Table of Contents  
EDITORIAL
Year : 2022  |  Volume : 9  |  Issue : 3  |  Page : 211-214

Lymphedema – The stepchild of vascular surgeons


Department of Vascular Surgery, Sultan Qaboos University Hospital, Muscat, Oman

Date of Submission19-Jul-2022
Date of Acceptance19-Jul-2022
Date of Web Publication21-Aug-2022

Correspondence Address:
Edwin Stephen
Department of Vascular Surgery, Sultan Qaboos University Hospital, Muscat
Oman
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijves.ijves_48_22

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How to cite this article:
Stephen E. Lymphedema – The stepchild of vascular surgeons. Indian J Vasc Endovasc Surg 2022;9:211-4

How to cite this URL:
Stephen E. Lymphedema – The stepchild of vascular surgeons. Indian J Vasc Endovasc Surg [serial online] 2022 [cited 2022 Sep 25];9:211-4. Available from: https://www.indjvascsurg.org/text.asp?2022/9/3/211/354077




  Introduction Top


As I begin to write this Editorial, I wonder why there is a dearth of literature and research when compared to that which is available for the pathophysiology and management of arterial and venous disorders. It is certainly not for want of patients who are suffering for the disease.

“Lymphedema is not just a little swelling. It can be a complete physical, emotional, and mental toll on the mind and body” – Lymphie Strong.[1] Patients suffering from chronic edema/lymphedema struggle with disability, getting and maintaining gainful employment, and acceptance in society. It has been described as a hidden epidemic.[2]

The number of vascular surgeons who have a keen interest in lymphedema is few and far between, as are sessions during annual conference/s. Dare I say – Is this because neither the disease nor the patients are glamorous or simply due to a lack of knowledge and understanding of the disease. The lack of interest leads to underdiagnosis, underdocumentation, and undertreatment. Resource mobilization, program implementation, and advocacy aren't a priority for health professionals and governments – when India accounts for 42% of the 120 million global lymphatic filarial (LF) cases.[3],[4]

What do we know?

The lymphatic system has unidirectional flow and runs along the venous system. Its primary function is fluid homeostasis, besides facilitating fat absorption from the bowel, aiding the immune system, and helping reduce the inflammatory process. Lymph contains immune cells, macromolecules, and fluid in vessels that then drain into a collection system that has circumferential smooth muscle and valves. These channels then converge at lymph nodes.[5]

Etiology could be primary [Figure 1], is rare, and is attributed to known genetic defects. It can be inherited/congenital. Genetic testing yield is poor, and it does not affect management in most cases. Secondary lymphedema [Figure 2] is far more common. It is secondary to any cause that impairs the flow of lymph with LF being the most common manifestation. Other causes include malignancy, surgery, radiation, cellulitis, trauma, and obesity.[6],[7],[8] Lymphedema developing secondary to breast cancer is thought to have a genetic predisposition.[9] The coexistence of chronic venous insufficiency (phlebolymphedema) and lipedema (lipo-lymphedema) has been reported.[10],[11]
Figure 1: Primary lymphedema

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Figure 2: Secondary lymphedema

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Lymphedema is not just the accumulation of fluid but that of fat deposition which is thought to be secondary to hypertrophic fat lobules compressing the feeding lymphatic capillaries and compressing them. This leads to a downward spiral in the transport mechanism of fluid and lipid, leading to fat accumulation. As a dysfunctional lymphatic vessel swells the smooth muscle cells within its media become slim and flattened and the dermal capillary vessels become hypertrophic leading to backflow of lymphatic fluid. When fibrosis within the skin and subcutaneous tissues is superadded to this accumulation of fat and fluid it leads to nonpitting edema.[12]

Toward the later stages of the disease, the skin can become “leathery” in texture secondary to hyperkeratosis making it prone to recurrent infection, cellulitis, ulceration, and wart formation [Figure 3]a and [Figure 3]b.[13]
Figure 3: (a and b) Lymphedema with cellulitis, hyperkeratosis, and wart

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What can we offer?

Well begun is half done and hence a thorough history and examination of the patient are crucial to diagnosis. The disease needs to be staged based on the International Society of Lymphology staging system. Stage 0 is latent where swelling is not evident despite impaired drainage; Stage I is characterized by dependent edema; Stage II is nondependent edema and is pitting. This stage can progress to nonpitting edema depending on the extent of fibrosis. Stage III lymphedema [Figure 4]a and [Figure 4]b, is nonpitting and is also known as lymphostatic elephantiasis.[12]
Figure 4: (a and b) Lymphatic elephantiasis

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Lymphoscintigraphy using sulfur colloid which is injected subcutaneously helps visualize lymphatic drainage.[14] Computed tomography (CT) and magnetic resonance imaging (MRI) have been reported to have sensitivities of about 80%, but there are limitations such as radiation with CT and venous contamination with contrast in MRI. Fluorescence is useful for mapping tissue for surgery.[15],[16]

