Abstract

Conservative therapy

Complex Decongestive Therapy (CDT)

Complex decongestive therapy (CDT), also known as Combined Physical Therapy (CPT) or Complex Decongestive Physiotherapy (CDP) (among others), is the standard of care for the management of lymphedema, although data endorsing this concept are lacking (1,2) It is a noninvasive and cost-effective therapy that effectively reduces limb volumes in both upper and lower extremity lymphedema. [1]

• In the U.S., usually physical or occupational therapists administer lymphedema treatments. Massage therapists, nurses and physicians can also perform certain kinds of lymphedema interventions if they have been specially trained in lymphedema. [2]
• The beneficial effects of CDT are thought to occur secondary to improved collateral lymphatic drainage, increased lymphatic pumping function and reduced fibrogenesis [3].
• Indications for CDT therapy are: high clinical suspicion of lymphedema, or the inability to rule out other causes of disease in patients with low clinical suspicion of lymphedema [4].
• CDT generally involves a two-stage treatment program [5] [6] [1]:
• Intensive CDT: initial treatment phase combining 4 elements of therapy: compression therapy typically with multicomponent bandages, manual lymphatic drainage (MLD), therapeutic exercise, and skin/nail care. Ideally, phase one is administered 1 or 2 times a day, every day for 4 to 6 weeks. [2] Weekly volumetric limb assessments are conducted at the end of each week to determine if volume reduction occurred or if a plateau has been reached.
• Maintenance CDT: second phase initiated after the patient’s response to intensive CDT has plateaued as a life-long therapy. Involves self-care to maintain limb size, with low-stretch compression garments, continued exercise, self-skin/nail care, and self-MLD, as needed [5].

Elements of intensive CDT

• Compression therapy
• Compression therapy is the mainstay of treatment and is associated with a significant reduction in swelling in lymphedema patients [7]
• Compression therapy exerts its therapeutic effects by providing constant physical pressure that reduces lymphatic filtration and increases lymphatic transport capacity [7]
• Typically, inelastic multicomponent compression bandage systems are indicated in the initial phase of CDT, followed by graduated compression stockings in the maintenance phase. [8] Brands include Lymphsets*, Lymph kits*, Rosidal® sys, Coban® 2 and Coban® 2 Layer Lite (cohesive) Actico®+ Sofban® (cohesive) [8]. This practice is supported by an RCT that showed that more sustained limb-volume reduction was achieved with the former as an initial phase of treatment for lymphedema patients, followed by hosiery, than hosiery alone. [8] [9]
• Rationale for using inelastic (high stiffness systems) include the fact that these bandages have a low, well tolerated resting pressure and a high working pressure. That is, stiff bandages do not give way during exercise and create a ‘massaging effect’ during muscle contractions, facilitating lymph and venous return. [10]
• See Compression Therapy (add link)
• Even non-ambulatory patients may be candidates for inelastic multicomponent bandage systems, despite the thought that these patients need elastic compression devices. In the initial phase, inelastic systems applied with high enough pressure by trained staff will lead to a fast reduction of leg volume, requiring change twice or three times a week as the limb shrinks in size. Later, weekly bandage changes may be sufficient and the patient and family-members should be instructed to move, actively or passively, as much as possible. In addition, intermittent pressure pumps may support this strategy. [10]
• As for pressure levels, it is generally recommended that the highest pressure tolerated be used, generally between 20-60 mm Hg [5]. An international expert committee [10] recommends an upper limit of 30 mmHg for upper extremities and around 50-60 mmHg on the lower extremity, as there is an upper limit beyond which further increase of compression pressure seems counterproductive.
• If arterial disease is present, pressure levels should be modified. Several algorithms exist outlining how to use ankle-brachial pressure index (ABPI) or a handheld Doppler ultrasound to screen for underlying arterial disease and select adequate compression therapy pressure levels (https://woundreference.com/app/topic?id=how-to-select-compression-therapy#algorithms). If an ABPI measurement cannot be reliably obtained due to significant edema or limb size, an arterial Duplex ultrasound should be used instead to stage arterial disease and modify pressure levels.
• In practice, compression pressure levels are not measured. Pressure measuring devices are typically used for training purposes and research to estimate the pressure applied by compression bandages and garments to adjust pressure settings to adequate levels [11] [12].
• General recommendations for compression therapy specific to the initial phase of CDT include :
• Multi-component wrapping is preferred over single-layer wrapping, and should be carried out by trained professionals as incorrectly applied bandages can be useless or harmful [5].
• Unlike venous disease, which requires compression mainly during the day when patients are upright, lymphedema patients should preferably wear compression day and night [10]
• Lymphedema may alter limb shapes and cause deformities due to a peculiar distribution of lymphedema, fat and fibrosclerotic tissue. Reshaping the contour of the extremity may be achieved by molding the bandage to the limb in an individual manner while trying to avoid too much padding. [10] Prominent bony areas and popliteal and cubital fossae may be protected with foam padding [6].
• Bandages manufactured with velcro or pull-on technology might relieve patients of bandaging challenges and improve adherence [5].
• After reduction of leg volume is no longer observed, patient is transitioned to the maintenance phase of CDT.
• Manual lymphatic drainage (MLD)
• Lymphatic massage moves lymph fluid from the distal limbs to proximal regions where lymphatic flow is not obstructed.
• MLD is safe and may offer additional benefits to patients already on an intensive course of compression bandaging for mild-to-moderate BCRL [13].
• Once swelling is reduced with MLD, patients are more likely to maintain reduced swelling if they continue to use a custom-made compression sleeve [13].
• Some healthcare centers have developed guidelines for participation in MLD programs [14]. These include:
• No uncontrolled medical conditions that might affect the safety of MLD
• Ability to ambulate
• Ability to transfer to and from the MLD table
• Patient weight not exceeding the limit of the MLD table
• Patient BMI not exceeding 70
• Commitment to maintaining constant weight or losing weight for the duration of MLD therapy
• Exercise
• Muscle contractions increase lymphatic and vascular flow throughout the body, decreasing lymphatic fluid stasis.
• Progressive resistance training with weightlifting is shown to decrease the risk of lymphedema progression while posing minimal patient risk [15]
• Exercise can result in a significant improvements in shoulder range of motion in BCRL patients, with greater effects seen when exercise is begun earlier in the postoperative period [16].
• Skin and nail care
• Maintenance of skin integrity and nail cleanliness reduces the risk of inflammatory and infectious complications of lymphedema [6].
• In addition to treatment of skin infections, proper skin care includes daily hygiene using pH neutral soaps, use of emollients and sunscreen, avoidance of scented products, and skin monitoring for cuts and bites [7] [6]

