This document discusses lymphedema, which is swelling caused by a blockage in the lymphatic system. It can affect the extremities, genitals, and breasts. There are two main types - primary lymphedema due to genetic issues or hypoplasia/aplasia of lymph vessels, and secondary lymphedema usually due to surgery, radiation, trauma, infection, or filariasis. Diagnosis is usually clinical but tests like lymphoscintigraphy can help assess lymphatic function. Treatment involves compression therapy, manual lymph drainage, exercise, and compression garments long-term, as well as surgical procedures to improve drainage or reduce tissue.

1. ‫الرحيم‬ ‫الرحمن‬ ‫هللا‬ ‫بسم‬

2. Surgery of
lymphatic system
lymphedema

5. Lympedema
Is hypertrophy of the skin
and subcutaneous tissue
caused by chronic lymphatic
obstruction .
It affects extremities ,
scrotum , external genitalia ,
and rarely the breasts

6. Lymphedema
oAn abnormal accumulation of
protein-rich fluid in the
interstitium, causing chronic
inflammation and reactive
fibrosis of the affected
tissues

9. Lymph Vessels
oCapillaries
oPre-collectors
oCollectors
oTrunks

11. Trunks & Ducts
oLargest lymph vessels
o Thoracic duct-largest, pumping by
the diaphram.
o Form in cisterna chyle
o Ducts empty into venous system
Lower Body Upper Body
•R & L Lumbar Trunks
•Intestinal Trunks
•R & L Jugular
•R & L
Subclavian
•R & L Broncho-

14. Types of Lymphedema
Primary
o Hypoplasia (not as many lymph
nodes)
o Hyperplasia
o Aplasia
o Inguinal Node Fibrosis
o (Kineley Syndrome
o Milroy’s Disease-congentital,
males, unilateral typically
o Meige’s Syndrome: most females
around puberty, Bilateral, webbing of
fingers and toes, two rows of
lashes
Secondary
o Surgery
o Radiation Therapy
o Trauma: blunt trauma
o Filariasis: parasite,
blocks lymph nodes
o Cancer (Malignant)
o Infection
o Obesity
o Self Induced

15. Lower extremity
lymphedema
I- primary
lymphedema
II – secondary
lymphedema

16. Pathology
Accumulation of large
amount of protein-rich
fluids in the tissue due to
stagnation of lymph.
Recurrent attacks of
lymphangitis lead to more
lymphatic obstruction.

17. Pathological changes
Swelling due to fluid
accumulation
Early pitting oedema
Skin changes –thickening ,
hyperkeratosis and lymphatic
vesicles but no ulcers
Huge bulges of the skin in
severe cases –elephantiasis

18. Skin changes
o Chronic eczema
o Dermatophytosis
o Fissuring
o Verrucae
o Ulcerations
o Stewart Treves syndrome

21. Stages of Lymphedema
 Latency Stage
o Reduced transport capacity
o No noticeable edema
 Stage I
o Pitting edema
o Edema reduces with elevation (no fibrosis)
o Tight sleeve during the day
 Stage II
o Pitting becomes progressively more difficult
o Connective tissue proliferation (fibrosis)
 Stage III
o Non pitting
o Fibrosis and Sclerosis
o Skin changes (papillomas, hyperkeratosis, etc)

22. Brunner Classification
0 Histological abnormalities
Not clinical evident
I Pitting edema,
Subsides with elevation
II Non pitting edema
Not relieved with elevation
III Irreversible skin changes,
fibrosis, papillae

23. Presentation of lymphedema
o Characteristically foot involvement
o Ankle contours are lost with infilling of
the submalleolar depressions
o Buffalo hump on foot dorsum
o Square shaped toes
o Stemmer’s sign

25. 1- primary lymphedema
Congenital
Precox
tarda

26.  a-Congenital lymphedema
o < 1year of age
o 10-25% of all primary lymphedema
o Sporadic or familial (Milroy's disease)
o More common in males
o Lower extremity is involved 3 times more
frequently than the upper extremity
o 2/3 patients have bilateral lymphedema
o Aplasia pattern without subcutaneous
lymphatic trunks involvement

28. o Evident after birth and before age 35 years
o Most often arises during puberty
o 65-80% of all primary lymphedema cases
o Females are affected 4 times
o 70% of cases are unilateral, with the left
lower extremity being involved
o Hypoplastic pattern, with the lymphatics
reduced in caliber and number
 b-Lymphedema Precox

31. o Clinically not evident until 35 years or
older
o Rarest form of primary lymphedema
o Only 10% of cases
o Hyperplasic pattern, with tortuous
lymphatics increased in caliber and number
o Absent or incompetent valves
 c-Lymphedema Tarda
(Meige disease )

32. Primary Lymphedema of the
Left Leg

36. 11-Secondary lymphedema
o There is a known cause for the presence
of edema
o Surgery: breast cancer, melanoma,
prostate/bladder cancer, lymphoma,
ovarian cancer, Radiation therapy
o Trauma – scarring, crush injury
o Infection
o CVI -- Obesity

37. 11– Secondary lymphedema
1 – post-traumatic
o a- circumferential scar
ob- block dissection of regional nodes
o c- burns
od- irradiation of regional l. nodes

40. 11– Secondary lymphedema
2 – post-inflammatory
o a- non-specific
 recurrent lymphangitis
 recurrent cellulitis
 post-erysipelas
o b- specific
 Ffilaria filaria ---T.B.

