Highlights & Basics
- Lymphedema is a chronic, progressive swelling of tissue with protein-rich fluid as a consequence of developmental (primary lymphedema) or acquired (secondary lymphedema) disruption of the lymphatic system. Extremities are most commonly affected, followed by genitalia.
- Most cases are secondary to nematode infection (filariasis), malignancy, or cancer-related treatment.
- Typically presents with painless unilateral limb swelling; pitting edema is present in early disease, whereas nonpitting edema is a sensitive but nonspecific finding in advanced disease.
- Diagnosis is usually made on clinical grounds and confirmed by lymphoscintigraphy.
- First-line treatment involves compression, ranging from static garments to complex massage and pneumatic compression devices. Surgical procedures are reserved for patients refractory to conservative measures and/or with significant morbidity.
Quick Reference
History & Exam
Key Factors
Other Factors
Diagnostics Tests
Treatment Options
Definition
Epidemiology
Etiology
Pathophysiology
Images
Primary lymphedema of the right lower extremity
Secondary lymphedema: left lower extremity lymphedema after radiation, and lymphadenectomy for Hodgkin lymphoma
Lymphoscintigraphy showing dermal back flow and absent uptake of radiolabeled colloid in lymph nodes of left lower extremity consistent with lymphedema
Citations
Brix B, Sery O, Onorato A, et al. Biology of lymphedema. Biology (Basel). 2021 Mar 25;10(4):261.[Abstract][Full Text]
Manrique OJ, Bustos SS, Ciudad P, et al. Overview of lymphedema for physicians and other clinicians: a review of fundamental concepts. Mayo Clin Proc. 2020 Aug 20:S0025-6196(20)30033-1.[Abstract][Full Text]
Executive Committee of the International Society of Lymphology. The diagnosis and treatment of peripheral lymphedema: 2020 Consensus Document of the International Society of Lymphology. Lymphology. 2020;53(1):3-19.[Abstract][Full Text]
Huang TW, Tseng SH, Lin CC, et al. Effects of manual lymphatic drainage on breast cancer-related lymphedema: a systematic review and meta-analysis of randomized controlled trials. World J Surg Oncol. 2013 Jan 24;11:15.[Abstract][Full Text]
Nagase T, Gonda K, Inoue K, et al. Treatment of lymphedema with lymphaticovenular anastomoses. Int J Clin Oncol. 2005 Oct;10(5):304-10.[Abstract]
1. Brouillard P, Witte MH, Erickson RP, et al. Primary lymphoedema. Nat Rev Dis Primers. 2021 Oct 21;7(1):77.[Abstract]
2. Schook CC, Mulliken JB, Fishman SJ, et al. Primary lymphedema: clinical features and management in 138 pediatric patients. Plast Reconstr Surg. 2011 Jun;127(6):2419-31.[Abstract]
3. Maclellan RA, Greene AK. Lymphedema. Semin Pediatr Surg. 2014 Aug;23(4):191-7.[Abstract]
4. Brix B, Sery O, Onorato A, et al. Biology of lymphedema. Biology (Basel). 2021 Mar 25;10(4):261.[Abstract][Full Text]
5. Lopez M, Roberson ML, Strassle PD, et al. Epidemiology of lymphedema-related admissions in the United States: 2012-2017. Surg Oncol. 2020 Dec;35:249-53.[Abstract]
6. Douglass J, Kelly-Hope L. Comparison of staging systems to assess lymphedema caused by cancer therapies, lymphatic filariasis, and podoconiosis. Lymphat Res Biol. 2019 Oct;17(5):550-6.[Abstract][Full Text]
7. Ramaiah KD, Ottesen EA. Progress and impact of 13 years of the global programme to eliminate lymphatic filariasis on reducing the burden of filarial disease. PLoS Negl Trop Dis. 2014 Nov 20;8(11):e3319.[Abstract][Full Text]
