherwise accumulate in the tissues. This is why simply raising a lymphedematous arm (which helps venous flow) will not eliminate more advanced cases of lymphedema.

II) Complex Decongestive Therapy

The standard treatment for lymphedema is Complex (or Combined) Decongestive Therapy (CDT), which includes manual lymphatic drainage, compression wrapping and/or compression garments, and education on skin safety and exercise provided by a certified lymphedema therapist.

CDT can be very effective at reducing the volume of swelling, softening areas of fibrosis, and preventing flare-ups. A CDT therapist should also provide guidance to the child and caregiver for self-management, including wrapping and garment use, manual lymphatic drainage exercises, and education on preventing flare-ups and secondary complications, skin care, and other information.

Effective management of lymphedema requires active ongoing self-management, caregiver management or co-management.

One specialized clinic recommended compression bandages be worn as much as possible each day, overnight for children who walk, and 24 hours a day for non-walking children (ref5).

For more information on lymphedema treatment and prevention of lymphedema flare-ups and secondary complications, please see: “Patient Guide to Lymphedema Symptoms, Prevention and Management”.

III) Surgery

Surgical interventions for lymphedema are still relatively new, not well studied, are typically not curative, and may not be widely available. They are also generally only considered for advanced cases that do not respond to Complex Decongestive Therapy.

Surgery in lymphedematous children may be warranted for specific reasons, on a case-by-case basis. Use of surgery in children appears to be more common with genital lymphedema than for those with limb lymphedema (ref6).

Surgical interventions in children may be used to:

  1. Repair damage caused by swelling (such as damage to genitalia caused by a testicular hydrocele).
  2. Temporarily de-bulk advanced cases of hardened lymphedema by direct excision or liposuction of accumulated adipose and fibrotic tissue.
  3. Repair lymphatic damage in secondary lymphedema by a lymphovenous bypass. In this procedure, still healthy lymphatic vessels that have been disrupted or obstructed are connected to very small nearby veins in an attempt to improve lymph drainage.
  4. Repair missing or damaged lymph nodes in secondary lymphedema through lymph node transfer (along with artery and vein transfer) from another site in the body. As you might expect, this procedure has the risk of resulting in donor site lymphedema.

Triggers of swelling flare-up in children with Primary or Secondary LE.

Events that cause local swelling can initiate a flare-up of lymphedema in patients with early stage 1 lymphedema, be it primary or secondary lymphedema.

In stage 1 lymphedema the swelling is spontaneously reversible, meaning that it can come and go on its own. It is also usually reduced or absent in the morning after sleeping, or if the area of the body is elevated.

The same factors that can initiate the onset of asymptomatic primary lymphedema (as we saw above), can also trigger the temporary bouts of increased swelling observed in early lymphedema (stage 1).

Triggers of swelling flare-up in children:

  1. Minor trauma, such as a sprained ankle.
  2. Medical procedures, such as minor surgery.
  3. Insect bites.
  4. Infection. Infection causes inflammation and swelling, and lymphedematous areas of the body are predisposed to infection due to local immune suppression caused by lymph fluid stagnation.
  5. Sedentary periods. Immobility for a prolonged period of time, such as during an aeroplane flight, can cause swelling in the legs.

 

 

References:

  1. Smeltzer D.M., Stickler G.B., Schirger A. Primary lymphedema in children and adolescents: a follow-up study and review. Pediatrics. 1985 Aug;76(2):206-18.
  2. Phillips J.J., Gordon S.J. Conservative management of lymphoedema in children: a systematic review. J Pediatr Rehabil Med. 2014;7(4):361-72.
  3. Todd J., Craig G., Todd M., et al. Audit of childhood lymphoedema in the United Kingdom undertaken by members of the Children’s Lymphoedema Special Interest Group. J of Lymphoedema. 2014;9(2):14-19.
  4. Dale R.F. The inheritance of primary lymphoedema. J Med Genet. 1985;22:274-278.
  5. Vidal F., Arrault M., Vignes S. Pediatric primary lymphoedema: a cohort of 155 children and newborns. Br J Dermatol. 2016 Mar 16. [Epub ahead of print].
  6. Schook C.C., Mulliken J.B., Fishman S.J. et al. Primary lymphedema: clinical features and management in 138 pediatric patients. Plast Reconstr Surg. 2011 Jun;127(6):2419-31.
  7. Wright N.B., Carty H.M. The swollen leg and primary lymphoedema. Arch Dis Child. 1994 Jul;71(1):44-49.
  8. Baulieu F, Vaillant L, Gironet N et al. Intérêt de la lymphoscintigraphie dans l’exploration des lymphoedèmes de l’enfant. J Mal Vasc 2003;28:269–76.
  9. Browse N, Burnand KG, Mortimer PS (2003) Diseases of the Lymphatics. CRC Press, Boca Raton; FL 142–3
  10. Damstra R.J., Mortimer P.S. Diagnosis and therapy in children with lymphoedema. Phlebology. 2008;23(6):276-86.

 

LEAVE A REPLY

Please enter your comment!
Please enter your name here