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‫الرحيم‬ ‫الرحمن‬ ‫هللا‬ ‫بسم‬
Surgery of
lymphatic system
lymphedema
Lympedema
Is hypertrophy of the skin
and subcutaneous tissue
caused by chronic lymphatic
obstruction .
It affects extremities ,
scrotum , external genitalia ,
and rarely the breasts
Lymphedema
oAn abnormal accumulation of
protein-rich fluid in the
interstitium, causing chronic
inflammation and reactive
fibrosis of the affected
tissues
Lymph Vessels
oCapillaries
oPre-collectors
oCollectors
oTrunks
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-
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
Lower extremity
lymphedema
I- primary
lymphedema
II – secondary
lymphedema
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.
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
Skin changes
o Chronic eczema
o Dermatophytosis
o Fissuring
o Verrucae
o Ulcerations
o Stewart Treves syndrome
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)
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
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
1- primary lymphedema
Congenital
Precox
tarda
 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
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
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 )
Primary Lymphedema of the
Left Leg
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
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
11– Secondary lymphedema
2 – post-inflammatory
o a- non-specific
 recurrent lymphangitis
 recurrent cellulitis
 post-erysipelas
o b- specific
 Ffilaria filaria ---T.B.
Filariasis
Commonest cause worldwide
Endemic in 72 countries
Affecting 5-10% population
Africa, India, South America
Filariasis
Prevalent in 3rd world countries;
Can still be treated successfully with CDT.
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.
Endemic areas of Filariasis
Blood slide (Microfilaria)
Adult worms in lymph nodes
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
Skin changes
o Chronic eczema
o Dermatophytosis
o Fissuring
o Verrucae
o Ulcerations
o Stewart Treves syndrome
11– Secondary lymphedema
3 – neoplastic
a- primary lymphoma
b- metastasis
Malignant lymphedema
oPain, paresthesia, paralysis
oCentral location, proximal
onset
oRapid development,
continuous progression
Malignant lymphedema
oSwelling and nodules in
supraclavicular fossa
oHematoma-like
discoloration (angiosarcoma)
oUlcers and non-healing
open wounds
oRecurrent malignancy
Stewart-Treves
Syndrome
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.
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)
Filarial lymphedema of
lower limbs
differential diagnosis
• 1- Post-
phlebitic
limb
CVI
o Gaiter distribution
oNon-pitting
o Brawny
oHemosiderin staining
o Fibrosis of
subcutaneous
o tissue
o Atrophic skin
Filarial lymphedema of
lower limbs
differential diagnosis
• 2 –elephantiasis
neuro-fibromatosis
Filarial lymphedema of
lower limbs
differential diagnosis
• 3-Congenital A-V
fistula (klippel
Trenaunay
syndrome )
Klippel-Trénaunay
syndrome
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
Differential diagnosis
o Congestive heart failure
o Liver and renal failure
o Deep vein thrombosis
o Venous insufficiency
o Hypoalbuminemia
o Vascular malformations
Differential Diagnosis
oLipedema ( usually sparing the
feet )
oidiopathic
oFilariasis
oMyxedema
oComplex regional pain syndrome
oMalignancy / active cancer
Filarial lymphedema of
lower limbs
differential diagnosis
• 4 – other
causes
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
Acute DVT
o Sudden onset
o Unilateral
o Painful
o Cyanosis
o (+) Homan’s sign
o Potentially lethal (PE)
o Diagnosis with venous doppler
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
Lymphedema
Diagnosis
and
Therapy
Filarial lymphedema of
lower limbs
Diagnosis
o1 – clinical
o2 – midnight blood film to
show microfilaria
o3 – intradermal skin test
Investigations
o Infrequently required to establish
the diagnosis
o To determine residual lymphatic
function
o To establish treatment preferences
o To evaluate therapy
Other Diagnostic
Tests
o Lymphography
o Venous Doppler or Venous
Sonography
o Indirect Lymphography
o Fluorescence Microlymphography
o Lymphoscintigraphy
o CT Scan
oMRI
Contrast Lymphangiography
Was gold standard for mapping
Damages the normal lymphatic
channels
due to inflammation
Very painful procedure and needs GA
Dye needs to
be injected in
toe web
through a 27 G
needle
Lymphoscintigram
T 99 labelled colloidal particles with antimony sulphide
Normally after 90 min. 40%of the radioactive dye disappear at site of inj.
o An indication for
CT scan or MRI
o is suspicion of
malignancy,
o for which these
tests offer the
most information
MRI Scan
TREATMENT
Conservative Surgical
Conservative
Physical Medication
 Treatment
1 - conservative
• Pneumatic compression pump
• Complete decongestive
therapy (CDT)
• Elastic support garments
• Medications
Treatment
•1 – conservative :-
oLimb hygiene , elevation ,
massage
oElastic stocking
oDiuretics ?????
oLong term antibiotics
o Diethyl carbamazine ( anti-filarial
Complete Decongestive
Therapy (CDT)
• Skin Care
• Manual Lymph Drainage
• Compression Therapy
• Remedial exercise
CLT
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
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
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
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
MedaFit
garments
Sequential Pneumatic
Devices
Pneumatic Compression
Pumps
Advantages:
1. Can be used at home by patients
2. Fast application
3. Financially lucrative for DME vendors ($4000
per pump)
Manual Lymph Drainage
Night-time garments
Night-time Garments
Surgical
Ablative/reduction Bypass surgeries
treatment
•2 – surgical :-
oa – physiological i.e. improve
lymph drainage
ob – excisional i.e. reduce the
bulk of tissue
oc - Physiological and excisional
treatment
oa – physiological operations
oEntero-mesenteric bridge
oOmental flap
oMicro-lymphatic-venous
anastomosis
oLympho-venous anastomosis
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
Surgery
• Microsurgical techniques
• Liposuction
• Debulking/Reduction procedures
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
Sistrunk Procedure (1918)
Wedge of skin & subcutaneous
tissue excised & wound closed
primarily
Most commonly used to reduce
girth of thigh
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
Treatment
oc – phsiological and excisional
 Thompson”s , swiss roll ,
excision of skin and subcut.
Tissues then implantation of
the flaps between the muscles
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
‫هلل‬ ‫الحمد‬

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