42. Filariasis
Commonest cause worldwide
Endemic in 72 countries
Affecting 5-10% population
Africa, India, South America

43. Filariasis
Prevalent in 3rd world countries;
Can still be treated successfully with CDT.

45. Filarial lymph-edema
of lower limbs
o Caused by Wuchereria bancrofti
o Common in Damietta – Giza –
Sharkia and Assiut.
o Due to obstruction of lymph nodes
by the adult worms.
o Lymph stagnation leads to
accumulation of proteins in the
subcutaneous tissues.

47. Endemic areas of Filariasis

49. Blood slide (Microfilaria)

50. Adult worms in lymph nodes

51. Filarial lymph-edema
of lower limbs
oCellular reaction to proteins
in the form of plasma cells –
eosinophils –lymphocytes –
monocytes and polymorpho-
nuclear leukocytes.
oFibrosis and collage fibers
oAllergy to filarial antigens

52. Skin changes
o Chronic eczema
o Dermatophytosis
o Fissuring
o Verrucae
o Ulcerations
o Stewart Treves syndrome

56. 11– Secondary lymphedema
3 – neoplastic
a- primary lymphoma
b- metastasis

57. Malignant lymphedema
oPain, paresthesia, paralysis
oCentral location, proximal
onset
oRapid development,
continuous progression

62. Malignant lymphedema
oSwelling and nodules in
supraclavicular fossa
oHematoma-like
discoloration (angiosarcoma)
oUlcers and non-healing
open wounds
oRecurrent malignancy

63. Stewart-Treves
Syndrome

66. Differential Diagnosis
o Lipidema: females, symmetrical (no feet), no
pitting, very painful to palpations, bruise
easily, tissue is softer.
o Chronic Venous Insufficiency: gaiter
distribution, non-pitting, hemosiderin staining,
fibrotic.
o Acute Deep Venous Thrombophlebitis: swelling,
redness, painful, sudden onset
o Cardiac Edema: bilateral, pitting, complete
resolution when legs elevate above heart, no
pain.

67. Differential Diagnosis
.
oCongestive Heart Failure: pitting,
dyspnea, jugular vein distention.
oMalignancy:
o Filariasis:
o Myxedema: decreased ability to
sweat, orange skin
o Complex Regional Pain Syndrome
(RSD, Sudeck’s)

69. Filarial lymphedema of
lower limbs
differential diagnosis
• 1- Post-
phlebitic
limb

70. CVI
o Gaiter distribution
oNon-pitting
o Brawny
oHemosiderin staining
o Fibrosis of
subcutaneous
o tissue
o Atrophic skin

75. Filarial lymphedema of
lower limbs
differential diagnosis
• 2 –elephantiasis
neuro-fibromatosis

86. Filarial lymphedema of
lower limbs
differential diagnosis
• 3-Congenital A-V
fistula (klippel
Trenaunay
syndrome )

91. Klippel-Trénaunay
syndrome

92. Klippel–Trénaunay–Weber :
o Large port-wine stain
o Varicose vein with or without
A – V fistula
o Hypertrophy of soft tissue and bone
o Most commonly lower limb
Treatment = expectancy and supportive
elastic stocking – orthopedic
procedures embolization tech. or pulsed -
dye laser.
• 3-congenital A-V fistula

95. Differential diagnosis
o Congestive heart failure
o Liver and renal failure
o Deep vein thrombosis
o Venous insufficiency
o Hypoalbuminemia
o Vascular malformations

96. Differential Diagnosis
oLipedema ( usually sparing the
feet )
oidiopathic
oFilariasis
oMyxedema
oComplex regional pain syndrome
oMalignancy / active cancer

98. Filarial lymphedema of
lower limbs
differential diagnosis
• 4 – other
causes

99. Lipedema
o Mainly in women
o Bilateral, symmetrical edema
o from iliac crest to ankles
o Dorsum of feet never
involved
o (-) Stemmer’s sign
o Little or no pitting
o No cellulitis
o Painful to palpation

102. Acute DVT
o Sudden onset
o Unilateral
o Painful
o Cyanosis
o (+) Homan’s sign
o Potentially lethal (PE)
o Diagnosis with venous doppler

104. Cardiac edema
o Right heart insufficiency
o Greatest edema distally
o Always bilateral
oPitting
o Complete resolution with elevation
oNo pain
o May treat with PT if cleared by
Cardiologist