Elements of maintenance CDT

Besides continued exercise, self-skin/nail care, and self-MLD, expert committees recommend life-long use of graduated compression stockings (also known as hosiery, sleeves or garments) for lymphedema maintenance therapy to maintain lymphedema reduction after the initial phase of CDT. [8] [5]

• Preferably these garments should be prescribed by a qualified provider and custom made with correctly-obtained specific measurements to avoid inappropriate usage in a patient with medical contraindications such as arterial disease, painful postphlebitic syndrome or occult visceral neoplasia. Generally the highest compression level tolerated (~20-60 mmHg for lower extremities) by the patient is likely to be the most beneficial. Flat-knit garments are preferred by some clinics while others use both flat and round-knit garments (or combination). [8] [5])
• Hosiery should be worn during exercise [6] and replaced every 3-6 months [7]

Figures 1-2: Skin appearance before (left) and after (right) intensive phase of CDT. Intensive phase of CDT consisted of Unna boots wrapped with 2 layers of Coban, applied at the first visit and changed after 48 hours, then changed weekly for 5 weeks. Circaid garment compression was applied simultaneously until the skin started weeping again, usually after 6-12 months of use. Reduced erythema, hyperkeratosis and exudation in posterior calf are noted following therapy.

Compression therapy alone

• Recommended to decrease lymphedema symptoms, even when not a component of CDT [10]

Pneumatic compression devices (PCDs)

• Clinical practice guidelines [17]
• PCDs are recommended in the short term treatment of BCRL as part of a combined lymphedema treatment program.
• However, in the routine management of lymphedema with PCDs has not been shown to provide additional benefits to decongestive therapy for lymphedema of the lower extremity.
• There is a lack of studies comparing the clinical effectiveness of single- vs multi-chamber PCDs.
• Prospective data [18]
• PCDs are associated with reduced limb volumes and improved quality of life without adverse effects in the management of lower extremity lymphedema.
• Financial considerations [19]
• Treatment with PCDs is associated with improved clinical outcomes and reduced healthcare costs compared to untreated patients with lymphedema.

