J Reconstr Microsurg 2017; 33(01): 019-025
DOI: 10.1055/s-0036-1586257
Original Article
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

A Detailed Examination of the Characteristics and Treatment in a Series of 33 Idiopathic Lymphedema Patients

Satoshi Onoda
1   Department of Plastic and Reconstructive Surgery, Okayama University, Okayama, Japan
,
Kiyoshi Yamada
1   Department of Plastic and Reconstructive Surgery, Okayama University, Okayama, Japan
,
Kumiko Matsumoto
1   Department of Plastic and Reconstructive Surgery, Okayama University, Okayama, Japan
,
Yoshihiro Kimata
1   Department of Plastic and Reconstructive Surgery, Okayama University, Okayama, Japan
› Author Affiliations
Further Information

Publication History

04 April 2016

22 June 2016

Publication Date:
19 August 2016 (online)

Abstract

Background To date, only a few reports have been published on idiopathic lymphedema, and most of these are case reports. In this time, we performed a retrospective review of idiopathic lymphedema patients.

Methods Forty patients presented with idiopathic lymphedema that was unrelated to surgery, injury, and medical conditions such as circulatory disease, kidney disease, and thrombotic venous disease. For these patients, we performed a retrospective review for edema-onset age, sex, location and stage of the edema, presence of cellulitis, episode related to edema onset, therapeutic intervention, and follow-up duration in the 33 patients whom we followed up.

Results Among the 33 patients with idiopathic lymphedema, there were 11 patients with either congenital or childhood-onset lymphedema, 14 with adult-onset lymphedema, and the remaining 8 patients had an onset age of >60 years. Twenty of the 33 patients had undergone surgical treatment. Of the 19 patients who underwent lymphaticovenular anastomosis (LVA), 2 had good, 4 had fair, and 13 had poor treatment outcomes. Twelve patients successfully received only conservative treatment and follow-up for more than 3 months, without operation. The treatment outcome in these 12 patients was as follows: excellent in 4 patients, good in 6, and fair in 2 patients.

Conclusion In this study, the usefulness of LVA for most patients with idiopathic lymphedema was not confirmed. Although this review did not include a conducted case, the indication for new surgical treatments such as vascularized lymph node transfer should be considered in the future.

