Lymphedema

Lymphedema (lymphoedema in British English), also

known as lymphatic obstruction, is a condition of lo-
calized uid retention and tissue swelling caused by a
compromised lymphatic system, which normally returns
interstitial uid to the thoracic duct and then the blood-
stream. The condition can be inherited, though it is fre-
quently caused by cancer treatments, and by parasitic in-
fections. Though incurable and progressive, a number of
treatments can ameliorate symptoms. Tissues with lym-
phedema are at risk of infection.
1 Signs and symptoms
Symptoms may include a feeling of heaviness or fullness,
edema, and aching pain in the aected area. In advanced
lymphedema, there may be the presence of skin changes
such as discoloration, verrucous (wart-like) hyperplasia,
hyperkeratosis, and papillomatosis; and eventually defor-
mity (elephantiasis).
Lymphedema should not be confused with edema aris-
ing from venous insuciency, which is not lymphedema.
However, untreated venous insuciency can progress
into a combined venous/lymphatic disorder which is
treated the same way as lymphedema.
Presented here is an extreme case of severe unilateral
hereditary lymphedema which had been present for 25
years without treatment:
Comparison of normal and swollen limb
Size of swollen foot, toes underneath
Another view of lymphedemic foot
Foot and leg (held vertically)
2 Causes
Lymphedema aects approximately 140 million people
worldwide.
[1]
Lymphedema may be inherited (primary) or caused by
injury to the lymphatic vessels (secondary). It is most fre-
quently seen after lymph node dissection, surgery and/or
radiation therapy, in which damage to the lymphatic sys-
tem is caused during the treatment of cancer, most no-
tably breast cancer. In many patients with cancer, this
condition does not develop until months or even years af-
ter therapy has concluded. Lymphedema may also be as-
sociated with accidents or certain diseases or problems
that may inhibit the lymphatic system from functioning
properly. In tropical areas of the world, a common cause
of secondary lymphedema is lariasis, a parasitic infec-
tion. It can also be caused by a compromising of the lym-
phatic system resulting from cellulitis.
While the exact cause of primary lymphedema is still un-
known, it generally occurs due to poorly developed or
missing lymph nodes and/or channels in the body. Lym-
phedema may be present at birth, develop at the onset
of puberty (praecox), or not become apparent for many
years into adulthood (tarda). In men, lower-limb primary
lymphedema is most common, occurring in one or both
legs. Some cases of lymphedema may be associated with
other vascular abnormalities.
Secondary lymphedema aects both men and women. In
women, it is most prevalent in the upper limbs after breast
cancer surgery and lymph node dissection, occurring in
the armon the side of the body in which the surgery is per-
formed. In Western countries, secondary lymphedema
is most commonly due to cancer treatment.
[1]
Between
38 and 89% of breast cancer patients suer from lym-
phedema due to axillary lymph node dissection and/or
radiation.
[1][2][3]
Unilateral lymphedema occurs in up to
41% of patients after gynecologic cancer.
[1][4]
For men,
a 5-66% incidence of lymphedema has been reported
in patients treated with incidence depending on whether
staging or radical removal of lymph glands was done in
addition to radiotherapy.
[1][5][6]
Head and neck lymphedema can be caused by surgery or
radiation therapy for tongue or throat cancer. It may also
occur in the lower limbs or groin after surgery for colon,
ovarian or uterine cancer, in which removal of lymph
nodes or radiation therapy is required. Surgery or treat-
ment for prostate, colon and testicular cancers may re-
sult in secondary lymphedema, particularly when lymph
nodes have been removed or damaged.
The onset of secondary lymphedema in patients who have
had cancer surgery has also been linked to aircraft ight
(likely due to decreased cabin pressure). For cancer sur-
vivors, therefore, wearing a prescribed and properly tted
compression garment may help decrease swelling during
air travel.
Some cases of lower-limb lymphedema have been asso-
ciated with the use of tamoxifen, due to the blood clots
and deep vein thrombosis (DVT) that can be caused by
1
2 4 DIAGNOSIS
this medication. Resolution of the blood clots or DVT is
needed before lymphedema treatment can be initiated.
Lymphedema is common in newborns with Turner syn-
drome.
3 Pathophysiology
Lymph is formed from the uid that lters out of
the blood circulation to nourish cells. This uid re-
turns through venous capillaries to the blood circulation
through the force of osmosis in the venous blood; how-
ever, a portion of the uid which contains proteins, cel-
lular debris, bacteria, etc. must return through the lym-
phatic collection system to maintain tissue uid balance.
The collection of this prelymph uid is carried out by the
initial lymph collectors which are blind-ended epithelial-
lined vessels with fenestrated openings that allow uids
and particles as large as cells to enter. Once inside the
lumen of the lymphatic vessels, the uid is guided along
increasingly larger vessels, rst with rudimentary valves
to prevent backow, which later develop into complete
valves similar to the venous valve. Once the lymph en-
ters the fully valved lymphatic vessels, it is pumped by
a rhythmic peristaltic-like action by smooth muscle cells
within the lymphatic vessel walls. This peristaltic action
is the primary driving force, moving lymph within its ves-
sel walls. The regulation of the frequency and power of
contraction is regulated by the sympathetic nervous sys-
tem. The movement of lymph can also be inuenced
by the pressure of nearby muscle contraction, arterial
pulse pressure, and the vacuum created in the chest cav-
ity during respiration, but these passive forces contribute
only a minor percentage of lymph transport. The uids
collected are pumped into continually larger vessels and
through lymph nodes, which clean out debris and police
the uid for potential threats from dangerous microbes.