8. Manrique OJ, Bustos SS, Ciudad P, et al. Overview of lymphedema for physicians and other clinicians: a review of fundamental concepts. Mayo Clin Proc. 2020 Aug 20:S0025-6196(20)30033-1.[Abstract][Full Text]
9. Bernas M, Thiadens SRJ, Stewart P, et al. Secondary lymphedema from cancer therapy. Clin Exp Metastasis. 2021 May 5.[Abstract]
10. Rockson SG, Keeley V, Kilbreath S, et al. Cancer-associated secondary lymphoedema. Nat Rev Dis Primers. 2019 Mar 28;5(1):22.[Abstract]
11. Singh B, Disipio T, Peake J, et al. Systematic review and meta-analysis of the effects of exercise for those with cancer-related lymphedema. Arch Phys Med Rehabil. 2016 Feb;97(2):302-15.e13.[Abstract]
12. Rockson SG, Rivera KK. Estimating the population burden of lymphedema. Ann NY Acad Sci. 2008;1131:147-54.[Abstract]
13. McLaughlin SA, Wright MJ, Morris KT, et al. Prevalence of lymphedema in women with breast cancer 5 years after sentinel lymph node biopsy or axillary dissection: objective measurements. J Clin Oncol. 2008 Nov 10;26(32):5213-9.[Abstract]
14. Smeltzer DM, Stickler GB, Schirger A. Primary lymphedema in children and adolescents: a follow-up study and review. Pediatrics. 1985 Aug;76(2):206-18.[Abstract]
15. Goss JA, Maclellan RA, Greene AK. Adult-onset primary lymphedema: a clinical-lymphoscintigraphic study of 26 patients. Lymphat Res Biol. 2019 Dec;17(6):620-3.[Abstract]
16. Slavin SA, Greene AK, Borud LJ. Lymphedema. In: Weinzweig J, ed. Plastic surgery secrets plus. 2nd ed. Philadelphia, PA: Mosby; 2009.
17. Palumbo E. Filariasis: diagnosis, treatment and prevention. Acta Biomed. 2008 Aug;79(2):106-9.[Abstract]
18. WHO Fact sheet. Lymphatic filariasis. 18 May 2021 [internet publication].[Full Text]
19. Wynd S, Melrose WD, Durrheim DN, et al. Understanding the community impact of lymphatic filariasis: a review of the sociocultural literature. Bull World Health Organ. 2007 Jun;85(6):493-8.[Abstract][Full Text]
20. McDougal WS. Lymphedema of the external genitalia. J Urol. 2003 Sep;170(3):711-6.[Abstract]
21. Warren AG, Slavin SA. Scar lymphedema: fact or fiction? Ann Plast Surg. 2007 Jul;59(1):41-5.[Abstract]
22. Telinius N, Hjortdal VE. Role of the lymphatic vasculature in cardiovascular medicine. Heart. 2019 Dec;105(23):1777-84.[Abstract]
23. Irrthum A, Karkkainen MJ, Devriendt K, et al. Congenital hereditary lymphedema caused by a mutation that inactivates VEGFR3 tyrosine kinase. Am J Hum Genet. 2000 Aug;67(2):295-301.[Abstract][Full Text]
24. Irrthum A, Devriendt K, Chitayat D, et al. Mutations in the transcription factor gene SOX18 underlie recessive and dominant forms of hypotrichosis-lymphedema-telangiectasia. Am J Hum Genet. 2003 Jun;72(6):1470-8.[Abstract][Full Text]
25. Finegold DN, Kimak MA, Lawrence EC, et al. Truncating mutations in FOXC2 cause multiple lymphedema syndromes. Hum Mol Genet. 2001 May 15;10(11):1185-9.[Abstract][Full Text]
26. Boon LM, Ballieux F, Vikkula M. Pathogenesis of vascular anomalies. Clin Plast Surg. 2011 Jan;38(1):7-19.[Abstract][Full Text]
27. Alders M, Hogan BM, Gjini E, et al. Mutations in CCBE1 cause generalized lymph vessel dysplasia in humans. Nat Genet. 2009 Dec;41(12):1272-4.[Abstract]
28. Mortimer PS, Rockson SG. New developments in clinical aspects of lymphatic disease. J Clin Invest. 2014 Mar;124(3):915-21.[Abstract][Full Text]
