Lymphedema in Child
See also in: Cellulitis DDxAlerts and Notices
Synopsis
Secondary lymphedema is usually the result of filariasis, compression of lymphatic structures by a neoplasm, or destruction by surgery, radiation, or infection. Secondary lymphedema is typically diagnosed between 50 and 58 years of age.
In the United States and other developed nations, lymphedema most commonly occurs following breast cancer treatment. Major risk factors include axillary lymph-node dissection and adjuvant radiation therapy.
Lymphedema often presents clinically as painless swelling of an extremity that may grow over time to elephantiasic size. A lower extremity is the most common location. Elephantiasis nostras verrucosa refers to a rare group of cutaneous changes comprising dermal fibrosis and hyperkeratotic, verrucous, and papillomatous lesions arising after chronic secondary (nonfilarial) lymphedema.
Many types of lymphedema are more common in women.
Lymphedema is a chronic ailment that is generally incurable. Treatment is supportive. Lymphedematous limbs are often complicated by infection (eg, cellulitis, onychomycosis) and have considerable impact on quality of life (ie, impede daily activities, create negative self-esteem). The incidence of cellulitis in children with primary lymphedema begins early in life, with a median age of 11.5 years at first episode.
Various syndromes are associated with primary lymphedema such as Turner syndrome, Noonan syndrome, and yellow nail syndrome.
Codes
ICD10CM:I89.0 – Lymphedema, not elsewhere classified
SNOMEDCT:
234097001 – Lymphedema
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Diagnostic Pearls
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Differential Diagnosis & Pitfalls
Underlying causes of chronic lymphedema:- Filariasis
- Milroy disease
- Leprosy
- Radiation injury
- Obesity
- Tuberculosis
- Chronic pyogenic infection
- Postoperative (lymph node dissection such as after breast cancer)
- Lymphedema of malignancy (ie, Kaposi sarcoma, lymphoma)
- Cellulitis – The swelling of lymphedema is often more striking than that of cellulitis. Erythema is a consistent feature of cellulitis, but in lymphedema, it may or may not be present. Lymphedema predisposes to infection, so the 2 conditions may exist simultaneously.
- Angioedema
- Congestive heart failure
- Renal insufficiency
- Hepatic insufficiency
- Malignancy obstructing venous return (superior vena cava syndrome)
- Deep vein thrombosis
- Drugs (eg, corticosteroids, calcium channel blockers, NSAIDs, estrogens and progesterones, thiazolidinediones, cytokines, chemotherapies, acyclovir, phenothiazine, and trazodone)
- Myxedema
- Pulmonary hypertension
- Protein losing enteropathy
- Malabsorption / malnutrition
- Nephrotic syndrome
- Reflex sympathetic dystrophy
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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.Subscription Required
References
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Last Reviewed: 12/14/2017
Last Updated: 12/05/2018
Last Updated: 12/05/2018