Archives of Plastic Surgery

Korean Society of Plastic and Reconstructive Surgeons (대한성형외과학회)
권호 표지
DOI QR코드

DOI QR Code

Surgical Treatment for Primary Lymphedema: A Systematic Review of the Literature

  • Miguel Angel Gaxiola-Garcia (Plastic and Reconstructive Surgery Department, Mexico's Children's Hospital (Hospital Infantil de Mexico ) ;
  • Joseph M. Escandon (Division of Plastic and Reconstructive Surgery, Strong Memorial Hospital, University of Rochester Medical Center) ;
  • Oscar J. Manrique (Division of Plastic and Reconstructive Surgery, Strong Memorial Hospital, University of Rochester Medical Center) ;
  • Kristin A. Skinner (Department of Surgical Oncology, Strong Memorial Hospital, University of Rochester Medical Center) ;
  • Beatriz Hatsue Kushida-Contreras (Plastic and Reconstructive Surgery Department, Mexico's General Hospital (Hospital General de Mexico))
  • Received : 2023.09.14
  • Accepted : 2023.11.30
  • Published : 2024.03.15
Download PDF
⟨ Previous Next ⟩

Abstract

This is a retrospective review of surgical management for primary lymphedema. Data were extracted from 55 articles from PubMed MEDLINE, Web of Science, SCOPUS, and Cochrane Central Register of Controlled Trials between the database inception and December 2022 to evaluate the outcomes of lymphaticovenous anastomosis (LVA) and vascularized lymph node transfer (VLNT), and outcomes of soft tissue extirpative procedures such as suction-assisted lipectomy (SAL) and extensive soft tissue excision. Data from 485 patients were compiled; these were treated with LVA (n = 177), VLNT (n = 82), SAL (n = 102), and excisional procedures (n = 124). Improvement of the lower extremity lymphedema index, the quality of life (QoL), and lymphedema symptoms were reported in most studies. LVA and VLNT led to symptomatic relief and improved QoL, reaching up to 90 and 61% average circumference reduction, respectively. Cellulitis reduction was reported in 25 and 40% of LVA and VLNT papers, respectively. The extirpative procedures, used mainly in patients with advanced disease, also led to clinical improvement from the volume reduction, as well as reduced incidence of cellulitis, although with poor cosmetic results; 87.5% of these reports recommended postoperative compression garments. The overall complication rates were 1% for LVA, 13% for VLNT, 11% for SAL, and 46% for extirpative procedures. Altogether, only one paper lacked some kind of improvement. Primary lymphedema is amenable to surgical treatment; the currently performed procedures have effectively improved symptoms and QoL in this population. Complication rates are related to the invasiveness of the chosen procedure.

