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Lymphedema
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Lymphedema
Epidemiology
Incidence
: Primary Lymphedema occurs 1 in 10,000
Pathophysiology
Lymphatic System
typically is responsible for 10-20% fluid return (remaining 80-90% is venous return)
Lymphedema is accumulation of protein-rich fluid in extracellular space
Types
Primary Lymphedema
Congenital Lymphedema
Lymphedema Congenita
Milroy's Disease
(Hereditary)
Pubertal onset of edema
Lymphedema Praecox
(Most common primary Lymphedema)
Middle age onset of edema
Lymphedema tarda (associated with injury)
Secondary Lymphedema (acquired lymphatic obstruction)
Tumor obstruction of Regional lymph nodes
Prostate Cancer
Lymphoma
Surgical excision or radiation to regional nodes
Breast Cancer
with axillary node dissection
Infection of regional lymph nodes
Filariasis
(Wuchereria bancrofti)
Tuberculosis
Signs
Early edema (protein-rich fluid accumulation)
Soft "puffy" extremity swelling
Maximal increase increase in girth in first year
Easily pits
Responds to limb elevation and compression
Involves distal extremity (e.g. dorsal foot)
See
Stemmer's Sign
(pathognomonic for Lymphedema)
Late edema (inflammatory fibrosis)
Woody, firm non-
Pitting Edema
(
Brawny Edema
)
Skin thickened and hyperkeratotic
Refractory to limb elevation and compression
Differential Diagnosis
Acute Conditions critical to exclude (e.g. emergency department)
Deep Vein Thrombosis
Cellulitis
Lymphangitis
Differential Diagnosis
Subacute and Chronic Conditions
See
Edema
Chronic Venous Insufficiency
Postphlebitic syndrome
Myxedema
(
Hypothyroidism
)
Lipedema
Seen in women with onset after
Puberty
Increased subcutaneous fat between
Pelvis
and ankle
Malignant Lymphedema
Rapid, painful cancer-related edema begins centrally
Distinguishing characteristics of early edema
Subcutaneous fibrosis (peau d'orange)
Stemmer Sign
(skin does not tent on dorsal digits)
Preferential swelling of foot dorsum
Involved extremity of squared-off digits
Complications
Recurrent
Bacteria
l
Cellulitis
(
Gram Positive Bacteria
)
Refractory Late
Edema
(Non-
Pitting Edema
)
Pain and decreased extremity range of motion
Severe Lymphedema (acute swelling >80%)
Requires hospitalization and possible surgical intervention
Lymphagiosarcoma (Stewart-Treves Syndrome)
Upper extremity soft tissue malignancy complicating chronic upper extremity edema
Rare complication of
Breast Cancer Management
with lymph node dissection (<0.45% of cases)
Presents as arm blue-purple
Macule
or
Papule
(or as bulla or
Cellulitis
)
Biopsy suspected lesions
Management
Gene
ral
Indicated in aggressive decongestive lymphatic therapy
Compression (Prevents late edema or
Brawny Edema
)
Precautions
Contraindicated in limb
Arterial Insufficiency
(except for low resting pressure wraps)
Confirm adequate padding
Observe for friction sites (risk of open sores)
Wraps (active edema reduction)
High resting pressure (ACE Wrap, Tubigrip)
Absolutely contraindicated in
Peripheral Vascular Disease
Mult-layer compression
Multiple-layer: Cotton layer, ace wrap, firm wrap)
Most effective compression for
Wound Healing
Combines high and low resting pressure
Low resting pressure (e.g. Rosidahl, Lymphedema wrap)
These wraps do not require a resting force (rely on muscle movement for return)
Ineffective, if calf muscle is not functional to assist return (use high resting pressure instead)
Typically safe to use in
Peripheral Arterial Disease
Elastic Support Garment
s (built-in pressure gradient)
Used for maintenance only (not for acute, active edema reduction)
Replace every 3-6 months with loss of elasticity
Mechanical Pneumatic Pressure Device
Indicated for severe edema
Applied at night or 2-3 times per week
Special Massage Techniques
Massage fluid proximally
Limb Elevation
Elevate above heart level for 30 minutes three times daily
Good skin hygiene
Keep web spaces between digits dry
Apply
Antifungal
powder
Avoid local injury or
Trauma
Avoid walking barefoot (especially outdoors)
Medications
Benzopyrones (Not available in U.S)
Topical coumarin (Not available in U.S.)
Avoid
Diuretic
s (minimal to no effect)
Observe closely for
Cellulitis
Management
Surgery
Excision of hypertrophic fibrotic subcutaneous tissue
Indicated for elephantiasis
Types of procedures
Charles Operation
Kondoleon Procedure
Lymphatic pedicle transfer
Supplies alternative lymph drainage
No proven efficacy
Microvascular bypass of obstructed lymph channels
Experimental
Resources
National Lymphedema Network Position Papers
http://www.lymphnet.org/lymphedemaFAQs/positionPapers.htm
References
Novotny (2017)
Wound
Care Update, Park Nicollet Conference, St Louis Park, MN (attended 9/15/2017)
Shelby (2015) Crit Dec Emerg Med 29(6): 2-8
Sabiston (1997) Surgery, Saunders, p.1574-7
Rockson (2001) Am J Med 110:288-95 [PubMed]
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