The lymph ends its journey in the thoracic duct or right
lymphatic duct, which drain into the blood circulation.
4 Diagnosis
Assessment of the lower extremities begins with a visual
inspection of the legs. Color, presence of hair, visible
veins, size of the legs and any sores or ulcerations should
be noted. Lack of hair may indicate an arterial circula-
tion problem.
[7]
If swelling is observed, the calf circum-
ference should be measured with a tape measure. This
measurement can be compared to future measurements to
see if the swelling is getting better. Determine if elevat-
ing the legs makes the swelling go away. Pressure should
be applied with the ngertips over the ankle to determine
the degree of swelling. The assessment should also in-
clude a check of the popliteal, femoral, posterior tibial,
and dorsalis pedis pulses. When checking the femoral
pulse, feel for the inguinal nodes and determine if they
are enlarged. Enlargement of the nodes lasting more than
three weeks may indicate infection or some other disease
process requiring further medical attention.
[7]
The diagnosis or early detection of lymphedema is di-
cult. The rst signs may be subjective observations such
as my arm feels heavy or I have diculty these days
getting rings on and o my ngers. These may be symp-
tomatic of early stage of lymphedema where accumula-
tion of lymph is mild and not detectable by any dierence
in arm volume or circumference. As lymphedema devel-
ops further, denitive diagnosis is commonly based upon
an objective measurement of dierences between the af-
fected or at-risk limb at the opposite unaected limb,
e.g. in volume or circumference. Unfortunately, there is
no generally accepted worldwide criterion of dierence
denitively diagnostic, although a volume dierence of
200 ml between limbs or a 4-cm dierence (at a single
measurement site or set intervals along the limb) is often
used. Recently, the technique of bioimpedance measure-
ment (which measures the amount of uid in a limb) has
been shown to have greater sensitivity than these exist-
ing methods, and holds promise as a simple diagnostic
and screening tool.
[8]
Impedance analysers specically
designed for this purpose are nowcommercially available.
Chronic venous stasis changes can mimic early lym-
phedema, but the changes in venous stasis are more of-
ten bilateral and symmetric. Lipedema can also mimic
lymphedema, however in lipedema there is characteris-
tic sparing of the feet beginning abrubtly at the medial
malleoli (ankle level). Lipedema is common in over-
weight women. As a part of the initial work-up be-
fore diagnosing lymphedema, it may be necessary to ex-
clude other potential causes of lower extremity swelling
such as renal failure, hypoalbuminemia, congestive heart-
failure, protein-losing nephropathy, pulmonary hyperten-
sion, obesity, pregnancy, and drug-induced edema.
[9]
4.1 Stages
Whether primary or secondary, lymphedema develops in
stages, from mild to severe. Methods of staging are nu-
merous and inconsistent across the globe. Systems of
staging lymphedema range from three to as many as eight
stages.
Staging system of lymphedema to improve diagnosis
and outcome
A staging system was described in 2007 by Lee, Mor-
gan and Bergan
[10]
and endorsed by the American Society
of Lymphology. This system provides a clear technique
which can be employed by clinical and laboratory assess-
ments to more accurately diagnose and prescribe therapy
for lymphedema, as well as obtain measurable outcomes.
In this improved version, four stages are identied (I-IV).
Clear descriptors of symptoms and clinical presentation
3
must be established at the assessment by the physician
to prescribe interventions, monitor ecacy and support
medical necessity. Physicians and researchers can use
additional laboratory assessments, such as bioimpedance,
MRI, or CT, to build on the ndings of a clinical assess-
ment (physical evaluation). From this, results of therapy
can accurately be determined and reported in documen-
tation, as well as in research.
Risk and latency is a dierent measurement. As of 2012,
research using bioimpedance to measure risk of lym-
phedema appeared promising.
The most common method of staging was dened by the
Fifth WHO Expert Committee on Filariasis:
[11][12]
Stage 0 (latent): The lymphatic vessels have sus-
tained some damage which is not yet apparent.
Transport capacity is still sucient for the amount
of lymph being removed. Lymphedema is not
present.
Stage 1 (spontaneously reversible): Tissue is still
at the pitting stage: when pressed by the ngertips,
the aected area indents, and reverses with eleva-
tion. Usually upon waking in the morning, the limb
or aected area is normal or almost normal in size.
Stage 2 (spontaneously irreversible): The tissue
nowhas a spongy consistency and is considered non-
pitting: when pressed by the ngertips, the aected
area bounces back without indentation. Fibrosis
found in stage 2 lymphedema marks the beginning
of the hardening of the limbs and increasing size.
Stage 3 (lymphostatic elephantiasis): At this
stage, the swelling is irreversible and usually the
limb(s) or aected area is very large. The tissue
is hard (brotic) and unresponsive; some patients
consider undergoing reconstructive surgery, called
debulking. This remains controversial, however,
since the risks may outweigh the benets, and the
further damage done to the lymphatic system may
in fact make the lymphedema worse.
