Lymphedema

Lymphedema: Excerpt from The 5-Minute Pediatric Consult

Laura N. Sinai, MD

Lymphedema - BASICS

Lymphedema - description

• Accumulation of interstitial fluid in part of the body (usually an extremity) secondary to malformation or malfunction of the lymphatic system
• Divided into primary and secondary forms (although other classification tools exist):
  • Primary: Usually an isolated finding, thought to result from abnormal development of the lymphatics. Deep tissues and muscles not affected. Primary lymphedema often divided into:
    • Congenital lymphedema: Presenting in the 1st few months of life
    • Lymphedema praecox: Presenting from late in 1st year of life to early adulthood (20–35 years of age)
    • Lymphedema tarda: Presenting in adulthood (after 20–35 years of life)
  • Secondary: The etiology of lymphatic damage has been identified; causes include surgery, neoplasm (primary or metastatic), parasitic infestation, infection, irradiation, and trauma.

Lymphedema - epidemiology

• Most lymphedema in childhood is primary lymphedema.
• Congenital lymphedema comprises 10% of primary lymphedema cases; lymphedema praecox, 71%; and lymphedema tarda, 19%.
• Female: Male ratio in congenital lymphedema is close to 1:1; in lymphedema praecox and tarda the female:male ratio is, 2:1–9:1.
• Case reports of recurrent lymphedema secondary to herpes simplex virus 2 exist.

Lymphedema - incidence

Incidence of 1.5 per 100,000 in children <20 years.

Lymphedema - risk factors

Lymphedema - genetics

• In 1998, a defect in the long arm of chromosome 5 (5q35) was identified as causing dominantly inherited congenital lymphedema. Subsequently, additional genetic and endothelial receptor mutations have been identified.
• Genetic disorders are associated with lymphedema: Fabry disease, Milroy (congenital familial lymphedema with ocular findings), Meige disease (familial lymphedema praecox), and Down, Turner, Noonan, yellow nail, Klippel-Trenaunay-Weber, pes cavus, and other syndromes.
• Inheritance can be autosomal dominant, recessive, or sex linked.
• No increased rate of congenital anomalies of other organ systems, but may be associated with other vascular malformations

Lymphedema - pathophysiology

• Fluid accumulation in the interstitial space is secondary to an imbalance in capillary blood and oncotic pressures, capillary permeability, tissue pressures and lymphatic flow. Primary and secondary lymphedema are largely due to inadequate lymph drainage.
• Initially edema is pitting. Chronic edema may result in fibrosis.

Lymphedema - DIAGNOSIS

Lymphedema - signs & symptoms

Lymphedema - history

• Many report prior history of cellulitis or trauma of the limb later affected with lymphedema.
• Unilateral edema: Subacute or chronic painless swelling of one leg in an otherwise healthy pubertal female is classic for lymphedema praecox.
• Painless swelling in an extremity distal to surgical or trauma site is common for secondary lymphedema.

Lymphedema - physical exam

• Subcutaneous tissue filled with fluid resulting in pitting edema. Involvement of single limb strongly suggests lymphedema, whereas global involvement suggests other disease states.
• Chronic inflammation leads to fibrosis, which corresponds to a change from pitting to nonpitting edema and induration.
• Hair loss and hyperkeratosis of the affected limb develop over time.
• Pain in affected limb uncommon except when lymphedema is secondary to thrombophlebitis, cellulitis or reflex sympathetic dystrophy.

Lymphedema - tests

Lymphedema - lab

• Urinalysis for the presence of protein: Proteinuria should raise the suspicion of glomerulonephrosis.
• Serum total protein and albumin: If low, suggests hypoproteinemia, which can result in edema
• Liver function tests to assess functional status
• Pregnancy test, if appropriate for sexual maturity rating

Lymphedema - imaging

• Ultrasound, CT scan, or MRI of pelvis: Useful when evaluating lower extremity edema, as obstructive pelvic lesions may be otherwise inapparent
• MRI is also useful to differentiate between primary lymphedema and other causes of edema such as lipolymphedema and phlebolymphedema.
• Doppler ultrasound: Helpful when deep venous thrombosis is a diagnostic possibility
• Radionuclide lymphangioscintigraphy:
  • Has largely replaced lymphangiography
  • When history and physical examination yield a diagnosis of uncomplicated primary lymphedema in childhood, it is controversial as to whether or not this test is necessary; in straightforward cases, most feel it adds little to the diagnosis and treatment.
• Venography and biopsy: Considered unnecessary for diagnosis

Lymphedema - differencial diagnosis

• Infection:
  • Cellulitis
  • Lymphangitis
  • Herpes simplex virus type 2
• Tumors:
  • Pelvic mass
  • Multiple enchondromatosis
• Metabolic:
  • Cushing disease
  • Hyperthyroidism
  • Lipedema
• Anatomic:
  • Venous stasis
  • Deep venous thrombosis
  • Hemihypertrophy
  • Arterio-venous fistula
  • Popliteal arterial aneurysm
  • Popliteal cyst (Baker cyst)
• Miscellaneous:
  • Heart failure
  • Glomerulonephrosis
  • Cirrhosis
  • Hypoproteinemia
  • Reflex sympathetic dystrophy
  • Pregnancy

Lymphedema - TREATMENT

Lymphedema - general measures

• Therapy should be instituted as soon as possible and before fibrosis develops.
• Goals: Maintain or possibly decrease status of swelling, decrease risk of infections, minimize skin changes.
• Extremity elevation and compression (e.g., Jobst stocking, Ace wrap) recommended long-term for all patients
• Meticulous skin care
• Pneumatic machines or manual massage: Short-term recommendation to achieve greatest reduction of edema
• Cellulitis and lymphangitis treated with hospitalization and intravenous antibiotics

Lymphedema - diet

In children with chylous reflux syndromes, a diet low in long-chain triglycerides may be of benefit.