The best medical therapy would include control of comorbidities – diabetes, dyslipidemia, heart failure, renal failure, and obesity and use of diuretics, good skin, and podiatry care. The mainstay is complete decongestive therapy (CDT) which includes manual lymphatic drainage that redirects and promotes lymphatic drainage by repetitive, nibble-taught movements, and compression therapy using custom and flat knit grade II/III compression stockings. CDT takes up to 6 weeks of focused therapy followed by maintenance therapy for it to be effective and requires a motivated patient who is on follow-up. The benefit of intermittent pneumatic compression devices is controversial. The use of anti-inflammatory medication ketoprofen has been shown to help reduce skin thickness and histological changes. Whether it will help patients with fibrosis and adipose tissue deposition remains to be seen. [12,17-19]

Surgical options include among others – Lymphovenous bypass (LVB), vascularized lymph node transplant (VLNT,) and debulking or liposuction. LVB and VLNT are considered physiologic, functional treatment options which lead to a better quality of life from a reduction in volume and recurrent cellulitis. VLNT, it is hypothesized that lymphangiogenesis occurs as does the development of new collaterals.[20],[21] LVB and VLNT have superior results when performed in the early stage of the disease. Excision of excessive adipose tissue and skin [Figure 4] often leaves unstable scars with poor cosmetic outcomes. On the other hand, liposuction wherein the hypertrophic fat is removed after careful planning following an MRI seems to have better volume reduction and lesser recurrence. Risks would include bleeding, skin necrosis, and contour irregularity.[12],[22],[23] Whatever the modality used compression hosiery for life is advised. Recent advances have found it useful to combine the use of indocyanine green to map the lymphatic channels and anastomose <1 mm vessels with supermicrosurgical techniques.[24] The use of silicone tubes [Figure 5] as artificial lymphatic channels, placed above the fascia, has been tried by some surgeons and found useful in conjunction with compression therapy.[25]
Figure 5: Silicone tubes placed above the fascia – left leg. Courtesy Dr. Rajesh Hyderabadi

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Surgical intervention for obesity can reverse Stage I lymphedema and will stay that way provided body mass index remains normal [Figure 6].
Figure 6: Pre- and postbariatric surgery

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On occasion, patients can present with maggots in the ulcer [Figure 7] with or without necrotizing fasciitis and would need emergency debridement.
Figure 7: Lymphedema with an ulcer and maggots within

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What does the future hold?

There is ongoing research about – how to target the T-cells; use lymphangiogenic growth factor/s, tissue engineering, and immunomodulatory therapy as a therapeutic strategy.

Vascular societies need to advocate for these patients by facilitating C.M.E's/workshops, encouraging research, and getting companies to offer custom-made compression hosiery at affordable prices which would be covered by either the public health system or insurance companies.


  Conclusion Top


Lymphedema is a chronic disease and efforts need to be made to diagnose patients early and offer strategies that will prevent progression. VLB, VLNT, and microlymphatic surgery should be considered in the early stages.

The battle with this “step-child” is a long (sometimes arduous) one, and both the patient and the treating multidisciplinary team comprising vascular/plastic surgeons, physicians, dermatologists, physiotherapists, podiatrists, and nurses, need to be motivated for the long haul.

Acknowledgments

I would like to thank the patients who have consented to the use of their photos, my colleagues, and the support staff from physiotherapy and nursing.



 
  References Top

1.
Lymphie Strong. Youth and Lymphedema. Twitter; November 11, 2021. Available from: https://twitter.com/LymphieStrong/status/1458544290829844485?s=20&t=1jxcciVuJzIGHxE-tGGyWw. [Last accessed on 2022 Jul 08].  Back to cited text no. 1
    
2.
Keast DH, Moffatt C, Janmohammad A. Lymphedema impact and prevalence international study: The Canadian data. Lymphat Res Biol 2019;17:178-86.  Back to cited text no. 2
    
3.
World Health Organization. Global programme to eliminate lymphatic filariasis: Progress report for 2017. Wkly Epidemiol Rec 2018;44:589-604.  Back to cited text no. 3
    
4.
Larry S, Eileen S. Economic costs and benefits of community based lymphedema-Management programs for lymphatic filariasis in India. Am J Trop Med Hyg 2020;103:295-302.  Back to cited text no. 4
    
5.
Tammela T, Alitalo K. Lymphangiogenesis: molecular mechanisms and future promise. Cell 2010;140:460-76.  Back to cited text no. 5
    
6.
Rockson SG, Rivera KK. Estimating the population burden of lymphedema. Ann N Y Acad Sci 2008;1131:147-54.  Back to cited text no. 6
    