4.2 Grades
Lymphedema can also be categorized by its severity (usu-
ally referenced to a healthy extremity):
Grade 1 (mild edema): Lymphedema involves the
distal parts such as a forearm and hand or a lower
leg and foot. The dierence in circumference is
less than 4 cm, and other tissue changes are not yet
present.
Grade 2 (moderate edema): Lymphedema involves
an entire limb or corresponding quadrant of the
trunk. Dierence in circumference is more than
4 but less than 6 cm. Tissue changes, such as pit-
ting, are apparent. The patient may experience
erysipelas.
Grade 3a (severe edema): Lymphedema is present
in one limb and its associated trunk quadrant.
The dierence in circumference is greater than 6
centimeters. Signicant skin alterations, such as
cornication or keratosis, cysts and/or stulae, are
present. Additionally, the patient may experience
repeated attacks of erysipelas.
Grade 3b (massive edema): The same symptoms
as grade 3a, except two or more extremities are af-
fected.
Grade 4 (gigantic edema): Also known as elephan-
tiasis, in this stage of lymphedema, the aected ex-
tremities are huge due to almost complete blockage
of the lymph channels. Elephantiasis may also aect
the head and face.
5 Treatment
Treatment for lymphedema varies depending on the
severity of the edema and the degree of brosis of the
aected limb. Most people with lymphedema follow a
daily regimen of treatment as suggested by their physician
or certied lymphedema therapist. The most common
treatments for lymphedema are a combination of manual
compression lymphatic massage, compression garments
or bandaging. Complex decongestive physiotherapy is
an empiric system of lymphatic massage, skin care, and
compressive garments. Although a combination treat-
ment program may be ideal, any of the treatments can
be done individually.
5.1 Complete decongestive therapy
CDT is a primary tool in lymphedema management con-
sisting of manual manipulation of the lymphatic ducts,
[13]
short-stretch compression bandaging, therapeutic exer-
cise, and skin care. The technique was pioneered by Emil
Vodder in the 1930s for the treatment of chronic sinusi-
tis and other immune disorders. Initially, CDT involves
frequent visits to a certied therapist with a doctors pre-
scription. Once the lymphedema is reduced, increased
patient participation is required for ongoing care, along
with the use of elastic compression garments and nonelas-
tic directional ow foam garments.
Manual manipulation of the lymphatic ducts consists of
gentle, rhythmic massaging of the skin to stimulate the
ow of lymph and its return to the blood circulation sys-
tem. In the bloods passage through the kidneys, the ex-
cess uid is ltered out and eliminated from the body
through urination. The treatment is very gentle and a typ-
ical session will involve drainage of the neck, trunk, and
4 5 TREATMENT
involved extremity (in that order), lasting approximately
40 to 60 minutes. CDT is generally eective on non-
brotic lymphedema and less eective on more brotic
legs, although it has been shown to help break up brotic
tissue.
5.2 Compression
5.2.1 Garments
Elastic compression garments are worn by persons with
lymphedema on the aected limb following complete de-
congestive therapy to maintain edema reduction. Inelas-
tic garments have also been shown to provide contain-
ment and reduction.
5.2.2 Bandaging
Compression bandaging, also called wrapping, is the ap-
plication of several layers of padding and short-stretch
bandages to the involved areas. Short-stretch bandages
are preferred over long-stretch bandages (such as those
normally used to treat sprains), as the long-stretch ban-
dages cannot produce the proper therapeutic tension nec-
essary to safely reduce lymphedema and may in fact
end up producing a tourniquet eect. During activity,
whether exercise or daily activities, the short-stretch ban-
dages enhance the pumping action of the lymph vessels by
providing increased resistance for them to push against.
This encourages lymphatic ow and helps to soften uid-
swollen areas.
A Stanford University medical study showed patients
receiving the combined modalities of manual lymph
drainage (MLD) with complete decongestive therapy
(CDT) and pneumatic pumping had a greater overall
reduction in limb volume than patients receiving only
MLD/CDT.
[14]
5.2.3 Intermittent pneumatic compression therapy
Intermittent pneumatic compression therapy (IPC) uti-
lizes a multi-chambered pneumatic sleeve with over-
lapping cells to promote movement of lymph uid.
Pump therapy should be used in addition to other treat-
ments such as compression bandaging and manual lymph
drainage. In some cases, pump therapy may help soften
brotic tissue and therefore potentially enable more ef-
cient lymphatic drainage. However, there have been
reports linking pump therapy to an increased incidence
of edema proximal to the aected limb, such as genital
edema arising after the use pump therapy in the lower
limb.
[15]
IPC should be used in combination with com-
plete decongestive therapy.
[14]
5.3 Exercise
Most studies investigating the eects exercise in pa-
tients with lymphedema or those at risk of develop-
ing lymphedema have examined patients with breast-
cancer related lymphedema. In these studies, resis-
tance training did not increase swelling in patients with
pre-existing breast cancer-related lymphedema, and may
actually decrease edema in some patients, in addition
to other potential benecial eects on cardiovascular
health.
[16][17][18][19]
Moreover, resistance training and
other forms of exercise were not associated with an
increased risk of developing lymphedema in patients
who previously received breast cancer-related treatment.
However, exercise should be only be performed while
wearing compression garments (with the possible excep-
tion of swimming in some patients).
