Transcript Lymphedema - Middlesex Hospital

Lymphedema
Jeffrey Takahashi, M.D.
Center for Vein Restoration
Regional Medical Director
Lymphedema
I have no disclosures
LYMPHEDEMA
Abnormal collection of interstitial
lymph fluid due to either
congenital maldevelopment of
the lymphatic system or to
secondary lymphatic dysfunction
A chronic debilitating disease
that is frequently
misdiagnosed, treated too
late, or not treated at all
Leg Swelling: Causes
Renal failure/Nephrotic synd.
CHF
Cardiomyopathy
Cirrhosis
DVT/VTE
Venous Insufficiency
Lymphedema
Trauma
Cellulitis
Baker’s cyst
Bursitis
Arthritis
Pregnancy
Lymphedema: An Orphan disease
Wound care centers
 PT/OT centers
 Vascular surgery
 Orthopedic surgery
 Interventional radiology
 Vein centers
 Family practice/Internal
medicine
 Lymphedema centers

Lymphedema: Epidemiology
300 million people worldwide (app. 1.4 out of 1,000
people)
 3-11 % of the world’s population
 Female > male
 True incidence in the US is not known as
lymphedema is often not recognized but there are at
least 220,000 documented cases of lymphedema in
the US

Nicolaides AN Circulation 2000 102;e126-163
Lymphedema:
A System Failure
Heart
Pancreas
Kidneys
Brain
Ovary
Adrenal gland
Lymphatic System
CHF
Diabetes
Uremia
Dementia
Infertility
Addison’s
LYMPHEDEMA
Lymphatic System: Function
 Removes
excess interstitial fluid from body
tissues and returns it to the vascular system
 Absorption of fatty acids and subsequent
transport of fat, as chyle from the digestive
system to the venous system
 Defense against invading microorganisms
 Pathway for cancer spread (Breast/Melanoma)
Lymphatic Drainage
 Relies
on contraction of the subjacent
skeletal muscles
 Rhythmic contractions of the
lymphatic vessel wall smooth muscle
(“lymphatic pump”)
 Respiratory assisted
 Stimulated by the sympathetic
nervous system
 One way valves prevent backflow
Lymphedema
NORMAL
CVI/DVT
BCRL
Lymphatic Anatomy
Lymphatic Anatomy
Lymphatic vessel
Primary Lymphedema
At birth or under 1 year:
Lymphedema congenita; if familial
Milroy’s disease
Between 1 and 35 years:
Lymphedema praecox; Meige’s
disease; most common
After 35 years:
Lymphedema tarda
Primary Lymphedema
Aplasia – the lymphatic channels have not been developed at all
and are absent. This is usual in Milroy’s Disease.
Hypoplasia – underdeveloped lymphatics with the vessels and
lymph nodes being few in number and/or with a smaller diameter
than normal. Hypoplasia is the most common type of abnormality
of the lymphatics in primary lymphedema.
Hyperplasia – there is an excessive number of lymphatics with
impaired function due to them often being enlarged or distorted
(similar to varicose veins) so that normal lymph flow is
hampered.
Syndromes associated with
Lymphedema
Turners syndrome
 Klinefelter syndrome
 Trisomy 21
 Noonan syndrome
 Klippel Trenauny Webber
syndrome

Lymphedema Distichiasis
Biomarkers in Lymphedema
FLT-4 gene (Milroy’s disease)
 FOX C2 (Meige’s disease; tarda)
 SOX 18
 VEGF-3
 EGF

Secondary Lymphedema
Causes:
 Trauma-Operative
 Inflammation
 Infection/Filariasis
 Chonic Venous Insufficiency (CVI)
 Chronic Venous Obstruction (DVT)
 Radiation treatment
 Malignancy
 Medications
Cancer Related
Lymphedema (CRL)
Affects up to 50% of patients who
undergo lymph node dissection
(axillary and groin)
Highest incidence in breast cancer
patients (BCRL) but also seen with bladder
gyneocologic, prostate, head and neck
cancers and melanoma
Lymphedema may not present itself
until many years after the
surgery/LND/XRT
Incidence increases with time after surgery
25-50,000 new cases are diagnosed
annually in the US
Filariasis
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Most common cause of
lymphedema worldwide
More prevalent in Africa, India,
and South America
Caused by Wuchereria
Bancrofit and spread by a
mosquito
Causes proximal lymphatic
obstuction by the adult
parasites and associated
lymphatic inflammation due to
immune response of the host
Filariasis
ISL Staging
ISL Stage 0:
Subclinical- no edema detectable
ISL Stage I:
Tissue fluid accumulation subsides with limb
elevation; tissue is soft
ISL Stage II:
Elevation alone rarely reduces swelling, pitting
is present; tissue becoming firm
ISL Late Stage II:
Tissue fibrosis becomes evident, even if pitting
is
present
ISL Stage III (lymphostatic elephantiasis):
Tissue is fibrotic and pitting is absent; skin
changes are prominent; pronounced skin folds,
fat deposits, verrucous growths
Lymphedema: On the Rise
Incidence is increasing:
 Increased rate of obesity
 Increased use of radiation
therapy
 Aging population
 Improving survival from
breast cancer (BCRL)
 Increased awareness
History
Filariasis
Cancer
Surgery
Radiation Rx
Familial
Venous Stasis
Trauma
Medications
Travel
Obesity
DVT
Hypoalbumenia (DDX)
Renal failure (DDX)
Hypercholesterolemia (DDX)
Physical Exam
Extremities
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edema (pitting/non pitting)
symmetry
foot involvement (Stemmer)
Nodes (enlarged)
Appearance of the skin
 venous stasis changes
 thickening
 ulcers
 cellulitis/erythema
Sausage Toes
Kaposi Stemmer Sign
Pitting Edema
Medications Associated
with Lymphedema