29. Keast DH, Despatis M, Allen JO, et al. Chronic oedema/lymphoedema: under-recognised and under-treated. Int Wound J. 2015 Jun;12(3):328-33.[Abstract][Full Text]
30. Williams AF, Franks PJ, Moffatt CJ. Lymphoedema: estimating the size of the problem. Palliat Med. 2005 Jun;19(4):300-13.[Abstract]
31. Centres for Disease Control and Prevention. Lymphatic filariasis. 22 Oct 2018 [internet publication].[Full Text]
32. Kissin MW, Querci della Rovere G, Easton D, et al. Risk of lymphoedema following the treatment of breast cancer. Br J Surg. 1986 Jul;73(7):580-4.[Abstract]
33. Cemal Y, Pusic A, Mehrara BJ. Preventative measures for lymphedema: separating fact from fiction. J Am Coll Surg. 2011 Oct;213(4):543-51.[Abstract]
34. Rockson SG. Lymphedema after breast cancer treatment. N Engl J Med. 2018 Nov 15;379(20):1937-44.[Abstract]
35. Greene AK, Grant FD, Slavin SA. Lower-extremity lymphedema and elevated body-mass index. N Engl J Med. 2012 May 31;366(22):2136-7.[Abstract][Full Text]
36. Greene AK, Grant FD, Slavin SA, et al. Obesity-induced lymphedema: clinical and lymphoscintigraphic features. Plast Reconstr Surg. 2015 Jun;135(6):1715-9.[Abstract]
37. Greene AK. Diagnosis and management of obesity-induced lymphedema. Plast Reconstr Surg. 2016 Jul;138(1):111e-8e.[Abstract]
38. Greene AK, Grant FD, Maclellan RA. Obesity-induced lymphedema nonreversible following massive weight loss. Plast Reconstr Surg Glob Open. 2015 Jul 8;3(6):e426.[Abstract][Full Text]
39. Maclellan RA, Zurakowski D, Grant FD, et al. Massive localized lymphedema: a case-control study. J Am Coll Surg. 2017 Feb;224(2):212-6.[Abstract]
40. McLaughlin SA, Wright MJ, Morris KT, et al. Prevalence of lymphedema in women with breast cancer 5 years after sentinel lymph node biopsy or axillary dissection: objective measurements. J Clin Oncol. 2008 Nov 10;26(32):5213-9.[Abstract]
41. Mansel RE, Fallowfield L, Kissin M, et al. Randomized multicenter trial of sentinel node biopsy versus standard axillary treatment in operable breast cancer: the ALMANAC Trial. J Natl Cancer Inst. 2006 May 3;98(9):599-609.[Abstract][Full Text]
42. Stemmer R. A clinical symptom for the early and differential diagnosis of lymphedema [in German]. Vasa. 1976;5(3):261-2.[Abstract]
43. Brorson H, Svensson H. Liposuction combined with controlled compression therapy reduces arm lymphedema more effectively than controlled compression therapy alone. Plast Reconstr Surg. 1998 Sep;102(4):1058-67.[Abstract]
44. Warren AG, Janz BA, Slavin SA, et al. The use of bioimpedance analysis to evaluate lymphedema. Ann Plast Surg. 2007 May;58(5):541-3.[Abstract]
45. Ward LC, Dylke E, Czerniec S, et al. Confirmation of the reference impedance ratios used for assessment of breast cancer-related lymphedema by bioelectrical impedance spectroscopy. Lymphat Res Biol. 2011 Mar;9(1):47-51.[Abstract]
46. Jain MS, Danoff JV, Paul SM. Correlation between bioelectrical spectroscopy and perometry in assessment of upper extremity swelling. Lymphology. 2010 Jun;43(2):85-94.[Abstract]
47. Executive Committee of the International Society of Lymphology. The diagnosis and treatment of peripheral lymphedema: 2020 Consensus Document of the International Society of Lymphology. Lymphology. 2020;53(1):3-19.[Abstract][Full Text]
