definition - types - history - management of lymphedema lymph physiology lymphatic system

1. By
Ahmed Fawzy EL Sayed Mashaly MSc.
Eygptian fellowship of Plastic surgery In
Plastic & Reconstructive Surgery and Burn Department,
Tanta University

2. An abnormal accumulation
of protein-rich fluid in the
interstitium, secondary to
defective lymph drainage in
the presence of normal net
capillary filtration causing
chronic inflammation and
reactive fibrosis of the
affected tissues.

3. *Usually in an
extremity, but can
also occur in the
head, neck,
genitals, and
abdomen

4. *Affects 1% of the American population (2.5 million people)
*Still poorly understood in the medical community
*Largest cause of lymphedema in the world is Filariasis
(considered secondary lymphedema)
*Filariasis is a parasitic infiltration into the lymphatics that
is very common in third world countries (affects 90 million
people)

8. *
*Primary lymphedema is a result of lymphatic
dysplasia.
*May be present at birth Can develop later in life
without known cause.
*Secondary lymphedema is much more common.
*Result of surgery, radiation, injury, trauma,
scarring, or infection of the lymphatic system

9. *
*Lymphangiodysplasia or Hypoplasia –
fewer than normal # of lymph collectors
*Aplasia – absences of collectors in a
distinct area
1. Milroy's Disease is congenital
lymphedema evident at birth
2. Meige’s Syndrome is primary
lymphedema onset at puberty
(lymphedema praecox)
3. Lymphedema Tardum is primary
lymphedema onset after age 35

10. Facial oedema and epicanthic folds in a 2
year old with compound heterozygote
mutations in PIEZO1

12. *Historical background of
Lymphatic filariasis
* Bancroftian filariasis has been
endemic in Egypt for centuries
with all the clinical manifestations.
* The statue of a Pharaoh, created
4000 years ago, shows clear visible
signs of the disease.
*The mummified body of Natsef-
Amun, a priest at Karnak in the
times of Ramses XI proven after
3000 years by autopsy to have LF
worms in the groin.

14. * "The Secret Medicine of the Pharaohs,“
* Queen Hatasu's temple at Deir el-Bahari
*carved into the limestone wall nearly 3500 years ago.
* voyage of the Egyptian Queen to the neighboring country of Punt
and She is greeted by the Prince of Punt and his wife, the Princess
Ati who has a serious disease condition: lymphedema. "Bags of flesh
hang from her arms and thighs only the joints of her hands and
ankles are visible.

16. *
* Early stage: erysipelas with no line of
*demarcation
* Late stages: dilatation of lymphatic
*vessels followed by their dysfunction,
*accumulation of fluid in tissues and
*increased risk of infection
*(lymphoedema)
* Skin becomes infected---thickening of
*lower limb--- elephantiasis
* Thickening of scrotum ( hydrocele)

19. *
Physical: disfigurment
Social: isolation, loss of
social support, family stress
care giving, shame,sexual
disability.
Psychological: depression,
hopelesness, sucidial
tendencies
Economic: loss of work, loss
of family income, costly
treatment

20. *National program for elimination of
lymphatic Filarisis in Egypt
WHO recommended IDA (combination
of ivermectin, DEC and albendazole) -
an alternative three drug regimen to
accelerate the global elimination of
lymphatic filariasis.

22. *secondary lymphoedema
Cancer treatment is primary cause of
lymphedema
• Estimate of 20% of breast, genitourinary,
gynecological, or melanoma survivors will
experience secondary lymphoedema
• More than 8,000 new cases per year
Review of research evidence on secondary lymphoedema, National Breast and Ovarian
Cancer Centre 2008

24. *LYMPAHATIC SYSTEM
*Lymphatic comes from the Latin word lymphaticus, meaning
"connected to water," as lymph is clear.
*Network of vessels & lymph nodes which are located in all
major tissues of body.
* Lymphatic system is absent in CNS, Cornea, Superficial
layer of skin, Bones, Alveoli of lung.
*CONSIST OF
*Lymph
*Lymphatic Channels
*Lymph Nodes
*Lymph Organs
Capillaries
Vessels
Ducts

25. *LYMPH
Lymph is
*Transudative fluid.
*Transparent & slightly yellowish
liquid.
*Alkaline in nature.
*Derived from tissue fluid.
*When blood passes through
tissues
9/10 of fluid - venous end
1/10 of fluid - lymph capillaries
*“CHYLE” - Lymph from small
intestine.