[20]
Patients who have
lymphedema or are at risk of developing lymphedema
should consult their physician or certied lymphedema
therapist before beginning a new exercise regimen. Re-
sistance training is not recommended in the immediate
post-operative period in patients who have undergone ax-
illary lymph node dissection for breast cancer.
There is a paucity of studies examining the eects of
exercise in primary lymphedema or in secondary lym-
phedema that is not related to breast cancer treatment.
5.4 Surgery
Several eective surgical procedures exist to provide
long-term solutions for patients who suer from lym-
phedema. Prior to any lymphedema surgery, patients
typically have been treated by a physical therapist, or an
occupational therpist, trained in providing lymphedema
treatment for initial conservative treatment of their lym-
phedema. CDT, MLD and compression bandaging
are all helpful components of conservative lymphedema
treatment.
[21]
5.4.1 Vascularized lymph node transfer
Vascularized lymph node transfers (VLNT) can be an ef-
fective method for the treatment of lymphedema of the
arm and upper extremity. Lymph nodes are harvested
from the groin area with their supporting artery and vein
and moved to the axilla (armpit). Microsurgeons use spe-
cialized microsurgical techniques to reconnect the artery
and vein to new blood vessels in the axilla to provide vital
support to the lymph nodes while they develop their own
blood supply over the rst few weeks after surgery.
The newly transferred lymph nodes then serve as a con-
duit or lter to remove the excess lymphatic uid from
the arm and return it to the bodys natural circulation.
This technique of lymph node transfer usually is per-
formed together with a DIEP ap breast reconstruction.
This allows for both the simultaneous treatment of the
5.4 Surgery 5
arm lymphedema and the creation of a breast in one
surgery. The lymph node transfer removes the excess
lymphatic uid to return form and function to the arm.
In selected cases, the lymph nodes may be transferred as
a group with their supporting artery and vein, but without
the associated abdominal tissue for breast reconstruction.
Lymph node transfers are most eective in patients
whose extremity circumference reduces signicantly
with compression wrapping, indicating most of the
edema is uid.
VLNT has been shown to signicantly improve the uid
component of lymphedema and decrease the amount
of lymphedema therapy and compression garment use
required.
[22]
5.4.2 Lymphaticovenous anastomosis
Lymphaticovenous anastomosis (LVA) can be an eec-
tive and long-term solution for extremity lymphedema,
and many patients have results which range from a mod-
erate improvement to an almost complete resolution of
the problem. LVAs are most eective early in the course
of the disease in patients whose extremity circumference
reduces signicantly with compression wrapping, indi-
cating most of the edema is uid. Patients who do not
respond to compression are less likely to fare well with
LVA, as a greater amount of their increased extremity
volume consists of brotic tissue, protein or fat. LVAs
have been shown to be eective in multiple studies in the
medical literature.
[23][24][25]
Lymphaticovenous anastomosis was rst introduced by
Dr. B.M. O'Brien and his colleagues for the treatment of
obstructive lymphedema in the extremities.
[26]
In 2003,
Dr. Isao Koshima, a pioneer in the eld of supermicro-
surgery, with his colleagues, vastly improved the surgery
with supermicrosurgical techniques and established the
new standard in reconstructive microsurgery.
[26]
Stud-
ies involving long-term follow-up after LVA for lym-
phedema indicated patients showed remarkable improve-
ment compared to conservative treatment using continu-
ous elastic stocking and occasional pumping.
[26]
Clinical studies involving LVA indicate immediate and
long-term results showed signicant reductions in vol-
ume and improvement in systems that appear to be long-
lasting.
[27][28][29]
In addition, a 2006 study comparing
two groups of breast cancer patients at high risk for
lymphedema in whom LA was used to prevent the on-
set of clinically evident lymphedema. Results showed
a statistically signicant improvement in the reduction
of patients who went on to develop clinically signi-
cant lymphedema.
[29]
Other medical studies also show
LVA surgeries are eective to reduce the severity of lym-
phedema in breast cancer patients.
[30][31]
In particular, a
clinical study of 1,000 cases of lymphedema treated with
microsurgery from1973 to 2006 showed highly benecial
results.
[31]
Clinical reports from microsurgeons and phys-
ical therapists have documented more than 1,500 patients
treated with LA surgery over a span of 30 years showing
signicant improvement and eectiveness.
[25]
Indocyanine green uoroscopy has been established as a
safe, minimally invasive and useful tool for the surgical
evaluation of lymphedema.
[32]
Microsurgeons use indo-
cyanine green lymphography to assist in performing suc-
cessful LA surgeries.
[33]
Lymphaticovenous anastomosis uses supermicrosurgery
to connect the aected lymphatic channels directly to tiny
veins located nearby. The lymphatics are tiny, typically
0.1 mm to 0.8 mm in diameter. The procedure requires
the use of specialized techniques with superne surgical
suture and an adapted, high-power microscope.
5.4.3 Suction Assisted Lipectomy for advanced
lymphedema
Patients who have limbs which no longer adequately re-
spond to standard lymphatic compression therapy may
be candidates for suction assisted lipectomy (SAL). This
procedure has also been called liposuction for lym-
phedema, and is a technique specically adapted to treat
this advanced condition. SAPL is very dierent in both
the operative technique and the signicant amount of
lymphedema therapy and compression garment tting
and care which must be administered by a lymphedema
therapist experienced in the technique.