7.
Gordon K, Varney R, Keeley V, Riches K, Jeffery S, Van Zanten M, et al. Update and audit of the St George's classification algorithm of primary lymphatic anomalies: A clinical and molecular approach to diagnosis. J Med Genet 2020;57:653-9.  Back to cited text no. 7
    
8.
Greene AK. Diagnosis and management of obesity-induced lymphedema. Plast Reconstr Surg 2016;138:111e-8e.  Back to cited text no. 8
    
9.
Cormier JN, Askew RL, Mungovan KS, Xing Y, Ross MI, Armer JM. Lymphedema beyond breast cancer: A systematic review and meta-analysis of cancer-related secondary lymphedema. Cancer 2010;116:5138-49.  Back to cited text no. 9
    
10.
Lee BB. Phlebolymphedema: Neglected outcome of combined venous and lymphatic insufficiency. Vasc Specialist Int 2020;36:1-3.  Back to cited text no. 10
    
11.
Dheepak AS, Prabhu P, Vimalin S, Edwin S, Sunil A. Co-existence of chronic venous insufficiency in patients with lymphedema – An under diagnosed entity. Indian J Appl Res 2016;6:496-8.  Back to cited text no. 11
    
12.
Azhar SH, Lim HY, Tan BK, Angeli V. The unresolved pathophysiology of lymphedema. Front Physiol 2020;11:137.  Back to cited text no. 12
    
13.
Grada AA, Phillips TJ. Lymphedema: Pathophysiology and clinical manifestations. J Am Acad Dermatol 2017;77:1009-20.  Back to cited text no. 13
    
14.
Bernas M, Thiadens SRJ, Smoot B, Armer JM, Stewart P, Granzow J. Lymphedema following cancer therapy: Overview and options. Clin Exp Metastasis 2018;35:547-51.  Back to cited text no. 14
    
15.
Monnin-Delhom ED, Gallix BP, Achard C, Bruel JM, Janbon C. High resolution unenhanced computed tomography in patients with swollen legs. Lymphology 2002;35:121-8.  Back to cited text no. 15
    
16.
Ripley B, Wilson GJ, Lalwani N, Briller N, Neligan PC, Maki JH. Initial clinical experience with dual-agent relaxation contrast for isolated lymphatic channel mapping. Radiology 2018;286:705-14.  Back to cited text no. 16
    
17.
Rockson SG, Tian W, Jiang X, Kuznetsova T, Haddad F, Zampell J, et al. Pilot studies demonstrate the potential benefits of antiinflammatory therapy in human lymphedema. JCI Insight 2018;3:123775.  Back to cited text no. 17
    
18.
Fu MR, Deng J, Armer JM. Putting evidence into practice: Cancer-related lymphedema. Clin J Oncol Nurs 2014;18 Suppl: 68-79.  Back to cited text no. 18
    
19.
Lerman M, Gaebler JA, Hoy S, Ihakoff J, Gullet L, Niecko T, et al. Health and economic benefits of advanced pneumatic compression devices in patients with phlebolymphedema. J Vasc Surg 2019;69:571-80.  Back to cited text no. 19
    
20.
Joseph WJ, Aschen S, Ghanta S, Cuzzone D, Albano N, Gardenier J, et al. Sterile inflammation after lymph node transfer improves lymphatic function and regeneration. Plast Reconstr Surg 2014;134:60-8.  Back to cited text no. 20
    
21.
Cheng MH, Loh CY, Lin CY. Outcomes of vascularized lymph node transfer and lymphovenous anastomosis for treatment of primary lymphedema. Plast Reconstr Surg Glob Open 2018;6:e2056.  Back to cited text no. 21
    
22.
Chen WF, Zeng WF, Hawkes PJ, Man J, Bowen M. Lymphedema liposuction with immediate limb contouring. Plast Reconstr Surg Glob Open 2019;7:e2513.  Back to cited text no. 22
    
23.
Hoffner M, Ohlin K, Svensson B, Manjer J, Hansson E, Troëng T, et al. Liposuction gives complete reduction of arm lymphedema following breast cancer treatment-a 5-year prospective study in 105 patients without recurrence. Plast Reconstr Surg Glob Open 2018;6:e1912.  Back to cited text no. 23
    
24.
Rajan S, Venkatramani H. Recent advances in management of lymphedema. J Skin Sex Transm Dis 2021;3:26-32.  Back to cited text no. 24
    
25.
Singh AP, Dhar A, Srivastava A, Kumar R, Pandey RM. Comparing the efficacy of a combination of artificial lymphatics in the form of silicone tube and compressive therapy versus compressive therapy only in upper limb lymphedema following axillary lymph node dissection in breast cancer patients: A randomized controlled trial. Indian J Vasc Endovasc Surg 2019;6:283-90.  Back to cited text no. 25
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    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]



 

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