26. Lymph flow
(Drainge)

27. * RATE OF LYMPH FLOW
• Total estimated lymph flow is 120 ml / hr
• About 100 ml flows through Thoracic duct in resting man per hour
• Approx 20 ml flow into circulation through other channels
• 3 – 4 liters / day

28. Contracting to drive lymph forward.
Backflow is restricted by a pocket valve
visible in the centre of the frame

29. • Lymph carries protein and large particulate matter away from the
tissue space.
• End products of digestion are absorbed mainly by lymph channels.
• Important role in redistribution of fluid in the body.
• Bacteria, toxins and other foreign bodies are removed from the
tissues.
• Maintenance of structural and functional integrity of tissue.
• In immune response of the body.
• Production and maturation of lymphocytes.
FUNCTIONS OF LYMPHATIC SYSTEM

30. *LYMPHATIC CAPILLARIES
*Smallest lymphatic vessels
*They begin in the tissue spaces as blind-ended sacs.
*These capillaries form plexuses which collect lymph from
the interstitial space mark the beginning of lymphatic
system

31. *They are lined by a single layer
of endothelial cells.
*These are attached to C.T by
anchoring filaments.
*The edge of one endothelial cell
overlaps the adjacent cell.
*Overlapping edge is free to flap
inward minute valve.
*Permits passage of high
molecular weight substance.
*LYMPHATIC CAPILLARIES

33. A lymph collecting
vessel is shown using a
mixture of hydrogen
peroxide and blue
dye in the dorsum of a
cadaver foot. Arrow
indicates the
intravascular valves.
(Scale bar 1 mm)

34. «lymphosomes»
we were able to
demarcate the skin into
lymphatic territories for
which we coined the
term «lymphosomes»

35. • In recent years, with the technology to
visualize the maze of vessels and the flow
of lymph fluid through them.
• Investigators in the cancer field have
discovered, that lymph vessels are
necessary for the proliferation of tumors.
• The depth of penetration of lymph
vessels into a tumor has even been
proposed as a predictor of the likelihood
of later metastasis.

36. *lymphatic distribution of
the lower limb

37. Multiple lymph collectors were identifid in the
subcutaneous tissue of the lower limb .
originated beneath the dermis of each side of the toes, the
foot, and the lateral side of the thigh.
The diameters of the vessels varied from 0.2 to 2.2 mm.
The vessels traveled in a tortuous fashion toward the
lymph nodes in the popliteal fossa, those adjacent to the
superficial femoral vessels, in the inguinal region.
sometimes they anastomosed with or crossed over
neighboring vessels.

38. *most vessels converged to form
larger collectors. Some of these larger
collectors then split into small
branches just before entering the
lymph nodes.
*The patterns were different in the
individual and even asymmetrical
between each side of the same body.

39. *Three-dimensional volume-rendering
image of the lower extremity.
*The superficial lymphatic vessels
(green) connect to the inguinal lymph
node.
*The collateral lymphatic vessel purple)
runs through the calf region, connects
to the popliteal lymph node (arrow)
and becomes a deep lymphatic vessel
.
*(Reproduced from Yamazaki et al.

41. Lymphatic vessels
(filled with a barium
sulfate mixture) of the
medial group in the
middle of the left leg
traveling with the great
saphenous vein (GSV)
and its branch. Green
arrows indicate the
direction of lymphatic
flow.