Some patients that's the only intervention - eg calciphylaxis - morbidly obese - 

Pharmacological therapy

• Benzopyrones [20]
• No clear evidence supports the use of benzopyrones such as flavonoids, oxerutins, escins, coumarin and ruscogen combined with hesperidin for lymphedema management [20].
• Coumarin has been associated with significant hepatotoxicity, while flavonoids have been shown to reduce swelling in some studies [10].
• Diuretics
• Expert committees [5] [10] recommend clinicians reserve use of diuretics only for specific patient populations, namely for patients with:
• Effusions in body cavities (e.g., ascites, hydrothorax),
• Protein-losing enteropathy
• Peripheral lymphedema from malignant lymphatic blockage, who may benefit from a short course of diuretic drug treatment.
• Intolerance to fluid overload, such as those with congestive heart failure, cirrhosis, nephrotic syndrome and older adults in whom enhanced lymphatic drainage as a result of lymphoedema therapy precipitates cardiac failure
• For these patients above, a short course or temporary increase in the dose of diuretics can be administered during the first week of compression therapy. Dose can be reassessed 24-48 hours after initiation of compression therapy.

skin substitutes

approved for non-healing VLU - puraply, apligraf, 

References

Bibliography

PICO Questions

• PICO: Should lymphedema wounds be debrided? If so, is one method better over the others?

• a) Summary of evidence
• Systematic reviews, meta-analyses, and guidelines
• None directly relating to lymphedema
• RCTs
• None directly relating to lymphedema
• Papain/urea combination enzymatic debridement is significantly more effective than collagenase or DNase alone for pressure ulcer debridement [21].
• Reviews
• Both chemical and surgical debridement can work in lymphedema. Proteolytic enzyme debridement lowers the risk of infection compared to surgical debridement, but surgical debridement is faster [22].
• Case study
• None directly relating to lymphedema ulcers
• Papain chemical debridement using OPAL A successfully treated skin hyperkeratosis associated with chronic pressure ulcer [23].
• Daily application of papain-based filtrate or cream using OPAL001 resulted in improved healing of pressure ulcers [24].
• b) Patient/clinician preference
• c) Harms/benefits
• d) Use of resources

• Indications for referral to a lymphedema service [6]:
• • Special groups: midline lymphedema (head and neck, breast, abdomen, genitalia), primary lymphedema, swelling of unknown origin
  • Complicating factors: comorbid arterial disease, comorbid venous insufficiency, severe skin changes, severe deformity, chyle disease, neuropathy, sudden increase in pain or swelling
  • Management challenges: compression garments not fitting, failure to response to three months of treatment, worsening wounds, recurrent skin infection

https://upload.wikimedia.org/wikipedia/commons/8/8f/Pathological_Steps_of_Cancer_Related_Lymphedema%2C_Histological_Changes_in_the_Collecting_Lymphatic_Vessels_after_Lymphadenectomy1.png

Coding and Documentation

ICD-10 coding

Hereditary lymphedema, Q82.0 (https://www.icd10data.com/ICD10CM/Codes/Q00-Q99/Q80-Q89/Q82-/Q82.0)

• Chronic hereditary lymphedema
• Congenital elephantiasis
• Congenital lymphedema
• Familial lymphedema
• Hereditary lymphedema of the legs
• Idiopathic lymphedema
• Meige’s disease/syndrome
• Milroy’s disease/syndrome
• Nonne-Milroy-Meige disease/syndrome
• Pseudoelephantiasis neuroarthritica
• Trophedema
• Tropholymphedema

Postmastectomy lymphedema syndrome, I97.2 (https://www.icd10data.com/ICD10CM/Codes/I00-I99/I95-I99/I97-/I97.2)

• Complication from surgical procedure on elephantiasis or lymphedema, postmastectomy
• Complication of lymphedema after mastectomy
• Elephantiasis (nonfilarial) due to mastectomy
• Elephantiasis (nonfilarial), postmastectomy
• Lymphatic edema due to mastectomy
• Lymphedema acquired postmastectomy
• Obliteration of lymphatic vessel due to mastectomy

Lymphedema, not elsewhere classified, I89.0 (https://www.icd10data.com/ICD10CM/Codes/I00-I99/I80-I89/I89-/I89.0)

