Lymphedema is an accumulation of lymphatic fluid in the interstitial tissue that causes swelling, most often in the arm and/or leg, and occasionally in other parts of the body. Lymphedema can develop when lymphatic vessels are missing or impaired (primary), or when lymph vessels are damaged or lymph nodes removed (secondary).
When the impairment becomes so great that the lymphatic fluid exceeds the lymphatic transport capacity, an abnormal amount of protein-rich fluid collects in the tissues of the affected area. Left untreated, this stagnant, protein-rich fluid not only causes tissue channels to increase in size and number, but also reduces oxygen availability in the transport system, interferes with wound healing and provides a culture medium for bacteria that can result in lymphangitis.
Primary lymphedema, which can affect from one to as many as four limbs and/or other parts of the body, can be present at birth, develop at the onset of puberty or in adulthood, all from unknown causes, or associated with vascular anomalies such as hemangioma, lymphangioma, port wine stain or Klippel Trenaury.
Secondary lymphedema, or acquired lymphedema, can develop as a result of surgery, radiation, infection or trauma. Specific surgeries, such as surgery for melanoma or breast, gynecological, head and neck, prostate or testicular, bladder or colon cancer, all of which currently require removal of lymph nodes, put patients at risk of developing secondary lymphedema. If lymph nodes are removed, there is always a risk of developing lymphedema. Secondary lymphedema can develop immediately post-operatively, or weeks, months, even years later.
Lymphedema develops in stages, from mild to severe, and is defined below:
Stage 1 (spontaneously reversible):
Tissue is still at the "pitting" stage, which means that when pressed by fingertips, the area indents and holds the indentation. Usually, upon waking in the morning, the limb or affected area is normal or almost normal size.
Stage 2 (spontaneously irreversible):
The tissue now has a spongy consistency and is "non-pitting," meaning that when pressed by fingertips, the tissue bounces back without any indentation forming. Fibrosis found in this stage of lymphedema marks the beginning of the hardening and increasing size of the limbs.
Stage 3 (lymphostatic elephantiasis):
At this stage the swelling is irreversible and usually the limbs are very large. The tissue is hard (fibrotic) and unresponsive; some patients consider undergoing reconstructive surgery called "debulking" at this stage.
When lymphedema remains untreated, protein-rich fluid continues to accumulate, leading to an increase of swelling and a hardening or fibrosis of the tissue. In this state, the swollen limb becomes a perfect culture medium for bacteria and subsequent recurrent lymphangitis. Moreover, untreated lymphedema can lead into a decrease or loss of functioning of the limb, skin breakdown, chronic infections and, sometimes, irreversible complications. In the most severe cases, untreated lymphedema can develop into a rare form of lymphatic cancer called lymphangiosarcoma (most often in secondary lymphedema).
Microsurgical techniques for the anastomosis of blood or lymphatic vessels have introduced a possible method for treating lymphedema in the extremities. These techniques attempt to create a means for lymphatic fluid to bypass the obstruction by being channeled through the venous system (lymphatic-venous anastomoses), or by using venous grafts between lymphatic collectors above and below the obstruction (lymphatic-venous-lymphatic plasty).
Another proposed technique for treatment of lymphedema is vascularized lymph node transfer. Vascularized healthy tissues with lymph nodes are transferred to a site affected by lymphedema. Donor sites, as well as the recipient sites, can vary. An example would be the transfer of vascularized groin lymph nodes to a wrist area in a patient with postmastectomy upper extremity lymphedema.
Surgery for lymphedema (e.g., microsurgical lymphovenous anastomoses or vascularized lymph node transfer) is considered INVESTIGATIONAL.
Harris and colleagues conducted a systematic review of literature retrieved from MEDLINE (1966-2000) and CANCERLIT (1985-2000), as well as a non-systematic review of breast cancer literature published to October 2000. They concluded that surgery as a treatment option for lymphedema (e.g., microsurgical lymphovenous anastomoses) has produced disappointing, inconsistent results and should be avoided.
The management of lymphedema in breast cancer patients is based primarily on results from case studies, clinical experience and anecdotal information. The natural history and most effective therapies for lymphedema are poorly understood and need further study. Accurate assessment requires agreement on a standardized and reliable system of measurement; randomized controlled trials to answer these questions should be encouraged.
Gloviczki and colleagues from the Mayo Clinic followed their patients for an average of three years after microsurgical lymphovenous anastomosis for treatment of lymphedema. All patients who underwent the operations had failed medical management that consisted of prolonged periods of intermittent compression with pneumatic pumps and elastic support. Their trial was small, involving only 18 patients. Fourteen patients were evaluated and of these, five had improvement, five were unchanged and four had progression of their lymphedema at the time of last follow-up. The authors concluded that there was no objective evidence supporting the value of microsurgical treatment for lymphedema.
Per the National Cancer Institute (NCI), lymphedema is treated by physical methods and drug therapy. This includes compression garments, antibiotics, diuretics, anticoagulants and dietary management. Surgery for treating lymphedema usually results in complications and is seldom recommended for cancer patients.
The trials that report on surgical techniques to treat lymphedema are very small with little follow-up and inadequate information regarding the outcome of those patients where the surgery was ineffective.
Surgery for lymphedema is considered experimental, investigational and unproven due to lack of adequate evidence of safety and effectiveness documented in published, peer-reviewed medical literature.