Calcium channel blockers (Nifedipine,
Amodipine, Felodipine)
 Steroids –less with NSAIDS
 Cytokines (Interferon/Interleukin)
 Chemotherapeutics (Tamoxifen,
Megestrol, Anastrazole)
Laboratory Tests
 EKG
 Thyroid
panel
 CBC
 Serum
albumin/Protein
 Electrolytes/BUN/Cr
 Serum aldosterone/renin
 LFTs
 UA
Imaging for Lymphedema
Lymphoscintigraphy
 Computed Tomography (CT)
 MRI
 Lower Extremity Duplex/IVUS
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DVT/Obstruction
Venous insufficiency
* If needed: Cardiac echo, CTV, MRV, Venogram
Lymphangiogram
Radionucleotide
Lymphoscintigraphy
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Tc 99m SC (filtered)
Web space x 2
Exercise and specific timed imaging
Delay to nodes > 40-60 min
Dermal activity (“backflow”)
Collaterals
Evaluate for obstruction
Determine relative flow rates
Predicts response to CDT for breast CA
Radionucleide
Lymphoscintigraphy
MR Lymphography
Dynamic Indocyanine green
Lymphography (ICG)
Sensitivity of Imaging for Early
Lymphedema
ICG Lymphography
MR Lymphography
Lymphoscintigraphy
CT
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Phlebolymphedema
VENOUS LYMPHEDEMA
 Secondary lymphedema that develops due to
the inability of the lymphatic system to
adequately drain the interstitial fluid that
develops from chronic venous hypertension
 Manifested by skin changes seen with venous
stasis such as hyperpigmentation,
lipodermatosclerosis and edema
How Common is Venous
Lymphedema?
Single center retrospective study of 365 patients with
lymphedema
 35 venograms performed in patients suspected of
having phlebolymphedema based on clinical exam
 Venous obstruction found in 17 patients
 5% of patients found to have venous obstruction
requiring stent placement and further endovascular
vein ablation
 90% of patients had significant improvement in edema
and function of the extremity
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Szuba A et. Al JVIR 2002 12(8):799-803
Venous lymphedema
Venous insufficiency may mask signs of
lymphedema
 Lymphedema may mask signs of venous
insufficiency
 Obtain Doppler US and +/- CT venogram to
evaluate for venous insufficiency or obstruction
 Treat the venous insufficiency or obstruction first
(ablation, sclerotherpay, venoplasty, stenting, etc.)
and reassess
 Refer to an CVI specialist to excluded venous
insufficiency or obstruction
 Refer to certified lymphedema specialist
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Diagnosing Venous Lymphedema
Chronic Lipodermatosclerosis
Venous Lymphedema
Acute Lipodermatosclerosis
Lymphedema Rubra
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Blanching,
erythematous
skin
No cellulitis
No fever
Does not
progress over
time
h/o being
treated multiple
times for
infection
Lipidema
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“Painful fat syndrome”
More common in females
 Bilateral, usually symmetrical fatty tissue excess
 Mainly in hip/torso and legs
 Worsens with prolonged standing
 Exacerbated by weight gain and improved by weight
loss
 Ring of fat above the ankle with the feet unaffacted
(negative Stemmer sign)
 Can coexist with lymphedema (“Lipolymphedema”)
 Less responsive to compression bandaging