• Compression of lymphatic vessel
• Dilatation of lymphatic vessel
• Edema due to lymphatic obstruction
• Edema, lymphatic
• Elephantiasis (nonfilarial), lymphangiectatic
• Elephantiasis (nonfilarial), lymphatic vessel
• Elephantiasis (nonfilarial), glandular
• Elephantiasis (nonfilarial), scrotum
• Elephantiasis (nonfilarial), streptococcal
• Elephantiasis (nonfilarial), telangiectodes
• Lymphangiectasis, postinfectional
• Lymphangiectasis, scrotum
• Lymphangiectatic elephantiasis
• Lymphectasia
• Lymphedema praecox
• Lymphedema secondary
• Obliteration of lymphatic vessel
• Obstruction of lymphatics
• Obstruction of thoracic duct
• Occlusion of lymph or lymphatic channel
• Occlusion of thoracic duct

Other specified noninflammatory disorders of vulva and perineum, N90.89 (https://www.icd10data.com/ICD10CM/Codes/N00-N99/N80-N98/N90-/N90.89)

• Deformity of clitoris, acquired
• Deformity of labium, acquired
• Deformity of vulva, acquired
• Disease of vulva, not elsewhere classified
• Edema of vulva
• Elephantiasis (nonfilarial) of vulva
• Hydrocele of vulva
• Lesion (nontraumatic) of vulva
• Necrosis of perineum
• Necrosis of vulva
• Scar of labia
• Scar of vulva
• Sclerosis of corpus cavernosum, female

Elephantiasis of eyelid, H02.85 (https://www.icd10data.com/ICD10CM/Codes/H00-H59/H00-H05/H02-/H02.85)

Other postprocedural complications and disorders of the circulatory system, not elsewhere classified, I97.89 (https://www.icd10data.com/ICD10CM/Codes/I00-I99/I95-I99/I97-/I97.89)

• Complication of circulatory system, postprocedural, not elsewhere classified
• Complication from surgical procedure on elephantiasis
• Complication from surgical procedure on lymphedema
• Elephantiasis (nonfilarial), surgical
• Lymphedema (acquired), surgical, not elsewhere classified

Documentation needed on the chart to support ICD-10 related to lymphedema

• A complete history and physical examination documented in the patient’s chart is necessary to clinically diagnose lymphedema. This can be facilitated by using the Lymphoedema Assessment Form developed by the International Lymphoedema framework *can insert link to form* [10].
• Medicare criteria for the coverage of pneumatic compression devices in the management of lymphedema require the following to be included in the patient’s medical record: [25]
• • Documented diagnosis of lymphedema
  • Objective findings to establish lymphedema severity
  • Documentation that the patient has completed 4 weeks of conservative therapy
  • Documentation that the patient continues to have significant symptoms
  • Physician oversight of all phases of treatment

Coverage and Reimbursement

CPT codes - A/B Mac

HCPCS codes - DME - compression

Outpatient setting:

services - provider visit, covered.

OT/PT visit for CDT- Medicare covers OT/PT visits, but there is a monetary cap to outpatient services. https://f1000.com/work/item/5817071/resources/4861970/pdf

Medically necessary hands-on MLD is a covered Medicare service and is coded using CPT 97140 for manual therapy. There is no Medicare coverage for LYMPHEDEMA compression bandage application as this is considered to be an unskilled service. Medicare will however cover a brief period (e.g. three or fewer sessions if no new specific issues are identified), of patient/caregiver instruction in compression bandaging home management. Medical necessity for this education must be clearly documented and meet the code descriptor requirements for CPT 97535. Minutes spent applying compression bandaging without patient/caregiver education should not be billed as skilled therapy services. Noridian article

Providers should note that the treatment of LYMPHEDEMA with the application of high compression bandage systems continues to be non-covered by Medicare. However, a brief period, i.e. three or fewer sessions if no new specific issues are identified, of patient and/or caregiver education for home management of LYMPHEDEMA with compression wrap applications may be medically necessary and reimbursable. Medical necessity for the education must be clearly indicated in the patient's record and must meet the code descriptor requirements for CPT 97535, supporting home management training.

Following review of the current literature, the practices of our providers, and the January 2012 implementation of the specific CPT codes describing the application of multi-layered compression bandage systems, Noridian will cover and separately reimburse the costs of the following procedures for non-LYMPHEDEMA applications that meet Medicare coverage requirements:

• 29581 - Application of multi-layer compression system; leg (below knee), including ankle and foot
• 29584 - upper arm, forearm, hand, and fingers

Noridian

DME - Treatment for lymphedema includes use of compression. There are three types of compression. bandages, garments, night time bandage alternatives. Medicare does not currently pay for any type of compression when used for lymphedema.

Sequential compression pumps ?

Summary - outpatient setting

https://f1000.com/work/item/5817071/resources/4861970/pdf