|Year : 2022 | Volume
| Issue : 3 | Page : 211-214
Lymphedema – The stepchild of vascular surgeons
Department of Vascular Surgery, Sultan Qaboos University Hospital, Muscat, Oman
|Date of Submission||19-Jul-2022|
|Date of Acceptance||19-Jul-2022|
|Date of Web Publication||21-Aug-2022|
Department of Vascular Surgery, Sultan Qaboos University Hospital, Muscat
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Stephen E. Lymphedema – The stepchild of vascular surgeons. Indian J Vasc Endovasc Surg 2022;9:211-4
| Introduction|| |
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. 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.
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.,
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.
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.,, Lymphedema developing secondary to breast cancer is thought to have a genetic predisposition. The coexistence of chronic venous insufficiency (phlebolymphedema) and lipedema (lipo-lymphedema) has been reported.,
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.
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.
|Figure 3: (a and b) Lymphedema with cellulitis, hyperkeratosis, and wart|
Click here to view
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.
Lymphoscintigraphy using sulfur colloid which is injected subcutaneously helps visualize lymphatic drainage. 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.,
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., 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.,, 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. 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.
|Figure 5: Silicone tubes placed above the fascia – left leg. Courtesy Dr. Rajesh Hyderabadi|
Click here to view
Surgical intervention for obesity can reverse Stage I lymphedema and will stay that way provided body mass index remains normal [Figure 6].
On occasion, patients can present with maggots in the ulcer [Figure 7] with or without necrotizing fasciitis and would need emergency debridement.
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|| |
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.
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.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]