Keywords

References

  1. Grada AA, Phillips TJ. Lymphedema: pathophysiology and clinical manifestations. J Am Acad Dermatol 2017;77(06):1009-1020 
  2. Schook CC, Mulliken JB, Fishman SJ, Grant FD, Zurakowski D, Greene AK. Primarylymphedema: clinical features andmanagement in 138 pediatric patients. Plast Reconstr Surg 2011;127(06):2419-2431 
  3. Greene AK, Goss JA. Diagnosis and staging of lymphedema. Semin Plast Surg 2018;32(01):12-16 
  4. Allen E. Lymphedema of the extremities. Classification, etiology and differential diagnosis: a study of three hundred cases. Arch Intern Med (Chic) 1934;54(04):606-624 
  5. Smeltzer DM, Stickler GB, Schirger A. Primary lymphedema in children and adolescents: a follow-up study and review. Pediatrics 1985;76(02):206-218 
  6. Kinmonth JB, Taylor GW, Tracy GD, Marsh JD. Primary lymphoedema; clinical and lymphangiographic studies of a series of 107 patients in which the lower limbs were affected. Br J Surg 1957;45(189):1-9 
  7. Van Damme A, Seront E, Dekeuleneer V, Boon LM, Vikkula M. New and emerging targeted therapies for vascular malformations. Am J Clin Dermatol 2020;21(05):657-668 
  8. Brouillard P, Boon L, Vikkula M. Genetics of lymphatic anomalies. J Clin Invest 2014;124(03):898-904 
  9. Connell F, Brice G, Mortimer P. Phenotypic characterization of primary lymphedema. Ann N Y Acad Sci 2008;1131:140-146 
  10. Kinmonth JB, Eustace PW. Lymph nodes and vessels in primary lymphoedema. Their relative importance in aetiology. Ann R Coll Surg Engl 1976;58(04):278-284 
  11. Liu NF, Yan ZX, Wu XF. Classification of lymphatic-system malformations in primary lymphoedema based on MR lymphangiography. Eur J Vasc Endovasc Surg 2012;44(03):345-349 
  12. Wolfe JHN, Kinmonth JB. The prognosis of primary lymphedema of the lower limbs. Arch Surg 1981;116(09):1157-1160 
  13. Murdaca G, Cagnati P, Gulli R, et al. Current views on diagnostic approach and treatment of lymphedema. Am J Med 2012;125(02):134-140 
  14. Koshima I, Inagawa K, Urushibara K, Moriguchi T. Supermicrosurgical lymphaticovenular anastomosis for the treatment of lymphedema in the upper extremities. J Reconstr Microsurg 2000;16(06):437-442 
  15. Hara H, Mihara M, Ohtsu H, Narushima M, Iida T, Koshima I. Indication of lymphaticovenous anastomosis for lower limb primary lymphedema. Plast Reconstr Surg 2015;136(04):883-893 
  16. Cheng MH, Loh CYY, Lin CY. Outcomes of vascularized lymph node transfer and lymphovenous anastomosis for treatment of primary lymphedema. Plast Reconstr Surg Glob Open 2018;6(12):e2056 
  17. Mosti G, Cavezzi A. Compression therapy in lymphedema: between past and recent scientific data. Phlebology 2019;34(08):515-522 
  18. Chang DW, Masia J, Garza R III, Skoracki R, Neligan PC. Lymphedema: Surgical and medical therapy. Plast Reconstr Surg 2016;138(3, Suppl)209S-218S 
  19. Desai SS, Shao MVascular Outcomes Collaborative. Superior clinical, quality of life, functional, and health economic outcomes with pneumatic compression therapy for lymphedema. Ann Vasc Surg 2020;63:298-306 
  20. Becker C, Arrive L, Saaristo A, et al. Surgical treatment of congenital lymphedema. Clin Plast Surg 2012;39(04):377-384 
  21. Ciudad P, Manrique OJ, Bustos SS, et al. Comparisons in long-term clinical outcomes among patients with upper or lower extremity lymphedema treated with diverse vascularized lymph node transfer. Microsurgery 2020;40(02):130-136 
  22. Cheng MH, Liu TTF. Lymphedema microsurgery improved outcomes of pediatric primary extremity lymphedema. Microsurgery 2020;40(07):766-775 
  23. Cheng MH, Chen SC, Henry SL, Tan BK, Chia-Yu Lin M, Huang JJ. Vascularized groin lymph node flap transfer for postmastectomy upper limb lymphedema: flap anatomy, recipient sites, and outcomes. Plast Reconstr Surg 2013;131(06):1286-1298 
  24. Wilting J, Becker J. The lymphatic vascular system: much more than just a sewer. Cell Biosci 2022;12(01):157 
  25. Gianesini S, Rimondi E, Raffetto JD, et al. Human collecting lymphatic glycocalyx identification by electron microscopy and immunohistochemistry. Sci Rep 2023;13(01):3022 
  26. Dellon AL, Hoopes JE. The Charles procedure for primary lymphedema. Long-term clinical results. Plast Reconstr Surg 1977;60(04):589-595 