Lipidema
Stewart Treves Syndrome
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Rare, deadly angiosarcoma
Overall survival only 10-20%
Arises in the setting of
chronic lymphedema
Most commonly seen in
women following breast
cancer surgery with ALND
Rarely seen in the lower
extremities
Complex Decongestive
Therapy (CDT)
Manual lymphatic drainage (MLD)
Multiplayer short stretch compression bandaging
Lymphedema specific exercise
Skin and nail care
Weight loss education
Education in lymphedema self-management and
compression garment use
Goals of Complex Decongestive
Therapy (CDT)
Gold Standard of treatment for Lymphedema
 Decrease limb volume (swelling)
 Increase lymph drainage from the congested area
 Reduce skin fibrosis
 Prevent/arrest progression to more advanced stages
 Enhance patient’s functional status and quality of life
 Relieve discomfort
 Reduce the risk of cellulitis
 Reduce the risk of Stewart Treves Syndrome (rare)
Treatment Goals
 Reduce Risk
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 Detect Early
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 Arrest and
Reduce
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 Maintain Gains
Complications
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CDT
Manual lymphatic drainage
Compression bandaging
Decongestive exercise
Skin and nail care
Early Intervention
Benefits of Early Intervention
More
treatment options at Stage I
Better position to prevent progression to more
advanced stages (II and III)
Better position to downstage (II to I)
Reduction in skin breakdown/fibrosis
Decreased rate of cellulitis
Increased patient desire to continue treatment/patient
compliance (drop off)
Improved QOL and patient satisfaction
Reduction in rate of out-patient and in-patient hospital
visits
Reduced lymphedema related costs for patient and
health care system ($3 billion/year)
Hamner JB Ann of Surg Oncol 2007; 14:1904-8
Medicare: LCD Guidelines for
PCD
The following pre-Existing Conditions must be met for
Pneumatic Compression Device (PCD):
A. Open venous leg ulcer for 6 months without
significant improvement after appropriate
wound care (MD evaluation and signature).
B. Cancer history involving surgery/radiation
impairing lymphatic vessels (+/- post
mastectomy)
C. Primary lymphedema; e.g. Milroy’s disease
(congenita), Meige’s disease (praecox), and
late onset (tarda)
Medicare: LCD Guidelines
Lymphedema must be “severe” enough to warrant use
of a PCD. AT LEAST ONE of the following symptoms
must exist:
1. Hyperkeratosis (fibrosis)
2. Hyperplasia
3. Hyperpigmentation
4. Papillomas
5. Skin breakdown with lymphorrhea (weeping)
Medicare: LCD Guidelines
Documentation including measurements of
FAILED CONSERVATIVE THERAPY:

Measurements prior to conservative therapy
and another set of measurements after at least
4 weeks of conservative therapy
 Evaluation and documentation by MD that
symptoms are “significant” and still remain after
the 4 weeks of conservative therapy.
Short vs Long
Stretch Bandaging
Multilayer Short
Stretch Bandaging
Working vs Resting Pressures
Pneumatic Device Selection
Simple (Basic) Devices:
Single to multi chamber (4-8 cells);
higher pressures (30-60mm Hg); e.g.
ActiTouch
Advanced Devices:
Multi chamber (up to 32 cells),
programmable; lower pressures (9-15
vs 15-30 mm Hg) ; e.g. FlexiTouch
Simple Pneumatic Compression
Devices
ACTitouch®
Entre
Advanced Pneumatic Compression
Devices
Flexitouch®
Flexitouch® System: A Clinically Proven Advanced
PCD Therapy
•
Up to 32 curved chambers direct fluid away
from affected areas toward healthy lymph nodes
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Delivers low pressure through gentle “work and
release” mechanism of action
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Offers truncal clearance, followed by distal to
proximal treatment of limbs
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Demonstrated high patient compliance &
satisfaction8
Flexitouch is the only PCD clinically
proven to stimulate the lymphatic system9
8. Ridner, S.H. et al. (2008) Home-Based Lymphedema Treatment in Patients with and Without Cancer-Related Lymphedema. Oncology Nursing Forum. July 2008; Vol.
35(4): 671–680.
9. Adams, K.E. et al. (2010) Direct Evidence of Lymphatic Function Improvement After Advanced Pneumatic Compression Device Treatment of Lymphedema. Biomed Opt.
Express. 2010 Jul15; 1(1): 114-125.
Compression Garments:
Maintenance
Lymphatico-venular Bypass
Lymphaticovenous
Anastamosis
 Microsurgical
bypass procedure that
anastomose lymphatic channels to
surface venules (0.5-0.8mm)
 Requires a competent venous system
and intact regional lymphatic channels
 Efficacy is proportional to the number of
LVA performed
Vascularized Lymph Node
Transfer
Lymph Node Transfer
with Flap
Surgical Treatment
Stage I :
Lymphaticovenous anastomosis (LVA)
Stage II :
LVA + Vascularized lymph node transfer
(VLNT)
Late Stage II/III :
VLNT, radical reduction with perforators
preservation (RRPP), +/ liposuction
Stage III:
Liposuction, Charles procedure (debulking)
Generally reserved for those who have failed conservative options
Candidates for Surgery
 Failed
to control disease despite optimal
conservative therapy
 Limited fibrosis
 No active infection
 Patient insistence
 Normal lymphatic and vascular anatomy
and function (LVA & VLNT)
Limitations of Lymphedema
Surgery
 Not
curative
 Does not address the underlying
pathophysiology of lymphedema
 Long term effects on lymphatic
system still unclear
Lymphedema: Do’s and Don’ts
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Inappropriately use diuretics
Assume the patient has swelling of unknown origin or just
“fat legs”
Prescribe pneumatic compression devices and not follow up
Prescribe standard venous stasis compression stockings
(e.g. Jobst) and not follow up
Neglect underlying chronic venous insufficiency or
obstruction sign/symptoms
Neglect underlying arterial insufficiency (ulcers/ischemia)
Neglect looking for specific signs of Lymphedema (pitting
edema, Stemmer sign, sausage toes, etc.)
Neglect to take a comprehensive history looking for
previous surgery/trauma/XRT, recurrent infections, familial
patterns, pattern of swelling.
Thank You !