This procedure was pioneered by Dr. Hakan Brorson,
Department of Plastic and Reconstructive Surgery,
Skne University Hospital in Malm, Sweden in
1987.
[1]
Well-controlled clinical trials conducted
from 1993 to 2014 show lymphatic liposuction, com-
bined with controlled compression therapy (CCT),
to be an eective lymphedema treatment without
recurrence.
[34][35][1][36][37][38][39][40]
Long-term followup
(11 13 years) of patients with lymphedema showed no
recurrence of swelling.
[1]
Lymphatic liposuction when
combined with controlled compression therapy was more
eective than controlled compression therapy alone to
reduce lymphedema.
[41][42]
SAL has been rened in recent years by using vibrating
cannulae which are ner and more eective than previous
equipment.
[1]
In addition, the introduction of the tourni-
quet and tumescent technique has led to minimized blood
loss.
[1][43]
SAL uses specialized techniques that dier from conven-
tional liposuction procedures which requires specialized
training. This surgical procedure is an eective method
of reducing the size and stiness of the aected extrem-
ity. However, SAL is generally followed by use of com-
pression garments to prevent a recurrence of the lym-
phedema.
6 7 SEE ALSO
5.4.4 Lymphatic vessel grafting
With the possibilities of advanced microsurgical tech-
niques, lymph vessels can be sutured and used as grafts.
A locally interrupted or obstructed lymphatic pathway,
mostly after resection of lymph nodes, can be recon-
structed by a bypass using lymphatic vessels. These ves-
sels are specialized to drain lymph by active pumping
forces. These grafts are connected with main lymphatic
collectors in front and behind the obstruction. The tech-
nique is mostly used in arm edemas after treatment of
mammary carcinomas and in unilateral edemas of lower
extremities after resection of lymphnodes and radiation.
The procedure is less widely used than the other sur-
gical procedures, mainly in centers in Germany. The
method was developed experimentally at the Institute of
Experimental Surgery, the Ludwig Maximilians Univer-
sity (LMU) in Munich. It was introduced as treatment in
1980 by Prof. Ruediger Baumeister.
[44]
The method is proved to be eective.
[45]
Follow-up stud-
ies showed signicant reduction of volume of the extrem-
ities even 10 years after surgery.
[46]
The patients, who had been previously treated with both
MLD and compression therapy, gained signicant im-
provements in quality of life after being treated with
lymphatic vessel grafting.
[47]
Lymphoscintigraphic in-
vestigations at the Clinic of Nuclear Medicine at LMU
showed a lasting enhancement of lymphatic transport af-
ter grafting.
[48]
The patency of lymphatic grafts have been demonstrated
by the Institute for Clinical Radiology after more than 12
years, using indirect lymphography and MRI lymphogra-
phy.
5.5 Low level laser therapy
Low-level laser therapy (LLLT) was cleared by the US
Food and Drug Administration (FDA) for the treatment
of lymphedema in November 2006.
[49]
According to the US National Cancer Institute,
Studies suggest that low-level laser therapy
may be eective in reducing lymphedema in
a clinically meaningful way for some women.
Two cycles of laser treatment were found to
be eective in reducing the volume of the af-
fected arm, extracellular uid, and tissue hard-
ness in approximately one-third of patients
with postmastectomy lymphedema at 3 months
posttreatment. Suggested rationales for laser
therapy include a potential decrease in brosis,
stimulation of macrophages and the immune
system, and a possible role in encouraging
lymphangiogenesis.
[50][51]
5.6 Early prevention and disease regres-
sion in breast cancer
In 2008, an NIH study revealed early diagnosis of lym-
phedema in breast cancer patients (stage 0 in the arti-
cle) associated with an early intervention, a compression
sleeve and gauntlet for one month, led to a return to pre-
operative baseline status. In a ve-year followup, patients
remained at their preoperative baseline, suggesting pre-
clinical detection of lymphedema can halt if not reverse
its progression.
6 Complications
When the lymphatic impairment becomes so great that
the lymph uid exceeds the lymphatic systems ability to
transport it, an abnormal amount of protein-rich uid col-
lects in the tissues of the aected area. Left untreated,
this stagnant, protein-rich uid causes tissue channels to
increase in size and number, reducing the availability of
oxygen. This interferes with wound healing and provides
a rich culture medium for bacterial growth that can result
in infections: cellulitis, lymphangitis, lymphadenitis, and
in severe cases, skin ulcers. It is vital for lymphedema
patients to be aware of the symptoms of infection and to
seek treatment at the rst signs, since recurrent infections
or cellulitis, in addition to their inherent danger, further
damage the lymphatic system and set up a vicious circle.
In rare cases, lymphedema can lead to a form of can-
cer called lymphangiosarcoma, although the mechanism
of carcinogenesis is not understood. Lymphedema-
associated lymphangiosarcoma is called Stewart-Treves
syndrome. Lymphangiosarcoma most frequently oc-
curs in cases of long-standing lymphedema. The inci-
dence of angiosarcoma is estimated to be 0.45% in pa-
tients living 5 years after radical mastectomy.
[52][53]
Lym-
phedema is also associated with a low grade form of can-
cer called retiform hemangioendothelioma (a low grade
angiosarcoma).