42. Ankle
An average of 12 lymph-collecting
vessels (range 9 to 17) were found in
the subcutaneous tissue around the
ankle. The mean vessel diameter was
1.0 mm (range 0.2 to 2.0 mm).
Anterior Group Most of these vessels
(mean 10; range 8 to 13) were
distributed in the anterior aspect of the
ankle between the lateral and medial
malleoli. They were continuous with
vessels from the dorsum of the foot.
Posterior Group Only a few vessels
(mean 2; range 1 to 4) were distributed
in the posterior aspect of the ankle,
arising from the sides of the Achilles
tendon (tendocalcaneus) bilaterally in
the subcutaneous tissue just above the
heel.
Leg

43. Leg
An average of 13 vessels (range 12 to
16) in the subcutaneous tissue of the
leg.
The mean vessel diameter was 1.0 mm
(range 0.2 to 1.8 mm).
Anteromedial Group denser with a
straighter course, following the
adjacent great saphenous
vein (GSV) and its branches .
Anterolateral Group sparse, following
a curving course and tending toward
the anteromedial aspect of the
proximal third of the leg

44. *Posterior Group only
one or two large vessels
(mean diameter 1 mm;
range 0.7 to 1.4 mm) in
the posterior aspect of
the leg accompanying the
small saphenous vein
(SSV).

45. Thigh
An average of 29 lymph collectors
(range 27 to 31).
The mean vessel diameter was 0.8
mm (range 0.3 to 1.7 mm).
Anterior Group Vessels originated
from the anterolateral aspect of the
thigh, running obliquely in the
subcutaneous tissue before entering
the lateral group of the superficial
inguinal lymph nodes
Medial Group continuous with the
anteromedial and anterolateral
groups from the leg .
lay adjacent to the GSV and its
branches and entered the center
group of the superficial inguinal
lymph nodes
Lymphatic vessels after injection of a
lead oxide mixture traveling with the
great saphenous vein (GSV) in the calf.
(SILN, Superficial inguinal lymph nodes.)

46. *CLINICAL
MANIFESTATIONS

47. *
*Early detection and treatment
can lead to reversal and
prevention of progression.
* 10% change in limb volume
* a 2-cm change in arm
circumference.
*Tape measurements of arm
circumference at 10 cm intervals
low cost and simplicity.
*An increase in size between
measurements (>10 cm or >10%)
is found to correlate with
subclinical lymphedema.

48. The two most commonly used staging systems for lymphedema are the
International Society of Lymphology and Campisi systems
months or years before any detectable physical change occurs.
As interstitial fluid accumulates, patients experience increased extremity
circumference followed by pitting edema that worsens at the end of the day
A 2 cm or greater difference in arm circumference difference between
affected and nonaffected arms.
alleviated with compressive garments, limb elevation, and physical therapy
with manual lymph drainage and massage to minimize symptoms.
the disease progresses, irreversible, nonpitting edema develops. Patients
report increased firmness, decreased functionality, and disfigurement

52. *Pain/heaviness/fatigue
*• Decreased quality of
life
*• Recurrent Infection
*• Disfigurement

53. *

54. * Typical changes of chronic lymphedema lichenification g, all
Manifestations of cellular proliferation and fibrosis. Lymph cysts h, i
may be observed, and chronic inflammatory changes may also
be present j, k

55. * Characteristic changes of malignant
lymphedema in metastatic breast
* cancer
Pitting edema in
early-stage lymphedema of
the
upper extremity

56. *32-year-old male with
massive lower extremity
lymphedema causing
congestive heart failure
because of excessive blood
flow to the limb.

59. *
Lymphography was historically.

62. *Current guidelines recommend lymphoscintigraphy as the gold
standard to assess the caliber and anatomic location of lymphatic
vessels, functional status, and disease severity.
*duplex ultrasound
*computed tomography scan/ MRI, which can delineate lymphatic
abnormalities at multiple tissue levels.

64. *near-infrared fluorescence (NIRF) imaging has
*highly sensitive, quick and reproducible
techniqueuses indocyanine green (ICG) as an optical
contrast agent.
*high-resolution images that assess contractile
lymphatic flow volume and velocity, the lymphatic
anatomy,including lymph nodes and surrounding
collateral lymphatic network.
*NIRF imaging is equally beneficial
intraoperatively when performing microsurgical
procedures, and postoperatively to evaluate
Posttherapeutic response.

67. • Conservative Treatment:
Complex Decongestive Physiotherapy (CDT)
- Manual Lymph Drainage
- Compression Garment
- Exercise under Compression
- Skin Care
• Surgical Treatment
Management of
Lymphoedema

68. Combined Physical Therapy and
Compression
complex decongestive therapy
The goals of CPT are to:
1. Decrease swelling.
2. Increase lymph drainage from
the congested areas.
3. Reduce skin fibrosis and improve
the skin’s condition.
4. Enhance the patient’s functional
status.
5. Relieve discomfort and improve
quality of life.
6. Reduce the risk of cellulitis and
Stewart-Treves syndrome, a rare
form of angiosarcoma.

