Lymphedema

Lymphedema is a chronic condition that develops over months to years due to a physiological imbalance of blood flow and lymphatic drainage and results in an accumulation of protein-rich fluid in the extremities. Lymphedema is classified into primary and secondary forms. Primary lymphedema refers to inherited causes in which females have a higher incidence. Secondary lymphedema is a result of damage or obliteration of the lymphatic system caused by either surgery, radiation therapy, infection, or trauma. Although lymphedema may be clinically apparent, imaging is required to confirm the diagnosis and to rule out other conditions.

Conservative therapy is the main stay of treatment comprised of manual lymph drainage, physical exercise, skin care, compression therapy and compression garments. When conservative therapy fails and lymphedema becomes chronic, refractory, and nonpitting, surgical methods may be medically necessary to improve lymphatic drainage, such as liposuction, lymphovenous bypass or lymph node transplant.

Clinical Guideline Coverage Criteria

Lipectomy or Liposuction

The plan considers liposuction (including suction-assisted protein lipectomy (SAPL), also referred to as suction lipectomy), as medically necessary for the treatment of lymphedema when ALL of the following criteria below are met.

Surgical Treatments for Lymphedema and Lipedema

1. Patient meets All of the following diagnostic criteria:
   • A certified lymphedema therapist confirms a diagnosis of lymphedema by symptoms and findings and documents the Member has International Society of Lymphology stage ≥ II lymphedema (ISL)
   • A diagnosis of lymphedema by One of the following diagnostic measurements:
     1. Unilateral disease
        • Volumetry differential (circumferential measurements and/or perometry Differential) >10% for affected dominant extremity or >7% for affected non-dominant extremity; or
     2. Bilateral disease
        • Lymphoscintigraphy findings must show a minimum of a one-hour delayed transit time to first- level lymph nodes, axillary lymph nodes (upper extremity lymphedema) or inguinal lymph nodes (lower extremity lymphedema), or dermal back flow.
2. Patient meets ALL of the following criteria:
   • Results of MRI imaging show moderate to severe fat hypertrophy
   • BMI ≤ 35g/m2
   • Failure to respond to at least 6 months of optimal conservative treatment including compression therapy with bandaging, garments, or gauntlet and any of the following therapies: lymphoedema-specific manual lymphatic drainage, skin care, physical therapy, and exercises for lymphedema
   • There is documentation of One or more of the following:
     • Pain or sense of heaviness or discomfort in the limb; or
     • Restricted range-of-motion and functional limitation (difficulty ambulating or performing activities of daily living); or
     • Recurrent episodes of infection/cellulitis
   • The plan of care postoperatively is to continue to wear compression garments as instructed to maintain the benefits of surgery
3. Patient has NONE of the following:
   • Active comorbid condition(s) that would impede healing (i.e., untreated, or uncontrolled cancer, venous occlusive disease, active infection of the extremity)
   • Transient lymphedema
   • Lipedema without lymphatic dysfunction
   • Not compliant with wearing compression garments continuously and/or has not demonstrated the ability to tolerate compression therapy or physical therapy sessions
   • Pregnancy
4. Planned surgery to be performed at a certified lymphedema center of excellence

Vascularized Lymph Node Transplant (VLNT)

The plan considers lymph node transplant as medically necessary for the treatment of lymphedema when ALL of the following criteria below are met.

1. Patient meets ALL of the following diagnostic criteria:
   • A certified lymphedema therapist confirms a diagnosis of lymphedema by symptoms and findings and documents the Member has International Society of Lymphology stage ≥ II lymphedema (ISL)

2. Patient meets ALL of the following criteria:
   • BMI ≤ 35g/m2
   • Failure to respond to at least 6 months of optimal conservative treatment including compression therapy with bandaging, garments, or gauntlet and any of the following therapies: lymphoedema-specific manual lymphatic drainage, skin care, physical therapy, and exercises for lymphoedema
   • There is documentation of ONE or more of the following:
     1. Pain or sense of heaviness or discomfort in the limb; or
     2. Restricted range-of-motion and functional limitation (difficulty ambulating or performing activities of daily living); or
     3. Recurrent episodes of infection/cellulitis
   • The plan of care postoperatively is to continue to wear compression garments as instructed to maintain the benefits of surgery
3. Patient has NONE of the following:
   • Active comorbid condition(s) that would impede healing (i.e., untreated, or uncontrolled cancer, venous occlusive disease, active infection of the extremity)
   • Transient lymphedema
   • Lipedema without lymphatic dysfunction
   • Not compliant with wearing compression garments continuously and/or has not demonstrated the ability to tolerate compression therapy or physical therapy sessions
   • Pregnancy
4. Planned surgery to be performed at a certified lymphedema center of excellence