[54]
Since lymphedema is disguring, causes diculties in
daily living and can lead to lifestyle becoming severely
limited, it may also result in psychological distress.
7 See also
Milroys disease
Congenital lymphedema
Lymphotherapy
Waldemar Olszewski
7
8 References
[1] Brorson H, Ohlin K, Olsson G, Svensson B, Svensson H
(June 2008). Controlled compression and liposuction
treatment for lower extremity lymphedema. Lymphology
41 (2): 5263. PMID 18720912.
[2] Kissin MW, Querci della Rovere G, Easton D, West-
bury G (July 1986). Risk of lymphoedema following
the treatment of breast cancer. Br J Surg 73 (7): 580
4. doi:10.1002/bjs.1800730723. PMID 3730795.
[3] Segerstrm K, Bjerle P, Graman S, Nystrm A
(1992). Factors that inuence the incidence of brachial
oedema after treatment of breast cancer. Scand
J Plast Reconstr Surg Hand Surg 26 (2): 2237.
doi:10.3109/02844319209016016. PMID 1411352.
[4] Werngren-Elgstrm M, Lidman D (December 1994).
Lymphoedema of the lower extremities after surgery
and radiotherapy for cancer of the cervix. Scand
J Plast Reconstr Surg Hand Surg 28 (4): 28993.
doi:10.3109/02844319409022014. PMID 7899840.
[5] Pilepich MV, Asbell SO, Mulholland GS, Pajak T (1984).
Surgical staging in carcinoma of the prostate: the
RTOG experience. Radiation Therapy Oncology Group.
Prostate 5 (5): 4716. doi:10.1002/pros.2990050502.
PMID 6483687.
[6] Pilepich MV, Krall J, George FW, Asbell SO, Plenk
HD, Johnson RJ, Stetz J, Zinninger M, Walz BJ (1994).
Treatment-related morbidity in Phase III RTOG stud-
ies of extended-eld irradiation for carcinoma of the
prostate. Int. J. Radiat. Oncol. Biol. Phys. 10 (10):
18617. doi:10.1016/0360-3016(84)90263-3. PMID
6386761.
[7] Jarvis, C. (2004). Physical Examination and Health As-
sessment (5th ed.). Saunders Elsevier. pp. 530553.
ISBN 1-4160-5188-0.
[8] Ward LC (2006). Bioelectrical Impedance Analysis:
Proven Utility in Lymphedema Risk Assessment and
Therapeutic Monitoring. Lymphatic Research and Bi-
ology 4 (1): 516. doi:10.1089/lrb.2006.4.51. PMID
16569209.
[9] Burkhart CN, Adigun C, Burton CS. Chapter 174. Cu-
taneous Changes in Peripheral Venous and Lymphatic
Insuciency. In: Wol K, ed. Fitzpatricks Der-
matology in General Medicine. 8th ed. New York:
McGraw-Hill; 2012. http://www.accessmedicine.com/
content.aspx?aID=56081150. Accessed November 3,
2013.
[10] Lawrence L Tretbar; Cheryl L. Morgan; Byung-Boong
Lee; Benoit Blondeau; Simon J. Simonian (2007). Lym-
phedema: Diagnosis and Treatment. Springer. ISBN 1-
84628-548-8.
[11] Lymphatic lariasis: The disease and its control. Fifth
report of the WHO Expert Committee on Filariasis.
World Health Organization technical report series 821: 1
71. 1992. PMID 1441569.
[12] Untitled-1 (PDF). Retrieved 2014-05-16.
[13] http://www.lymphnet.org/pdfDocs/nlntreatment.pdf
[14] Szuba A, Achalu R, Rockson SG (2002). Deconges-
tive lymphatic therapy for patients with breast carcinoma-
associated lymphedema. Cancer 95 (11): 22607.
doi:10.1002/cncr.10976. PMID 12436430.
[15] Boris M, Weindorf S, Lasinski BB (Mar 1998). The
risk of genital edema after external pump compression
for lower limb lymphedema. Lymphology 31 (1): 1520.
PMID 9561507.
[16] Markes M, Brockow T, Resch KL (2006). Exer-
cise for women receiving adjuvant therapy for breast
cancer. Cochrane Database of Systematic Re-
views. The Cochrane database of systematic reviews (4):
CD005001. doi:10.1002/14651858.CD005001.pub2.
PMID 17054230.
[17] McKenzie DC, Kalda AL (2003). Eect of upper ex-
tremity exercise on secondary lymphedema in breast can-
cer patients: A pilot study. Journal of clinical oncology :
ocial journal of the American Society of Clinical Oncol-
ogy 21 (3): 4636. doi:10.1200/jco.2003.04.069. PMID
12560436.
[18] Ahmed RL, Thomas W, Yee D, Schmitz KH
(2006). Randomized controlled trial of weight
training and lymphedema in breast cancer survivors.
Journal of Clinical Oncology 24 (18): 276572.
doi:10.1200/JCO.2005.03.6749. PMID 16702582.
[19] Schmitz KH, Ahmed RL, Troxel A, Cheville A, Smith
R, Lewis-Grant L, Bryan CJ, Williams-Smith CT, Greene
QP (2009). Weight lifting in women with breast-cancer-
related lymphedema. New England Journal of Medicine
361 (7): 66473. doi:10.1056/NEJMoa0810118. PMID
19675330.
