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Lymphedema in Child
See also in: Cellulitis DDx
Other Resources UpToDate PubMed

Lymphedema in Child

See also in: Cellulitis DDx
Contributors: Nnenna Agim MD, Ayman Grada MD, MS, Vivian Wong MD, PhD, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

Lymphedema refers to a localized form of tissue swelling caused by excessive retention of lymphatic fluid in the interstitial spaces.

Normally, most of the interstitial fluid is reabsorbed via the venous microcirculation and returns to systemic circulation. The remainder (approximately 10%) has a relatively high protein concentration and is drained by blind-ended lymphatic capillaries. Once the protein-rich interstitial fluid enters the lymphatic system, it is referred to as lymph. Impaired lymphatic drainage causes buildup of lymph in the interstitial spaces, leading to tissue swelling.

It has been reported that lymphedema affects as many as 200 million people worldwide and approximately 3 million people in the United States. Lymphedema affects females more often than males.

Lymphedema may be primary, referring to a developmental abnormality of the lymphatic system, or secondary (acquired). Primary lymphedema is rare, affecting approximately 1 in 100 000 children. It may rarely be associated with other syndromes such as Turner syndrome, Noonan syndrome, and yellow nail syndrome.

Based on the age of onset, primary lymphedema can be subdivided into 3 categories: congenital lymphedema (presenting at or shortly after birth), lymphedema praecox (occurring around puberty or shortly thereafter), and lymphedema tarda (starting late in life). Lymphedema praecox is the most common type of primary lymphedema.

Secondary, or acquired, lymphedema is much more common. It results from damage or obstruction of previously normal lymphatics. Filariasis is the most common cause of secondary lymphedema worldwide. Treatment related to cancer is the most common cause of secondary lymphedema in the United States. Major risk factors include axillary lymph node dissection and adjuvant radiation therapy. Other causes include destruction by surgery, radiation, or infection. Secondary lymphedema is typically diagnosed in patients aged 50-58 years.

Lymphedema is a clinical diagnosis. The lower extremities are the most common location. Lymphedema can be unilateral or bilateral. Early-stage lymphedema mimics venous edema (transient pitting edema). Chronic lymphedema is characterized by skin thickening and tissue fibrosis.

Clinical Staging of Lymphedema:
Based on clinical findings in a physical examination, the International Society of Lymphology (ISL) developed a staging system that divides lymphedema into 4 stages:
  • Stage 0 (subclinical) – Swelling is not evident. Heaviness and discomfort.
  • Stage 1 (spontaneously reversible) – Swelling relieved by limb elevation. Includes pitting edema.
  • Stage 2 (spontaneously irreversible) – Swelling not improved by limb elevation. May or may not include pitting edema.
  • Stage 3 (lymphostatic elephantiasis) – Swelling not improved by limb elevation. Skin hardening, nonpitting edema, verrucous changes, and recurrent soft tissue infections.
Complications of chronic lymphedema include predisposition to cellulitis, recurrent cellulitis, and ulceration. Lymphangiosarcoma (Stewart-Treves syndrome) is a rare yet serious complication of long-standing chronic lymphedema. It is usually seen in adults and is associated with radical mastectomy. It is exceedingly rare in children, with only 4 cases reported to date. Additionally, it arises exceedingly rarely in the setting of congenital lymphedema, with only 24 cases reported to date.

Codes

ICD10CM:
I89.0 – Lymphedema, not elsewhere classified

SNOMEDCT:
234097001 – Lymphedema

Look For

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

For the differential diagnosis of syndrome-associated lymphedema, see congenital lymphedema.

Conditions that may mimic lymphedema:
  • Obesity
  • Chronic venous insufficiency (CVI) – Improves with elevation; usually spares the feet. Presence of typical varicose veins. CVI may coexist with lymphedema.
  • Drug-induced edema
  • Lipedema – Usually bilateral; tender; usually spares the feet.
  • Hypoalbuminemia
  • Generalized myxedema and pretibial myxedema – Nonpitting edema.
Kaposi sarcoma may have concomitant lymphedema as a presenting feature.

Best Tests

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Management Pearls

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Therapy

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Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

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References

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Last Reviewed:08/11/2020
Last Updated:09/21/2020
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Lymphedema in Child
See also in: Cellulitis DDx
Lymphedema : Hand edema, Lower extremity edema, Foot edema, Upper extremity edema
Clinical image of Lymphedema
Marked edema on the right arm and hand following radiation treatment of the axilla.
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