A search of peer-reviewed literature through November 2011 was performed. The following is a summary of the key literature to date.
Mukenge S.M. et al. reported on a study that included patients with external male genital organ lymphedema. In this study a novel surgical technique was used; this surgical technique was a microsurgical lymphovenous derivation. The patency of lymphovenous anastomoses was assessed by noninvasive lymphography at 3, 6 and 12 months after surgery. Five of the 11 patients underwent the microsurgical lymphovenous derivation. The authors noted:
“The present study shows that lymphovenous anastomosis is a valuable method of resolving the edematous condition.” The authors also noted the low number of patients enrolled in the study as a possible limitation of the study. This update failed to identify any additional information that would change the coverage position of this medical policy.
A search of peer reviewed literature was conducted through September 2014. Following is a summary of the key literature to date.
Several lymph node transfer studies were reviewed. (6-10) No randomized controlled trials (RCTs) were identified. Several techniques were identified in these studies for postmastectomy lymphedema to include vascularized lymph node transfer from the groin/femoral region to the wrist, elbow and axillary regions. One study reported on the results from using vascularized lymph node transfer with hilar perforators (10) and another performed simultaneous breast and lymphatic reconstruction (8).
Chang et al., in a prospective study of 100 consecutive lymphovenous bypass cases for both upper and lower extremity lymphedema secondary to cancer treatment, utilized Indocyanine green lymphangiography in patients to help identify the route of the lymphatic vessels. (11) The authors concluded lymphovenous bypass can be effective in reducing lymphedema. This surgery may be more effective in patients with early stage upper extremity lymphedema. Indocyanine green lymphangiography may play a role in objective patient selection for lymphovenous bypass.
A search of literature review revealed several small studies. Due to no large studies, no standardized techniques to deliver therapeutic treatment and no long-term results, the coverage statement remains unchanged.
- Gloviczki, P., Fisher, J., et al. Microsurgical lymphovenous anastomosis for treatment of lymphedema: A critical review. Journal of Vascular Surgery (1988) 7:647-52.
- Harris, S., Hugi, M., et al. Clinical practice guidelines for the care and treatment of breast cancer: 11. Lymphedema. Canadian Medical Association Journal (2001) 164 (2): 191-199.
- National Lymphedema Network. Lymphedema FAQ. Available at <www.lymphnet.org> (accessed – 2009 February 17).
- National Cancer Institute. Lymphedema. Available at <www.cancer.gov> (accessed – 2009 February 17).
- Mukenge S.M., Catena M. et al. Assessment and follow-up of patency after lymphovenous microsurgery for treatment of secondary lymphedema in external male genital organs. European Urology 2011 November: 60(5):1114-9. [Epub 2010 November 24]
- Lin, C., Ali, R., et al. Vacularized 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.
- Becker, C., Assouad, J., et al Postmastectomy lymphedema: long-term results following microsurgical lymph node transplantation. Ann Aurg. 2006 Mar; 243(3):313-5.
- Saaristo, A., Niemi, T., et al. Microvascular breast reconstruction and lymph node transfer for postmastectomy lymphedema patients. Ann Surg. 2012 Mar; 255(3):468-73.
- Cheng, M., Chen, S., et al. Vascularized groin lymph node flap transfer for postmastectomy upper limb lymphedema: flap anatomy, recipient sites, and outcomes. Plast Reconstr Surg. 2013 Jun; 131 (6): 1286-98.
- Gharb, B. Rampazzo, A., et. al. Vascularized lymph node transfer based on the hilar perforators improves the outcome in upper limb lymphedema. Ann Plast Surg. 2011 Dec; 67(6): 589-93.
- Chang, D., Suami, H. et.al. A Prospective Analysis of 100 Consecutive Lymphovenous Bypass Cases for Treatment of Extremity lymphedema. Plastic and Reconstructive Surgery. 2013 November ; 1305-14.
- Lymphedema (PDQ®) Management. Available at <www.cancer.gov>. Access on 2014 September 9.
||Free skin flap (microvascular transfer)
||Free fascia flap (microvascular transfer)
||Repair Procedures Blood Vessel Other Than for Fistula, With or Without Patch Angioplasty
||Lymphangiotomy or other operations on Lymphatic channels
||Injection procedure; Lymphangiography
||Unlisted procedure, Hemic or Lymphatic system
||Other noninfective disorders of lymphatic vessels and lymph nodes (intractable lymphedema)
||Postmastectomy lymphedema syndrome (vascularized lymph node transfer not covered for the treatment of post-mastectomy lymphedema or for the treatment of lymphedema due to cervical cancer)
|| Hereditary lymphedema
Procedure and diagnosis codes on Medical Policy documents are included only as a general reference tool for each policy. They may not be all-inclusive.
This medical policy was developed through consideration of peer-reviewed medical literature generally recognized by the relevant medical community, U.S. FDA approval status, nationally accepted standards of medical practice and accepted standards of medical practice in this community, Blue Cross and Blue Shield Association technology assessment program (TEC) and other non-affiliated technology evaluation centers, reference to federal regulations, other plan medical policies and accredited national guidelines.
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History From 2016 Forward
Annual review, no change in policy intent.
Annual review, no change to policy intent.
Annual review, no change to policy intent.