48. Hassanein AH, Maclellan RA, Grant FD, et al. Diagnostic accuracy of lymphoscintigraphy for lymphedema and analysis of false-negative tests. Plast Reconstr Surg Glob Open. 2017 Jul 12;5(7):e1396.[Abstract][Full Text]
49. Szuba S, Rockson SG. Lymphedema: classification, diagnosis and therapy. Vasc Med. 1998;3(2):145-56.[Abstract]
50. Slavin SA, Van den Abbeele A, Losken A, et al. Return of lymphatic function after flap transfer for acute lymphedema. Ann Surg. 1999 Mar;229(3):421-7.[Abstract][Full Text]
51. Maclellan RA, Zurakowski D, Voss S, et al. Correlation between lymphedema disease severity and lymphoscintigraphic findings: a clinical-radiologic study. J Am Coll Surg. 2017 Sep;225(3):366-70.[Abstract]
52. Warren AG, Brorson H, Borud LJ, et al. Lymphedema: a comprehensive review. Ann Plast Surg. 2007 Oct;59(4):464-72.[Abstract]
53. Warren AG, Janz BA, Borud LJ, et al. Evaluation and management of the fat leg syndrome. Plast Reconstr Surg. 2007 Jan;119(1):9e-15e.[Abstract]
54. Rudkin GH, Miller TA. Lipedema: a clinical entity distinct from lymphedema. Plast Reconstr Surg. 1994 Nov;94(6):841-7.[Abstract]
55. Moseley AL, Carati CJ, Piller NB. A systematic review of common conservative therapies for arm lymphoedema secondary to breast cancer treatment. Ann Oncol. 2007 Apr;18(4):639-46.[Abstract][Full Text]
56. Schmitz KH, Ahmed RL, Troxel A, et al. Weight lifting in women with breast-cancer-related lymphedema. N Engl J Med. 2009 Aug 13;361(7):664-73.[Abstract][Full Text]
57. Singh B, Disipio T, Peake J, et al. Systematic review and meta-analysis of the effects of exercise for those with cancer-related lymphedema. Arch Phys Med Rehabil. 2016 Feb;97(2):302-15.e13.[Abstract]
58. Huang TW, Tseng SH, Lin CC, et al. Effects of manual lymphatic drainage on breast cancer-related lymphedema: a systematic review and meta-analysis of randomized controlled trials. World J Surg Oncol. 2013 Jan 24;11:15.[Abstract][Full Text]
59. Morgan PA, Franks PJ, Moffatt CJ. Health-related quality of life with lymphoedema: a review of the literature. Int Wound J. 2005 Mar;2(1):47-62.[Abstract]
60. Macfarlane CL, Budhathoki SS, Johnson S, et al. Albendazole alone or in combination with microfilaricidal drugs for lymphatic filariasis. Cochrane Database Syst Rev. 2019 Jan 8;1(1):CD003753.[Abstract][Full Text]
61. Cormier JN, Rourke L, Crosby M, et al. The surgical treatment of lymphedema: a systematic review of the contemporary literature (2004-2010). Ann Surg Oncol. 2012 Feb;19(2):642-51.[Abstract]
62. Greene AK, Slavin SA, Borud L. Treatment of lower extremity lymphedema with suction-assisted lipectomy. Plast Reconstr Surg. 2006 Oct;118(5):118e-21e.[Abstract]
63. Greene AK, Maclellan RA. Operative treatment of lymphedema using suction-assisted lipectomy. Ann Plast Surg. 2016 Sep;77(3):337-40.[Abstract]
64. Brorson H, Ohlin K, Olsson G, et al. Controlled compression and liposuction treatment for lower extremity lymphedema. Lymphology. 2008 Jun;41(2):52-63.[Abstract]
65. Brorson H. Liposuction in arm lymphedema treatment. Scand J Surg. 2003;92(4):287-95.[Abstract]
66. Brorson H, Svensson H. Skin blood flow of the lymphedematous arm before and after liposuction. Lymphology. 1997 Dec;30(4):165-72.[Abstract]
67. Greene AK, Voss SD, Maclellan RA. Liposuction for swelling in patients with lymphedema. N Engl J Med. 2017 Nov 2;377(18):1788-9.[Abstract][Full Text]
