Emily Iker, MD

Lymphedema
Management Update

Lymphedema Center Santa Monica, California, USA

LYMPHEDEMA
Definition
Lymphedema is a pathological accumulation of protein-rich fluid, microorganisms and debris which occurs when lymphatic vessels are obstructed or impaired. This condition causes edema (swelling) in the affected tissues. Oxygen flow is reduced in the transport system resulting in a culture medium for bacteria causing various infections.

LYMPHATIC SYSTEM
Lymph fluid (LF)- protein, water, fatty acids, salt, white

blood cells, microorganisms, and foreign debris2 LF Interstitial spaces Lymph vessels venous system Superficial system Deep system Lymphatic system (LS): lymph capillaries, precollectors, lymph collectors, and lymphatic trunks1
2Foldi

E, Fold M, Foldis Textbook of Lymphology 2nd Edition 2006: 179-222; 1 Zuther E. Pathology. Lymphedema Management 2005: 45-99

CLASSIFICATION
PRIMARY LE
Lymphedema Precox Milroys Syndrome VEGFR-3 gene mutation (1/6,000 births) Lymphedema Tarda Meiges Syndrome Developmental abnormalities SOX18 gene mutation hypotrichosis-lymphedema-telangiectasis syndrome KT Syndrome

SECONDARY LE
Surgery, radiation, infections, filariasis, etc. Lymph transport is interrupted due to physical disruption or compression of lymphatic channels

CLASSIFICATION
PRIMARY LE
Plasma membrane of Lymphatic endothelial cells contains VEGFR3 VEGF3 is activated by VEGF-C & VEGF-D ANGIOGENESIS Mutation alters DNA sequence for the gene responsible for creation of VEGFR takes pathological form PRIMARY LE Nonne-Milroy syndrome L Capillary missing L Collectors present Turners Syndrome Dysplasia Yellow Fingernail syndrome Primary LE accompanied by chronic paranasal sinusitis, bronchiectasia, pleural effusion

PRIMARY LYMPHEDEMA

Milroys Syndrome

Meiges Syndrome

SECONDARY LYMPHEDEMA

SECONDARY LYMPEHDEMA

PRESENTATION

LYMPHEDEMA
system in

is

the

result

of

functional overload of the lymphatic which lymph volume exceeds transport capacity

ETIOLOGY
Surgery Radiation Sentinel lymph node biopsy

Infection
Other factors (Weight, post-surgical complications, post-radiation complications,)

PRECAUTIONS
Post-Surgical intervention Exercise (avoid vigorous) Skin care / Avoid heat Prevention of infections Avoid constriction garments Avoid injections/punctures/I.V. Others

SYMPTOMS

Pain (ache, awareness) Heaviness

Pressure
Swelling

DIAGNOSIS

Clinical history

Physical examination

STEMMERS SIGN

DIAGNOSIS 1. Clinical (staging)

2. Lymphoscintigraphy
3. Lymphangiogram

4. M R I / C T / U S

STAGING OF LYMPHEDEMA
STAGE 0 STAGE I

Swelling not evident yet

STAGE II

Early accumulation of LE fluid

No response to elevation, fibrotic tissue Elephantiasis, trophic skin changes

STAGE III

Lymphoscintigraphy

CASE 1:
Keltner 2 pics 2 limph

Normal

LYMPHEDEMA

MRI
Primary lymphedema Lower extremity Total area: 180.2% / Muscle: 59%

2010 Estimated US Cancer Cases*

Prostate Lung and bronchus Colon and rectum Urinary bladder Melanoma of skin Non-Hodgkin lymphoma Kidney Oral Cavity Leukemia Pancreas 4% 3% 3% 3%
4%

28% 15% 9% 7% 5% 4%

Men 710,040

Women 662,870 28% Breast 14% Lung and bronchus

10% 6% 4%

Colon and rectum Uterine corpus

Non-Hodgkin lymphoma
Melanoma of skin Ovary Thyroid Urinary bladder Pancreas

3% 5% 2% 3%

2010, American Cancer Society.

LYMPHEDEMA
TREATMENT

Conservative

Pharmacological
Surgical

LYMPHEDEMA TREATMENT
Conservative
Elevation, skin care CDP (Complex Decongestive physiotherapy) MLD (Manual lymphatic drainage) Multilayer bandaging Pneumatic pumps Compression garments Exercise program

LYMPHEDEMA
TREATMENT Pharmacological

Diuretics
Benzopyrones

Flavinoids

LYMPHEDEMA
TREATMENT
Surgical
Physiological: restore lymphatic flow by reconstruction of the lymphatic channels (lymph-tolymph or lymph-to-vein anastomosis) Reductive: remove excess tissue (Charles procedure) Liposuction
(Brorson)

PRIMARY LYMPHEDEMA

PRIMARY LYMPHEDEMA

LYMPHEDEMA
AXILLARY SENTINEL NODE AND AFFERENT CHANNEL

Node Channel Body outlines

Position of patient
Skin marks over node and channel

TREATMENT

R Ankle Before MLD

After MLD

After MLD+Pump With DM Sleeve

13 mm.

10.5 mm.

9 mm.

R Calf Before MLD

After MLD

After MLD+Pump W/ DM Sleeve

Left calf before MLD

2008 2007

Left calf after MLD

2008 2007 2008

Left calf after MLD & Pump w/DM sleeve

2007

LY

1.65

1.60

1.40

1.55

1.30

1.45

CONCLUSIONS Ultrasound is easily performed and is noninvasive Provides objective measures of severity of disease Objective documentation of response to therapy

UPPER EXTREMITY LYMPHEDEMA TREATMENT

Manual Lymph-Drainage

Compression Treatment