[20] Position Paper: Exercise | National Lymphedema Net-
work. Lymphnet.org. Retrieved 2014-05-16.
[21] Granzow JW, Soderberg JM, Kaji AH, Dauphine C. Re-
view of Current Surgical Treatments for Lymphedema.
Ann Surg Oncol. Ann Surg Oncol. 2014 Apr;21(4):1195-
1201. http://download.springer.com/static/pdf/440/art%
253A10.1245%252Fs10434-014-3518-8.pdf?auth66=
1407772232_810887e3a2e3b88b0b9c0b7aa1446fb5&
ext=.pdf
[22] Granzow JW, Soderberg JM, Kaji AH, Dauphine C.
An Eective System of Surgical Treatment of Lym-
phedema. Ann Surg Oncol. 2014 Apr;21(4):1189-94.
http://download.springer.com/static/pdf/258/art%
253A10.1245%252Fs10434-014-3515-y.pdf?auth66=
1407719571_c20be3cf59888a68ef7c6b48604982b1&
ext=.pdf
[23] Granzow JW, Soderberg JM, Dauphine C. A Novel Two-
Stage Surgical Approach to Treat Chronic Lymphedema.
Breast J. 2014 Jun 19.
[24] Granzow JW, Soderberg JM, Kaji AH, Dauphine C.
An Eective System of Surgical Treatment of Lym-
phedema. Ann Surg Oncol. 2014 Apr;21(4):1189-94.
http://download.springer.com/static/pdf/258/art%
253A10.1245%252Fs10434-014-3515-y.pdf?auth66=
8 8 REFERENCES
1407719571_c20be3cf59888a68ef7c6b48604982b1&
ext=.pdf
[25] Campisi C, Eretta C, Pertile D, Da Rin E, Campisi
C, Macci A, Campisi M, Accogli S, Bellini C, Bo-
nioli E, Boccardo F (2007). Microsurgery for treat-
ment of peripheral lymphedema: long-term outcome
and future perspectives. Microsurgery 27 (4): 3338.
doi:10.1002/micr.20346. PMID 17477420.
[26] Koshima I, Nanba Y, Tsutsui T, Takahashi Y, Itoh S (May
2003). Long-term follow-up after lymphaticovenular
anastomosis for lymphedema in the leg. J Reconstr
Microsurg 19 (4): 20915. doi:10.1055/s-2003-40575.
PMID 12858242.
[27] Granzow JW, Soderberg JM, Dauphine C. A Novel Two-
Stage Surgical Approach to Treat Chronic Lymphedema.
Breast J. 2014 Jun 19.
[28] Granzow JW, Soderberg JM, Kaji AH, Dauphine C.
An Eective System of Surgical Treatment of Lym-
phedema. Ann Surg Oncol. 2014 Apr;21(4):1189-94.
http://download.springer.com/static/pdf/258/art%
253A10.1245%252Fs10434-014-3515-y.pdf?auth66=
1407719571_c20be3cf59888a68ef7c6b48604982b1&
ext=.pdf
[29] Campisi C, Davini D, Bellini C, Taddei G, Villa G,
Fulcheri E, Zilli A, da Rin E, Eretta C, Boccardo F (2006).
Is there a role for microsurgery in the prevention of arm
lymphedema secondary to breast cancer treatment?". Mi-
crosurgery 26 (1): 702. doi:10.1002/micr.20215. PMID
16444710.
[30] Chang DW (September 2010). Lymphaticovenular by-
pass for lymphedema management in breast cancer pa-
tients: a prospective study. Plast. Reconstr. Surg.
126 (3): 7528. doi:10.1097/PRS.0b013e3181e5f6a9.
PMID 20811210.
[31] Campisi C, Davini D, Bellini C, Taddei G, Villa G,
Fulcheri E, Zilli A, Da Rin E, Eretta C, Boccardo
F (2006). Lymphatic microsurgery for the treat-
ment of lymphedema. Microsurgery 26 (1): 659.
doi:10.1002/micr.20214. PMID 16444753.
[32] Yamamoto T, Narushima M, Doi K, Oshima A, Ogata
F, Mihara M, Koshima I, Mundinger GS (May 2011).
Characteristic indocyanine green lymphography ndings
in lower extremity lymphedema: the generation of a novel
lymphedema severity staging system using dermal back-
ow patterns. Plast. Reconstr. Surg. 127 (5): 197986.
doi:10.1097/PRS.0b013e31820cf5df. PMID 21532424.
[33] Ogata F, Narushima M, Mihara M, Azuma R, Morimoto
Y, Koshima I (August 2007). Intraoperative lymphog-
raphy using indocyanine green dye for near-infrared u-
orescence labeling in lymphedema. Ann Plast Surg 59
(2): 1804. doi:10.1097/01.sap.0000253341.70866.54.
PMID 17667413.
[34] Granzow JW, Soderberg JM, Dauphine C. A Novel Two-
Stage Surgical Approach to Treat Chronic Lymphedema.
Breast J. 2014 Jun 19.
[35] Granzow JW, Soderberg JM, Kaji AH, Dauphine C.