106. Lymphedema
Diagnosis
and
Therapy

107. Filarial lymphedema of
lower limbs
Diagnosis
o1 – clinical
o2 – midnight blood film to
show microfilaria
o3 – intradermal skin test

108. Investigations
o Infrequently required to establish
the diagnosis
o To determine residual lymphatic
function
o To establish treatment preferences
o To evaluate therapy

109. Other Diagnostic
Tests
o Lymphography
o Venous Doppler or Venous
Sonography
o Indirect Lymphography
o Fluorescence Microlymphography
o Lymphoscintigraphy
o CT Scan
oMRI

110. Contrast Lymphangiography
Was gold standard for mapping
Damages the normal lymphatic
channels
due to inflammation
Very painful procedure and needs GA

111. Dye needs to
be injected in
toe web
through a 27 G
needle

112. Lymphoscintigram
T 99 labelled colloidal particles with antimony sulphide
Normally after 90 min. 40%of the radioactive dye disappear at site of inj.

113. o An indication for
CT scan or MRI
o is suspicion of
malignancy,
o for which these
tests offer the
most information
MRI Scan

115. TREATMENT
Conservative Surgical

116. Conservative
Physical Medication

117.  Treatment
1 - conservative
• Pneumatic compression pump
• Complete decongestive
therapy (CDT)
• Elastic support garments
• Medications

118. Treatment
•1 – conservative :-
oLimb hygiene , elevation ,
massage
oElastic stocking
oDiuretics ?????
oLong term antibiotics
o Diethyl carbamazine ( anti-filarial

119. Complete Decongestive
Therapy (CDT)
• Skin Care
• Manual Lymph Drainage
• Compression Therapy
• Remedial exercise

120. CLT

121. Compression Therapy
• Compression therapy is the
application of external pressure
on body tissue to support the
elasticity of the skin and its
underlying vessels
• Phase I with Compression
Bandages
• Phase II with medical
compression Garments

122. Compression bandages
• Compression bandages have
been shown to produce a
micromassage effect that
improves lymph transport.
• Increase temperature of up
to 5 degrees enhances the
lymphangion mobility

123. Compression bandaging
Short stretch bandages (Rosidal, Comprilan)
are applied to increase the tissue pressure
in the edematous extremity.
• Reduces the ultrafiltration rate
• Improves efficiency of the muscle and joint
pumps
• Prevents re-accumulation of evacuated
lymph fluid
• Helps break down fibrous connective tissue
that has developed

125. Sequential Pneumatic Devices
• Mobilizes interstitial fluid into the
venous system
• Single chamber - JOBST vs.
sequential Compression (gradient)
• Use MLD prior to using the pump
• Studies show that it moves only
venous fluid
• Pump never to exceed 40 mmHg for
extended periods of time

126. MedaFit
garments

127. Sequential Pneumatic
Devices

129. Pneumatic Compression
Pumps
Advantages:
1. Can be used at home by patients
2. Fast application
3. Financially lucrative for DME vendors ($4000
per pump)

130. Manual Lymph Drainage

131. Night-time garments

132. Night-time Garments

133. Surgical
Ablative/reduction Bypass surgeries

135. treatment
•2 – surgical :-
oa – physiological i.e. improve
lymph drainage
ob – excisional i.e. reduce the
bulk of tissue
oc - Physiological and excisional

136. treatment
oa – physiological operations
oEntero-mesenteric bridge
oOmental flap
oMicro-lymphatic-venous
anastomosis
oLympho-venous anastomosis

140. treatment
ob – excisional operations :
 Charies ,excision of skin and
subcutaneous tissues and split
thickness skin graft
 Sistrunk”s , excision of skin
and subcut. Ellipse , then close
the defect

141. Surgery
• Microsurgical techniques
• Liposuction
• Debulking/Reduction procedures

144. Charles Procedure
(1912)
Excision of all
skin/subcutaneous tissue
down to deep fascia
Covering by split thickness
skin grafts from the excised
skin
Girth can be greatly reduced
Unsatisfactory cosmetic
results

145. Sistrunk Procedure (1918)
Wedge of skin & subcutaneous
tissue excised & wound closed
primarily
Most commonly used to reduce
girth of thigh

146. Homan Procedure
Skin flaps are elevated
Subcutaneous tissue
excised
Skin flap trimmed &
closed
Usually staged procedure
with lateral & medial
separated by 3-6 months
to avoid necrosis
Mostly for calf

148. Treatment
oc – phsiological and excisional
 Thompson”s , swiss roll ,
excision of skin and subcut.
Tissues then implantation of
the flaps between the muscles

151. Thompson Procedure (1962)
Denuded skin flaps sutured to deep fascia &
buried (buried dermal flap)
To establish connection b/w superficial and
deep systems
Formation of pilonidal sinus

153. ‫هلل‬ ‫الحمد‬