 
  • References

  • 1 Moseley AL, Carati CJ, Piller NB. A systematic review of common conservative therapies for arm lymphoedema secondary to breast cancer treatment. Ann Oncol 2007; 18 (4) 639-646
  • 2 Szolnoky G, Lakatos B, Keskeny T , et al. Intermittent pneumatic compression acts synergistically with manual lymphatic drainage in complex decongestive physiotherapy for breast cancer treatment-related lymphedema. Lymphology 2009; 42 (4) 188-194
  • 3 Kwan ML, Darbinian J, Schmitz KH , et al. Risk factors for lymphedema in a prospective breast cancer survivorship study: the Pathways Study. Arch Surg 2010; 145 (11) 1055-1063
  • 4 Deltombe T, Jamart J, Recloux S , et al. Reliability and limits of agreement of circumferential, water displacement, and optoelectronic volumetry in the measurement of upper limb lymphedema. Lymphology 2007; 40 (1) 26-34
  • 5 Boccardo F, Casabona F, De Cian F , et al. Lymphedema microsurgical preventive healing approach: a new technique for primary prevention of arm lymphedema after mastectomy. Ann Surg Oncol 2009; 16 (3) 703-708
  • 6 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 (6) 437-442
  • 7 Beesley V, Janda M, Eakin E, Obermair A, Battistutta D. Lymphedema after gynecological cancer treatment : prevalence, correlates, and supportive care needs. Cancer 2007; 109 (12) 2607-2614
  • 8 Kerchner K, Fleischer A, Yosipovitch G. Lower extremity lymphedema update: pathophysiology, diagnosis, and treatment guidelines. J Am Acad Dermatol 2008; 59 (2) 324-331
  • 9 Shahpar H, Atieh A, Maryam A , et al. Risk factors of lymph edema in breast cancer patients. Int J Breast Cancer 2013; 2013: 641818
  • 10 Ugur S, Arıcı C, Yaprak M , et al. Risk factors of breast cancer-related lymphedema. Lymphat Res Biol 2013; 11 (2) 72-75
  • 11 Akita S, Mitsukawa N, Rikihisa N , et al. Early diagnosis and risk factors for lymphedema following lymph node dissection for gynecologic cancer. Plast Reconstr Surg 2013; 131 (2) 283-290
  • 12 Deng J, Ridner SH, Dietrich MS , et al. Factors associated with external and internal lymphedema in patients with head-and-neck cancer. Int J Radiat Oncol Biol Phys 2012; 84 (3) e319-e328
  • 13 Kim JH, Choi JH, Ki EY , et al. Incidence and risk factors of lower-extremity lymphedema after radical surgery with or without adjuvant radiotherapy in patients with FIGO stage I to stage IIA cervical cancer. Int J Gynecol Cancer 2012; 22 (4) 686-691
  • 14 Fujimoto N, Honda S, Nakanishi G, Tachibana T, Tanaka T. Acquired idiopathic penile lymphedema: Successful prevention of acute phase progression with low-dose corticosteroids. J Dermatol 2014; 41 (2) 157-159
  • 15 Hara H, Mihara M, Seki Y, Koshima I. Lymphoedema caused by idiopathic lymphatic thrombus. J Plast Reconstr Aesthet Surg 2013; 66 (12) 1780-1783
  • 16 Eva LJ, Narain S, Luesley DM. Idiopathic vulval lymphoedema. J Obstet Gynaecol 2007; 27 (7) 748-749
  • 17 Sukan A, Yaycioglu O, Aydin M, Yapar AF, Kizilkilic O, Reyhan M. Lymphoscintigraphic appearance of scrotal lymphatic reflux in a patient with idiopathic genital lymphedema. Clin Nucl Med 2005; 30 (12) 835-837
  • 18 Hornberger BJ, Elmore JM, Roehrborn CG. Idiopathic scrotal elephantiasis. Urology 2005; 65 (2) 389
  • 19 Hashem FK, Ahmed S. Idiopathic scrotal lymphoedema in Down's syndrome. Aust N Z J Surg 1999; 69 (1) 75-77
  • 20 Henze E, Schelbert HR, Collins JD, Najafi A, Barrio JR, Bennett LR. Lymphoscintigraphy with Tc-99m-labeled dextran. J Nucl Med 1982; 23 (10) 923-929
  • 21 Szuba A, Shin WS, Strauss HW, Rockson S. The third circulation: radionuclide lymphoscintigraphy in the evaluation of lymphedema. J Nucl Med 2003; 44 (1) 43-57
  • 22 Tomczak H, Nyka W, Lass P. Lymphoedema: lymphoscintigraphy versus other diagnostic techniques—a clinician's point of view. Nucl Med Rev Cent East Eur 2005; 8 (1) 37-43
  • 23 Modi S, Stanton AW, Mortimer PS, Levick JR. Clinical assessment of human lymph flow using removal rate constants of interstitial macromolecules: a critical review of lymphoscintigraphy. Lymphat Res Biol 2007; 5 (3) 183-202
  • 24 Maegawa J, Mikami T, Yamamoto Y, Satake T, Kobayashi S. Types of lymphoscintigraphy and indications for lymphaticovenous anastomosis. Microsurgery 2010; 30 (6) 437-442
  • 25 Ogata F, Azuma R, Kikuchi M, Koshima I, Morimoto Y. Novel lymphography using indocyanine green dye for near-infrared fluorescence labeling. Ann Plast Surg 2007; 58 (6) 652-655
  • 26 Ogata F, Narushima M, Mihara M, Azuma R, Morimoto Y, Koshima I. Intraoperative lymphography using indocyanine green dye for near-infrared fluorescence labeling in lymphedema. Ann Plast Surg 2007; 59 (2) 180-184
  • 27 Unno N, Inuzuka K, Suzuki M , et al. Preliminary experience with a novel fluorescence lymphography using indocyanine green in patients with secondary lymphedema. J Vasc Surg 2007; 45 (5) 1016-1021
  • 28 Unno N, Nishiyama M, Suzuki M , et al. Quantitative lymph imaging for assessment of lymph function using indocyanine green fluorescence lymphography. Eur J Vasc Endovasc Surg 2008; 36 (2) 230-236
  • 29 Unno N, Nishiyama M, Suzuki M , et al. A novel method of measuring human lymphatic pumping using indocyanine green fluorescence lymphography. J Vasc Surg 2010; 52 (4) 946-952
  • 30 Witte CL, Witte MH, Unger EC , et al. Advances in imaging of lymph flow disorders. Radiographics 2000; 20 (6) 1697-1719
  • 31 Brorson H, Ohlin K, Olsson G, Långström G, Wiklund I, Svensson H. Quality of life following liposuction and conservative treatment of arm lymphedema. Lymphology 2006; 39 (1) 8-25
  • 32 Qi F, Gu J, Shi Y, Yang Y. Treatment of upper limb lymphedema with combination of liposuction, myocutaneous flap transfer, and lymph-fascia grafting: a preliminary study. Microsurgery 2009; 29 (1) 29-34
  • 33 Brorson H. Liposuction in lymphedema treatment. J Reconstr Microsurg 2016; 32 (1) 56-65
  • 34 Narushima M, Yamamoto T, Ogata F, Yoshimatsu H, Mihara M, Koshima I. Indocyanine green lymphography findings in limb lymphedema. J Reconstr Microsurg 2016; 32 (1) 72-79
  • 35 Arrivé L, Derhy S, El Mouhadi S, Monnier-Cholley L, Menu Y, Becker C. Noncontrast magnetic resonance lymphography. J Reconstr Microsurg 2016; 32 (1) 80-86
  • 36 Liu N, Zhang Y. Magnetic resonance lymphangiography for the study of lymphatic system in lymphedema. J Reconstr Microsurg 2016; 32 (1) 66-71
  • 37 Suami H, Taylor GI, Pan WR. A new radiographic cadaver injection technique for investigating the lymphatic system. Plast Reconstr Surg 2005; 115 (7) 2007-2013
  • 38 Suami H, Taylor GI, O'Neill J, Pan WR. Refinements of the radiographic cadaver injection technique for investigating minute lymphatic vessels. Plast Reconstr Surg 2007; a 120 (1) 61-67
  • 39 Suami H, Pan WR, Taylor GI. Changes in the lymph structure of the upper limb after axillary dissection: radiographic and anatomical study in a human cadaver. Plast Reconstr Surg 2007; b 120 (4) 982-991
  • 40 Suami H, O'Neill JK, Pan WR, Taylor GI. Superficial lymphatic system of the upper torso: preliminary radiographic results in human cadavers. Plast Reconstr Surg 2008; 121 (4) 1231-1239
  • 41 Becker C. Autologous lymph node transfers. J Reconstr Microsurg 2016; 32 (1) 28-33
  • 42 Baumeister RG, Mayo W, Notohamiprodjo M, Wallmichrath J, Springer S, Frick A. Microsurgical lymphatic vessel transplantation. J Reconstr Microsurg 2016; 32 (1) 34-41