An Eective System of Surgical Treatment of Lym-
phedema. Ann Surg Oncol. 2014 Apr;21(4):1189-94.
http://download.springer.com/static/pdf/258/art%
253A10.1245%252Fs10434-014-3515-y.pdf?auth66=
1407719571_c20be3cf59888a68ef7c6b48604982b1&
ext=.pdf
[36] Brorson, Hakan; Karin Ohlin, OCT, Barbro Svensson,
PT, LT (2008). The Facts About Liposuction As
A Treatment For Lymphoedema. Journal of Lym-
phoedema 3 (1): 3847.
[37] Brorson H, Svensson H (June 1997). Complete reduc-
tion of lymphoedema of the arm by liposuction after
breast cancer. Scand J Plast Reconstr Surg Hand Surg 31
(2): 13743. doi:10.3109/02844319709085480. PMID
9232698.
[38] Brorson H (2000). Liposuction gives complete
reduction of chronic large arm lymphedema af-
ter breast cancer. Acta Oncol 39 (3): 40720.
doi:10.1080/028418600750013195. PMID 10987239.
[39] Brorson H (2003). Liposuction in arm lymphedema
treatment. Scand J Surg 92 (4): 28795. PMID
14758919.
[40] Brorson, H.; K. Ohlin; G. Olsson et al (2006). Long term
cosmetic and functional results following liposuction for
arm lymphedema: An eleven year study. Lymphology.
40 (Supp): 253255.
[41] Brorson H, Svensson H (September 1998). Liposuction
combined with controlled compression therapy reduces
arm lymphedema more eectively than controlled com-
pression therapy alone. Plast. Reconstr. Surg. 102 (4):
105867; discussion 1068. PMID 9734424.
[42] Damstra RJ, Voesten HG, Klinkert P, Brorson H (August
2009). Circumferential suction-assisted lipectomy for
lymphoedema after surgery for breast cancer. Br J Surg
96 (8): 85964. doi:10.1002/bjs.6658. PMID19591161.
[43] Wojnikow S, Malm J, Brorson H (2007). Use of a
tourniquet with and without adrenaline reduces blood loss
during liposuction for lymphoedema of the arm. Scan-
dinavian Journal of Plastic and Reconstructive Surgery
and Hand Surgery / Nordisk Plastikkirurgisk Forening
[and] Nordisk Klubb for Handkirurgi 41 (5): 2439.
doi:10.1080/02844310701546920. PMID 17886128.
[44] Baumeister RG, Seifert J, Wiebecke B, Hahn D
(May 1981). Experimental basis and rst applica-
tion of clinical lymph vessel transplantation of sec-
ondary lymphedema. World J Surg 5 (3): 4017.
doi:10.1007/BF01658013. PMID 7293201.
[45] Baumeister RG, Siuda S (January 1990). Treatment of
lymphedemas by microsurgical lymphatic grafting: what
is proved?". Plast. Reconstr. Surg. 85 (1): 6474; discus-
sion 756. doi:10.1097/00006534-199001000-00012.
PMID 2293739.
[46] Baumeister RG, Frick A(July 2003). "[The microsurgical
lymph vessel transplantation]". Handchir Mikrochir Plast
Chir (in German) 35 (4): 2029. doi:10.1055/s-2003-
42131. PMID 12968216.
9
[47] Springer S, Koller M, Baumeister RG, Frick A (June
2011). Changes in quality of life of patients with lym-
phedema after lymphatic vessel transplantation. Lym-
phology 44 (2): 6571. PMID 21949975.
[48] Weiss M, Baumeister RG, Hahn K (November 2002).
Post-therapeutic lymphedema: scintigraphy before and
after autologous lymph vessel transplantation: 8 years
of long-term follow-up. Clin Nucl Med 27 (11): 788
92. doi:10.1097/01.RLU.0000033613.05410.34. PMID
12394126.
[49] dotmed.com December 27, 2006 Low Level Laser FDA
Cleared for the Treatment of Lymphedema. (accessed 9
November 09)
[50] National Cancer Institute: Low-level laser therapy ac-
cessed 9 November 09
[51] Carati CJ, Anderson SN, Gannon BJ, Piller NB (2003).
Treatment of postmastectomy lymphedema with low-
level laser therapy. Cancer 98 (6): 111422.
doi:10.1002/cncr.11641. PMID 12973834.
[52] Martin MB, Kon ND, Kawamoto EH, Myers RT, Sterchi
JM (1984). Postmastectomy angiosarcoma. The Amer-
ican surgeon 50 (10): 5415. PMID 6541442.
[53] Chopra S, Ors F, Bergin D(2007). MRI of angiosarcoma
associated with chronic lymphoedema: Stewart Treves
syndrome. British Journal of Radiology 80 (960): e310
3. doi:10.1259/bjr/19441948. PMID 18065640.
[54] Requena L, Sangueza OP (1998). Cutaneous vascular
proliferations. Part III. Malignant neoplasms, other cu-
taneous neoplasms with signicant vascular component,
and disorders erroneously considered as vascular neo-
plasms. Journal of the American Academy of Derma-
tology 38 (2): 14375; quiz 1768. doi:10.1016/S0190-
9622(98)70237-3. PMID 9486670.
9 External links
Lymphedema at DMOZ
Lymphedema Resources
Mayo Clinic
10 10 TEXT AND IMAGE SOURCES, CONTRIBUTORS, AND LICENSES
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