Lymphedema - activity

Exercise (walking and/or swimming) is thought to enhance lymphatic return.

Lymphedema - special therapy

Complete decongestive physiotherapy is a comprehensive treatment program offered at a few selected centers, but seems to be effective in reducing swelling and maintaining reduction in fluid accumulation.

Lymphedema - medication

• Diuretics: Not used in children and adolescents; efficacy debated
• Prophylactic antibiotic use is indicated for patients with recurrent cellulitis or lymphangitis.

Lymphedema - surgery

• Surgery has 1 of 2 goals: Removal of excess edematous tissue or attempts to restore lymph drainage:
  • Both may decrease the rate of infections but have poor cosmetic results.
  • Recommended only for those with uncontrolled swelling with significant disability
• Microsurgical treatment has undergone many advances with excellent outcome in carefully selected patient populations.

Lymphedema - FOLLOW UP

• Some edema reduction achieved with pneumatic compression or massage over short term, but persistent use of elevation and compression stockings necessary to maintain benefit.
• Signs to watch for:
  • Fever, chills, red streaks on the extremity, inflamed lymph nodes all point to cellulitis or lymphangitis.
• Prevention:
  • Primary disease cannot be prevented, but progression should be minimized with therapies listed here.
  • Secondary disease can be prevented by altering surgical approaches to diseases that emphasize sparing of lymphatics and nodes.
  • Skin care and properly fitted shoes must be emphasized to avoid skin breakdown, which can lead to cellulitis and/or lymphangitis.

• Systemic reactions to lymphangiography are not uncommon.
• Compliance with compression stockings is poor because they are often hot and uncomfortable.

Lymphedema - prognosis

• Edema persists throughout life.
• Natural history: Plateau in severity of edema after an initial few years of progression in 50%, slow constant progression in 50%
• Edema of contralateral extremity (usually leg) develops in up to 10%.
• Chronic inflammation and edema ultimately lead to fibrosis and induration of the involved area.

Lymphedema - complications

• Cellulitis
• Lymphangitis
• Lymphangiosarcoma
• Psychologic problems
• Physical limitations
• Fibrosis

Lymphedema - bibliography

1. Campisi C, Boccardo F, Zilli A, et al. Peripheral lymphedema: New advances in microsurgical treatment and long-term outcome. Microsurgery. 2003;23:522–526.
2. Greenlee R, Hoyme H, Witte M, et al. Developmental disorders of the lymphatic system. Lymphology. 1993;26:156–168.
3. International Society of Lymphology. The diagnosis and treatment of peripheral lymphedema. Consensus document of the International Society of Lymphology. Lymphology. 2003;36:84–91.
4. Johansson K, Albertsson M, Ingvar C, et al. Effects of compression bandaging with or without manual lymph drainage treatment in patients with postoperative arm lymphedema. Lymphology. 1999;32:103–110.
5. Ko DSC, Lerner R, Klose G, et al. Effective treatment of lymphedema of the extremities. Arch Surg. 1998;133:452–458.
6. Miller AJ, Bruna J, Beninson J. A universally applicable clinical classification of lymphedema. Angiology. 1999;50:189–192.
7. Smeltzer DM, Stickler GB, Schirger A. Primary lymphedema in children and adolescents: A follow-up study and review. Pediatrics. 1985;76:206–218.

Lymphedema - CODES

Lymphedema - icd9

• 457.0 Hereditary edema of the legs
• 457.1 Other lymphedema acquired (chronic) praecox secondary

Lymphedema - FAQ

• Q: Is the swelling going to go away?
• A: No, this is a lifelong disorder in most cases.
• Q: Could this have been prevented?
• A: No, primary lymphedema is most likely owing to abnormal embryologic development.
• Q: If the lymph channels have been abnormal since birth, why does the swelling present during adolescence?
• A: No one really knows; hormones may affect lymphedema.

Book Source Details

• Book Title: The 5-Minute Pediatric Consult
• Author(s): M. William Schwartz MD; et al.
• Year of Publication: 2008
• Copyright Details: The 5-Minute Pediatric Consult, Copyright © 2008 Lippincott Williams & Wilkins.

More About Lymphedema

• Back to disease: Lymphedema: Introduction
• Next Book Extract About Lymphedema: Surveys relating to Lymphedema
• More Book Extracts: All Online Books for Lymphedema

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Copyright notice for book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All rights reserved.

More About This Book: Title: The 5-Minute Pediatric Consult Authors: M. William Schwartz MD; et al. Publisher: Lippincott Williams & Wilkins Copyright: 2008 ISBN: 0-7817-7577-9

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