69. *Medications
*Diuretics have been tried,
*especially in the first stage of CPT
*. However, they are not routinely used, because
*1- they can cause fluid and electrolyte imbalance and have only a
marginal benefit in reducing peripheral edema.
* 2-may also increase fibrosis because of worsening protein
accumulation.

71. *
Charles RH. Indian Medical Gazette 36: 84-11, 1901.

73. UT MD Anderson by Chang DW

79. Liposuction
Brorson H, Svensson H. Plast Reconstr Surg 102: 1058-1067, 1998.

80. *LIPECTOMY
*removal of fat and fibrosis with
suction technique.
*addresses the solid component
(fibrosis and hypertrophied fat) .
* Indications for lipectomy include
* stage II and III disease that has failed
conservative management.
* Contraindications include active
cancer, infection, wounds, or
insufficient conservative
management.
*If there is more than 4 to 5 mm of
pitting edema in the affected
extremity, the patient should
attempt conservative measures
rather than undergo liposuction.

86. *Lymphovenous Anastomosis
(LVA)
Koshima I, Inagawa K, Urushibara K, et al. J Reconstr Microsurg 16: 437-442, 2000.

87. *first described in 1969,
* a microsurgical procedure
*bypasses diseased lymphatics and restores adequate lymphatic
drainage into the venous system.
*Serial anastomoses small lymphatics and subdermal venules,
preferably less than 1 mm in diameter.
*under locoregional anesthetic.
* indicated after failed management of conservative therapy, and
stage II disease with evidence of partial lymphatic obstruction.

90. certain types of primary
lymphedema are
adequately treated with
LVA.
identify suitable venules
and lymphatic vessels
within the affected limb.
Ideally,
similar diameter,
less than 0.8mm,
in close proximity to one
another, and with minimal
to no venous backflow
after division.

91. * increased number of anastomoses
provided better results.
*a decrease in the overall incidence
of severe cellulitis, compression
garment discontinuation, and a
subjective improvement in
symptom compared with women
who received conservative
management alone.
*5 studies reported 91.7% symptom
improvement, 94.5% average
satisfaction rate, 90% improved
QoL, and 50% subjective
improvement in patients who
underwent LVA.
*BUT no limb volume reduction was
seen in stage III patients.

92. *Vascularized Lymph Node Transfer
* a microsurgical procedure.
* a soft tissue flap containing lymphatic tissue
*and its associated arteriovenous supply is
relocated from a donor site to the axilla.
*restore function in the impaired limb, but
the exact mechanism remains unclear.
1. serve as a“sponge” that absorbs lymphatic
fluid that is, then redistributed back into
the lymphovenous circulatory system.
2. lymphangiogenesis, primarily driven by
vascular endothelial growth factor, leads to
increased lymphatic vessel formation.

93. *Indications
*stages II to V lymphedema.
* absolute occlusion of lymphatic pathways on
imaging (MRI or lymphoscintigraphy).
*fibrosis preventing lymphaticovenous bypass
*brachial plexus neuropathy.
*chronic infections in the affected limb (ie,
repeated episodes of cellulitis).
*Failed conservative management.
*Conversely, some studies support the use of
VLNT in early-stage lymphedema owing to the
progressive course of the disease.
* early intervention may reduce the
accumulation of excess lymphatic fluid,
inhibit the positive feedback cycle that drives
the progression of lymphedema

94. *The optimal donor site remains unclear,
but the most common location is the
inguinal region; it is based off the
branches of the superficial circumflex
iliac or superficial inferior epigastric
vessels. Groin flaps are chosen owing to
their abundance of lymph nodes in a
well-understood anatomic region, an
easily hidden scar, and a dual role in
total breast reconstruction

104. • Non surgical approach
• surgical approach
• LVA-LNVA-VLNT
• DEBULKING
• LIPOSUCTIPON
• CHARLES
• AMPUTATION

107. Thank you