Lymphovenous Bypass

The plan considers lymphovenous bypass as medically necessary for the treatment of lymphedema when all of the following criteria below are met.

1. Patient meets ALL of the following diagnostic criteria:
   • A certified lymphedema therapist confirms a diagnosis of lymphedema by symptoms and findings and documents the Member has International Society of Lymphology stage ≥ I lymphedema (ISL)
   • A diagnosis of lymphedema by ONE of the following diagnostic measurements:
     1. Unilateral disease
        • Volumetry differential (circumferential measurements and/or perometry Differential) >10% for affected dominant extremity or >7% for affected non-dominant extremity; or
        • Lymphoscintigraphy shows at least a one-hour delayed transit time to first-level lymph nodes, axillary lymph nodes (upper extremity lymphedema) or inguinal lymph nodes (lower extremity lymphedema), or a dermal back flow pattern
     2. Bilateral disease
        • Lymphoscintigraphy findings must show a minimum of a one-hour delayed transit time to first- level lymph nodes, axillary lymph nodes (upper extremity lymphedema) or inguinal lymph nodes (lower extremity lymphedema), or a dermal back flow pattern
2. Patient meets ALL of the following criteria:
   • BMI ≤ 35g/m2
   • Lymphatic channels present by ICG lymphangiography
   • Failure to respond to at least 6 months of optimal conservative treatment including compression therapy with bandaging, garments, or gauntlet and any of the following therapies: lymphoedema-specific manual lymphatic drainage, skin care, physical therapy, and exercises for lymphoedema
   • There is documentation of ONE or more of the following:
     • Pain or sense of heaviness or discomfort in the limb; or
     • Restricted range-of-motion and functional limitation (difficulty ambulating or performing activities of daily living); or
     • Recurrent episodes of infection/cellulitis

3. Patient has NONE of the following:
   • Active comorbid condition(s) that would impede healing (i.e., untreated, or uncontrolled cancer, venous occlusive disease, active infection of the extremity)
   • Transient lymphedema
   • Lipedema without lymphatic dysfunction
   • Not compliant with wearing compression garments continuously and/or has not demonstrated the ability to tolerate compression therapy or physical therapy sessions
   • Pregnancy
4. Planned surgery to be performed at a certified lymphedema center of excellence*

Point32Health companies³ Surgical Treatments for Lymphedema and Lipedema

Note: Medical documentation must include all of the following: diagnosis, duration and onset of symptoms, relevant medical and surgical history including any history of prior infections and cellulitis, non-surgical treatment tried, level of functional impairment; specific procedure requested and treatment plan (inclusive of post-operative plan of care).

*Lymphedema Staging (International Society of Lymphology)

Description

• Stage 0 (subclinical) Swelling is not evident, and most patients are asymptomatic despite impaired lymphatic transport
• Stage I (mild) Accumulation of fluid that subsides (usually within 24 hours) with limb elevation: soft edema that may pit, without evidence of dermal fibrosis
• Stage II (moderate) Does not resolve with limb elevation alone; limb may no longer pit on examination
• Stage III (severe) Lymphostatic elephantiasis; pitting can be absent; skin has trophic Changes

**The Lymphatic Education & Research Network (LE&RN) is a non-profit organization dedicated to education, research and advocacy related to lymphatic diseases (LD). For more information about the LE&RN international standards for best practice multi-disciplinary care in the management of LD and a listing of institutions designated as centers of excellence refer to: https://lymphaticnetwork.org/centers-of-excellence.

Limitations

• Liposuction for lipedema will not be covered when performed for cosmetic purposes
• Immediate lymphatic reconstruction (e.g., Lymphatic Microsurgical Preventing Healing Approach [LYMPHA]) for prophylactic purposes) is considered investigational