68. Miller TA, Wyatt LE, Rudkin GH. Staged skin and subcutaneous excision for lymphedema: a favorable report of long-term results. Plast Reconstr Surg. 1998 Oct;102(5):1486-98.[Abstract]
69. Slavin SA, Upton J, Kaplan WD, et al. An investigation of lymphatic function following free-tissue transfer. Plast Reconstr Surg. 1997 Mar;99(3):730-41.[Abstract]
70. Baumeister RG, Siuda S. Treatment of lymphedemas by microsurgical lymphatic grafting: what is proved? Plast Reconstr Surg. 1990 Jan;85(1):64-74.[Abstract]
71. O'Brien BM, Mellow CG, Khazanchi RK, et al. Long-term results after microlymphaticovenous anastomoses for the treatment of obstructive lymphedema. Plast Reconstr Surg. 1990 Apr;85(4):562-72.[Abstract]
72. Becker C, Assouad J, Riquet M, et al. Postmastectomy lymphedema: long-term results following microsurgical lymph node transplantation. Ann Surg. 2006 Mar;243(3):313-5.[Abstract]
73. Campisi C, Eretta C, Pertile D, et al. Microsurgery for treatment of peripheral lymphedema: long-term outcome and future perspectives. Microsurgery. 2007;27(4):333-8.[Abstract]
74. Chang DW. Lymphaticovenular bypass for lymphedema management in breast cancer patients: a prospective study. Plast Reconstr Surg. 2010 Sep;126(3):752-8.[Abstract]
75. Lin CH, Ali R, Chen SC, et al. Vascularized groin lymph node transfer using the wrist as a recipient site for management of postmastectomy upper extremity lymphedema. Plast Reconstr Surg. 2009 Apr;123(4):1265-75.[Abstract]
76. Nagase T, Gonda K, Inoue K, et al. Treatment of lymphedema with lymphaticovenular anastomoses. Int J Clin Oncol. 2005 Oct;10(5):304-10.[Abstract]
77. Parrett BM, Sepic J, Pribaz JJ. The contralateral rectus abdominis musculocutaneous flap for treatment of lower extremity lymphedema. Ann Plast Surg. 2009 Jan;62(1):75-9.[Abstract]
78. Tourani SS, Taylor GI, Ashton MW. Vascularized lymph node transfer: a review of the current evidence. Plast Reconstr Surg. 2016 Mar;137(3):985-93.[Abstract]
79. Sulo E, Hartiala P, Viitanen T, et al. Risk of donor-site lymphatic vessel dysfunction after microvascular lymph node transfer. J Plast Reconstr Aesthet Surg. 2015 Apr;68(4):551-8.[Abstract]
80. Vignes S, Blanchard M, Yannoutsos A, et al. Complications of autologous lymph-node transplantation for limb lymphoedema. Eur J Vasc Endovasc Surg. 2013 May;45(5):516-20.[Abstract][Full Text]
81. Tiwari A, Cheng KS, Button M, et al. Differential diagnosis, investigation, and current treatment of lower limb lymphedema. Arch Surg. 2003 Feb;138(2):152-61.[Abstract][Full Text]
82. Slavin SA, Greene AK, Borud LJ. Lymphedema. In: Weinzweig J, ed. Plastic surgery secrets plus. 2nd ed. Philadelphia, PA: Mosby; 2009.
83. Aygit AC, Yildirim AM, Dervisoglu S. Lymphangiosarcoma in chronic lymphoedema. Stewart-Treves syndrome. J Hand Surg Br. 1999 Feb;24(1):135-7.[Abstract]
84. Sagen A, Karesen R, Skaane P, et al. Validity for the simplified water displacement instrument to measure arm lymphedema as a result of breast cancer surgery. Arch Phys Med Rehabil. 2009 May;90(5):803-9.[Abstract]
85. Brorson H, Ohlin K, Svensson B. The facts about liposuction as a treatment for lymphoedema. J Lymphoedema. 2008;3(1):38-47.[Full Text]
86. Greene AK, Borud L, Slavin SA. Blood pressure monitoring and venipuncture in the lymphedematous extremity. Plast Reconstr Surg. 2005 Dec;116(7):2058-9.[Abstract]
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