  27. McKEE DMEM, Edgerton MT Jr. The surgical treatment of lymphedema of the lower extremities. Plast Reconstr Surg Transplant Bull 1959;23(05):480-492 
  28. Brorson H, Ohlin K, Olsson G, Svensson B, Svensson H. Controlled compression and liposuction treatment for lower extremity lymphedema. Lymphology 2008;41(02):52-63 
  29. Boyages J, Kastanias K, Koelmeyer LA, et al. Liposuction for advanced lymphedema: a multidisciplinary approach for complete reduction of arm and leg swelling. Ann Surg Oncol 2015;22(Suppl 3):S1263-S1270 
  30. Vignes S, Albuisson J, Champion L, et al; French National Referral Center for Primary Lymphedema. Primary lymphedema French National Diagnosis and Care Protocol (PNDS; Protocole National de Diagnostic et de Soins). Orphanet J Rare Dis 2021;16(01):18 
  31. Shamseer L, Moher D, Clarke M, et al; PRISMA-P Group. Preferred reporting items for systematic review and meta-analysis protocols (PRISMA-P) 2015: elaboration and explanation. BMJ 2015;350:g7647 
  32. Moher D, Shamseer L, Clarke M, et al; PRISMA-P Group. Preferred reporting items for systematic review and meta-analysis protocols (PRISMA-P) 2015 statement. Syst Rev 2015;4(01):1 
  33. OCEBM Levels of Evidence Working Group. The Oxford Levels of Evidence 1. 2009 
  34. Murad MH, Sultan S, Haffar S, Bazerbachi F. Methodological quality and synthesis of case series and case reports. BMJ Evid Based Med 2018;23(02):60-63. Doi: 10.1136/bmjebm-2017-110853 
  35. Wells G, Shea B, O'Connell D, et al. The Newcastle-Ottawa Scale (NOS) for assessing the quality if nonrandomized studies in meta-analyses. 2012. Accessed September 1st 2023, at: http://www.ohri.ca/programs/clinical_epidemiology/oxford.asp 
  36. MacKmull G, Weeder SD. Congenital lymphedema; case report with results of surgical correction. Plast Reconstr Surg 1950;5(02):157-162 
  37. Fonkalsrud EW. Congenital lymphedema of the extremities in infants and children. J Pediatr Surg 1969;4(02):231-236 
  38. Tilley AR, Douglas LG. Staged treatment of lymphedema praecox. Can Med Assoc J 1974;110(03):309-312 
  39. Feins NR, Rubin R, Crais T, O'Connor JF. Surgical management of thirty-nine children with lymphedema. J Pediatr Surg 1977;12(03):471-476 
  40. Louton RB, Terranova WA. The use of suction curettage as adjunct to the management of lymphedema. Ann Plast Surg 1989;22(04):354-357 
  41. Dumanian GA, Futrell JW. Radical excision and delayed reconstruction of a lymphedematous leg with a 15 year follow-up. Lymphology 1996;29(01):20-24 
  42. Koshima I, Nanba Y, Tsutsui T, Takahashi Y, Itoh S. Long-term follow-up after lymphaticovenular anastomosis for lymphedema in the leg. J Reconstr Microsurg 2003;19(04):209-215 
  43. Fraga MFP, Junior AH, Guedes Neto HJ. Disarticulation of the left upper extremity for treatment of giant primary lymphedema-case report. Lymphology 2004;37(04):199-201 
  44. Hosnuter M, Buyukates M, Babuccu B. An unusual case of lymphedema tarda. Med Sci Monit 2006;12(10):CS99-CS102 
  45. Greene AK, Slavin SA, Borud L. Treatment of lower extremity lymphedema with suction-assisted lipectomy. Plast Reconstr Surg 2006;118(05):118e-121e 
  46. Espinosa-de-Los-Monteros A, Hinojosa CA, Abarca L, Iglesias M. Compression therapy and liposuction of lower legs for bilateral hereditary primary lymphedema praecox. J Vasc Surg 2009;49(01):222-224 
  47. Eryilmaz T, Kaya B, Ozmen S, Kandal S. Suction-assisted lipectomy for treatment of lower-extremity lymphedema. Aesthet Plast Surg 2009;33(04):671-673 
  48. van der Walt JC, Perks TJ, Zeeman BJV, Bruce-Chwatt AJ, Graewe FR. Modified Charles procedure using negative pressure dressings for primary lymphedema: a functional assessment. Ann Plast Surg 2009;62(06):669-675 
  49. Karonidis A, Chen HC. Preservation of toes in advanced lymphedema: an important step in the control of infection. Ann Plast Surg 2010;64(04):446-450 
  50. de Godoy JMP, Azoubel LMO, de Fatima Guerreiro Godoy M. Surgical treatment of elephantiasis of the feet in congenital lymphedema to facilitate the use of a compression mechanism. Int J Gen Med 2010;3:115-118 
  51. Mihara M, Hayashi Y, Murai N, et al. Regional diagnosis of lymphoedema and selection of sites for lymphaticovenular anastomosis using elastography. Clin Radiol 2011;66(08):715-719 
  52. Yamamoto T, Koshima I, Yoshimatsu H, Narushima M, Miahara M, Iida T. Simultaneous multi-site lymphaticovenular anastomoses for primary lower extremity and genital lymphoedema complicated with severe lymphorrhea. J Plast Reconstr Aesthet Surg 2011;64(06):812-81 
  53. Auba C, Marre D, Rodriguez-Losada G, Hontanilla B. Lymphaticovenular anastomoses for lymphedema treatment: 18 months postoperative outcomes. Microsurgery 2012;32(04):261-268 
  54. Suehiro K, Morikage N, Murakami M, Yamashita O, Hamano K. Primary lymphedema complicated by weeping chylous vesicles in the leg and scrotum: report of a case. Surg Today 2012;42(11):1100-1103 
  55. Yamamoto T, Yoshimatsu H, Yamamoto N, Narushima M, Iida T, Koshima I. Side-to-end lymphaticovenular anastomosis through temporary lymphatic expansion. PLoS ONE 2013;8(03):e59523 
  56. Ayestaray B, Bekara F. π-shaped lymphaticovenular anastomosis: the venous flow sparing technique for the treatment of peripheral lymphedema. J Reconstr Microsurg 2014;30(08):551-560 
  57. Gomez Martin C, Murillo C, Maldonado AA, Cristobal L, Fernandez-Canamaque JL. Double autologous lymph node transplantation (ALNT) at the level of the knee and inguinal region for advanced lymphoedema of the lower limb (elephantiasis). J Plast Reconstr Aesthet Surg 2014;67(02):267-270 
  58. Qiu SS, Chen HY, Cheng MH. Vascularized lymph node flap transfer and lymphovenous anastomosis for Klippel-Trenaunay syndrome with congenital lymphedema. Plast Reconstr Surg 2014;134(01):1-5 
  59. Akita S, Mitsukawa N, Kuriyama M, et al. Comparison of vascularized supraclavicular lymph node transfer and lymphaticovenular anastomosis for advanced stage lower extremity lymphedema. Ann Plast Surg 2015;74(05):573-579 
  60. Ito R, Wu CT, Lin MCY, Cheng MH. Successful treatment of early-stage lower extremity lymphedema with side-to-end lymphovenous anastomosis with indocyanine green lymphography assisted. Microsurgery 2016;36(04):310-315 
  61. Koshima I, Narushima M, Mihara M, et al. Lymphadiposal flaps and lymphaticovenular anastomoses for severe leg edema: functional reconstruction for lymph drainage system. J Reconstr Microsurg 2016;32(01):50-55 
  62. Chen WF, Yamamoto T, Fisher M, Liao J, Carr J. The "Octopus" lymphaticovenular anastomosis: evolving beyond the standard supermicrosurgical technique. J Reconstr Microsurg 2015;31(06):450-457 
  63. Gennaro P, Gabriele G, Mihara M, et al. Supramicrosurgical lymphatico-venular anastomosis (LVA) in treating lymphoedema: 36-months preliminary report. Eur Rev Med Pharmacol Sci 2016;20(22):4642-4653 
  64. Greene AK, Maclellan RA. Operative treatment of lymphedema using suction-assisted lipectomy. Ann Plast Surg 2016;77(03):337-340 
  65. Lamprou DAA, Voesten HGJ, Damstra RJ, Wikkeling ORM. Circumferential suction-assisted lipectomy in the treatment of primary and secondary end-stage lymphoedema of the leg. Br J Surg 2017;104(01):84-89 
  66. Lee M, Perry L, Granzow J. Suction assisted protein lipectomy (SAPL) even for the treatment of chronic fibrotic and scarified lower extremity lymphedema. Lymphology 2016;49(01):36-41 
  67. Yamamoto T, Yoshimatsu H, Yamamoto N. Complete lymph flow reconstruction: a free vascularized lymph node true perforator flap transfer with efferent lymphaticolymphatic anastomosis. J Plast Reconstr Aesthet Surg 2016;69(09):1227-1233 
  68. Chen WF, Zhao H, Yamamoto T, Hara H, Ding J. Indocyanine green lymphographic evidence of surgical efficacy following microsurgical and supermicrosurgical lymphedema reconstructions. J Reconstr Microsurg 2016;32(09):688-698 
  69. Mihara M, Hara H, Tange S, et al. Multisite lymphaticovenular bypass using supermicrosurgery technique for lymphedema management in lower lymphedema cases. Plast Reconstr Surg 2016;138(01):262-272 
  70. Lee KT, Park JW, Mun GH. Serial two-year follow-up after lymphaticovenular anastomosis for the treatment of lymphedema. Microsurgery 2017;37(07):763-770 
  71. Stewart CJ, Munnoch DA. Liposuction as an effective treatment for lower extremity lymphoedema: a single surgeon's experience over nine years. J Plast Reconstr Aesthet Surg 2018;71(02):239-245 
  72. Borz C, Muresan M, Jimborean O, et al. Modified enteromesenteric bridging operation for primary lymphedema. Ann Ital Chir 2018;89(00):350-356 
  73. Sachanandani NS, Chu SY, Ho OA, Cheong CF, Lin MCY, Cheng MH. Lymphedema and concomitant venous comorbidity in the extremity: comprehensive evaluation, management strategy, and outcomes. J Surg Oncol 2018;118(06):941-952 
  74. Bolletta A, Di Taranto G, Chen SH, et al. Surgical treatment of Milroy disease. J Surg Oncol 2020;121(01):175-181 
  75. Giacalone G, Yamamoto T, Belva F, et al. The application of virtual reality for preoperative planning of lymphovenous anastomosis in a patient with a complex lymphatic malformation. J Clin Med 2019;8(03):371 
  76. Maruccia M, Pezzolla A, Nacchiero E, et al. Efficacy and early results after combining laparoscopic harvest of double gastroepiploic lymph node flap and active physiotherapy for lower extremity lymphedema. Microsurgery 2019;39(08):679-687 
  77. AlJindan FK, Lin CY, Cheng MH. Comparison of outcomes between side-to-end and end-to-end lymphovenous anastomoses for early-grade extremity lymphedema. Plast Reconstr Surg 2019;144(02):486-496 
  78. Drobot A, Bez M, Abu Shakra I, et al. Microsurgery for management of primary and secondary lymphedema. J Vasc Surg Venous Lymphat Disord 2021;9(01):226-233.e1 
  79. Onoda S, Nishimon K. The utility of surgical and conservative combination therapy for advanced stage lymphedema. J Vasc Surg Venous Lymphat Disord 2021;9(01):234-241 
  80. Scaglioni MF, Meroni M, Fritsche E. Combining superficial and deep lymphovenous anastomosis for lymphedema treatment: preliminary results. Microsurgery 2022;42(01):22-31 
  81. Robertson B, Neville E, Broering M, Tobler W, Recht M, Muck P. Multidisciplinary approach to management of severe lymphedema with one-stage radical excision and split-thickness skin grafting: report of two cases. J Vasc Surg Venous Lymphat Disord 2020;8(04):658-661 
  82. Damstra RJ, Dickinson-Blok JL, Voesten HGJM. Shaving technique and compression therapy for Elephantiasis Nostras Verrucosa (Lymphostatic Verrucosis) of forefeet and toes in end-stage primary lymphedema: a 5 year follow-up study in 28 patients and a review of the literature. J Clin Med 2020;9(10):3139 
  83. Hayashi A, Visconti G, Yang CJ, Hayashi N, Yoshimatsu H. Additional lymphaticovenular anastomosis on the posterior side for treatment of primary lower extremity lymphedema. J Clin Med 2022;11(03):867 
  84. Mavili ME, Naldoken S, Safak T. Modified Charles operation for primary fibrosclerotic lymphedema. Lymphology 1994;27(01):14-20 
  85. Greene AK, Schook CC. Primary lymphedema: definition of onset based on developmental age. Plast Reconstr Surg 2012;129(01):221e-222e 
  86. Barone V, Borghini A, Tedone Clemente E, et al. New insights into the pathophysiology of primary and secondary lymphedema: histopathological studies on human lymphatic collecting vessels. Lymphat Res Biol 2020;18(06):502-509 
  87. Goss JA, Maclellan RA, Greene AK. Adult-onset primary lymphedema: a clinical-lymphoscintigraphic study of 26 patients. Lymphat Res Biol 2019;17(06):620-623 
  88. Ciudad P, Manrique OJ, Bustos SS, et al. Single-stage VASER-assisted liposuction and lymphatico-venous anastomoses for the treatment of extremity lymphedema: a case series and systematic review of the literature. Gland Surg 2020;9(02):545-557 
  89. Forte AJ, Huayllani MT, Boczar D, Ciudad P, Manrique O. Lipoaspiration and lymph node transfer for treatment of breast cancer-related lymphedema: a systematic review. Cureus 2019;11(11):e6096 
  90. Brazio PS, Nguyen DH. Combined liposuction and physiologic treatment achieves durable limb volume normalization in class IIIII lymphedema: a treatment algorithm to optimize outcomes. Ann Plast Surg 2021;86(5S, Suppl 3)S384-S389 
  91. Scaglioni MF, Fontein DBY, Arvanitakis M, Giovanoli P. Systematic review of lymphovenous anastomosis (LVA) for the treatment of lymphedema. Microsurgery 2017;37(08):947-953 
  92. Tang NSJ, Ramakrishnan A, Shayan R. Quality-of-life outcomes after operative management of primary and secondary lymphoedema: a systematic review. ANZ J Surg 2021;91(12):2624-2636 
  93. Fallahian F, Tadisina KK, Xu KY. Efficacy of microsurgical treatment of primary lymphedema: a systematic review. Ann Plast Surg 2022;88(02):195-199 
  94. Kong X, Du J, Du X, Cong X, Zhao Q. A meta-analysis of 37 studies on the effectiveness of microsurgical techniques for lymphedema. Ann Vasc Surg 2022;86:440-451.e6