الله بسمالرحيم الرحمن
Surgery of lymphatic
systemlymphedem
a
LOWER
LIMB
LYMPHED
EMA
H A ME D H
O R E YA P R OF E S S O
R OF S
U R G E R Y
MANSOURA
E G Y
P T
Lympedema Is hypertrophy of the skin and subcutaneous tissue caused by chronic lymphatic obstruction .
It affects extremities , scrotum , external genitalia , and rarely the breasts
LymphedemaoAn abnormal accumulation of protein-rich fluid in the interstitium, causing chronic inflammation and reactive fibrosis of the affected tissues
Lymph Vessels
oCapillariesoPre-collectorsoCollectorsoTrunks
Trunks & DuctsoLargest lymph vesselsoThoracic duct-largest,
pumping by the diaphram. oForm in cisterna chyleoDucts empty into venous
systemLower Body Upper Body•R & L Lumbar Trunks•Intestinal Trunks
•R & L Jugular•R & L Subclavian•R & L Broncho-mediastinal
Types of LymphedemaPrimary
o Hypoplasia (not as many lymph nodes)
o Hyperplasiao Aplasiao Inguinal Node Fibrosiso (Kineley Syndromeo Milroy’s Disease-congentital,
males, unilateral typicallyo Meige’s Syndrome: most
females around puberty, Bilateral, webbing of fingers and toes, two rows of lashes
Secondaryo Surgeryo Radiation Therapyo Trauma: blunt
traumao Filariasis: parasite,
blocks lymph nodeso Cancer (Malignant)o Infectiono Obesityo 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 oedemaSkin changes –thickening , hyperkeratosis and lymphatic vesicles but no ulcers
Huge bulges of the skin in severe cases –elephantiasis
Skin changeso Chronic eczemao Dermatophytosiso Fissuringo Verrucaeo Ulcerationso Stewart Treves syndrome
Stages of Lymphedema
Latency Stageo Reduced transport capacityo No noticeable edema
Stage Io Pitting edemao Edema reduces with elevation (no fibrosis)o Tight sleeve during the day
Stage IIo Pitting becomes progressively more difficulto Connective tissue proliferation (fibrosis)
Stage IIIo Non pittingo Fibrosis and Sclerosiso Skin changes (papillomas, hyperkeratosis, etc)
Brunner Classification 0 Histological abnormalities
Not clinical evident
I Pitting edema, Subsides with elevation
II Non pitting edemaNot 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 dorsumo Square shaped toes o Stemmer’s sign
1 -primary lymphedema
Congenital
Precoxtarda
a-Congenital lymphedema
o < 1year of ageo 10-25% of all primary lymphedema o Sporadic or familial (Milroy's
disease)o More common in maleso 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
caseso 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 injuryo Infectiono CVI -- Obesity
11 –Secondary lymphedema
1 – post-traumaticoa- circumferential scarob- block dissection of regional nodes
oc- burnsod- irradiation of regional l.
nodes
11 –Secondary lymphedema
2 – post-inflammatory oa- non-specific recurrent lymphangitis recurrent cellulitis post-erysipelasob- specific Ffilaria filaria ---T.B.
Filariasis Commonest cause worldwideEndemic 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
limbsoCaused by Wuchereria
bancrofti oCommon in Damietta – Giza –
Sharkia and Assiut.oDue to obstruction of lymph
nodes by the adult worms.oLymph 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
limbsoCellular reaction to proteins in the form of plasma cells – eosinophils –lymphocytes – monocytes and polymorpho-nuclear leukocytes.
oFibrosis and collage fibersoAllergy to filarial antigens
Skin changeso Chronic eczemao Dermatophytosiso Fissuringo Verrucaeo Ulcerationso Stewart Treves syndrome
11 –Secondary lymphedema
3 – neoplastic
a- primary lymphoma
b- metastasis
Malignant lymphedema
oPain, paresthesia, paralysisoCentral location, proximal onsetoRapid development, continuous progression
Malignant lymphedema
oSwelling and nodules in supraclavicular fossaoHematoma-like discoloration (angiosarcoma)oUlcers and non-healing open woundsoRecurrent 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:oFilariasis: oMyxedema: decreased ability
to sweat, orange skinoComplex Regional Pain
Syndrome (RSD, Sudeck’s)
Filarial lymphedema of
lower limbsdifferential diagnosis
• 1- Post-phlebitic limb
CVIoGaiter distributionoNon-pittingoBrawnyoHemosiderin
stainingoFibrosis of
subcutaneouso tissueoAtrophic skin
Filarial lymphedema of
lower limbsdifferential diagnosis
• 2 –elephantiasis neuro-fibromatosis
Filarial lymphedema of
lower limbsdifferential diagnosis
• 3-Congenital A-V fistula (klippel Trenaunay syndrome )
Klippel-Trénaunay syndrome
Klippel–Trénaunay–Weber :o Large port-wine staino Varicose vein with or without
A – V fistulao Hypertrophy of soft tissue and boneo Most commonly lower limbTreatment = 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 failureo Deep vein thrombosiso Venous insufficiencyo Hypoalbuminemiao Vascular
malformations
Differential Diagnosis
oLipedema ( usually sparing the feet )
oidiopathicoFilariasisoMyxedemaoComplex regional pain syndrome
oMalignancy / active cancer
Filarial lymphedema of
lower limbsdifferential diagnosis
• 4 – other causes
Lipedemao Mainly in womeno Bilateral, symmetrical
edema o from iliac crest to
ankleso Dorsum of feet never
involved o (-) Stemmer’s signo Little or no pittingo No cellulitiso Painful to palpation
Acute DVTo Sudden onseto Unilateralo Painfulo Cyanosiso (+) Homan’s signo Potentially lethal (PE)o Diagnosis with venous doppler
Cardiac edemaoRight heart insufficiencyoGreatest edema distallyoAlways bilateraloPittingoComplete resolution with
elevationoNo painoMay treat with PT if cleared by
Cardiologist
Lymphedema
Diagnosis and
Therapy
Filarial lymphedema of
lower limbsDiagnosiso1 – clinicalo2 – midnight blood film to show microfilaria
o3 – intradermal skin test
Investigationso Infrequently required to
establish the diagnosis
o To determine residual lymphatic function
o To establish treatment preferences
o To evaluate therapy
Other Diagnostic Tests
oLymphographyoVenous Doppler or Venous
Sonographyo Indirect LymphographyoFluorescence
MicrolymphographyoLymphoscintigraphyo CT ScanoMRI
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
Treatment1 - conservative • Pneumatic compression
pump• Complete decongestive therapy (CDT)
• Elastic support garments• Medications
Treatment •1 – conservative :-oLimb hygiene , elevation , massage
oElastic stockingoDiuretics ?????oLong term antibioticsoDiethyl 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
PumpsAdvantages:1. Can be used at home by patients2. Fast application3. Financially lucrative for DME vendors
($4000 per pump)
Manual Lymph Drainage
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 tissueoc - Physiological and excisional
treatmentoa – physiological operations
oEntero-mesenteric bridgeoOmental flapoMicro-lymphatic-venous anastomosis
oLympho-venous anastomosis
treatmentob – 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 skinGirth can be greatly reducedUnsatisfactory cosmetic results
Sistrunk Procedure (1918)
Wedge of skin & subcutaneous tissue excised & wound closed primarily Most commonly used to reduce girth of thigh
Homan ProcedureSkin flaps are elevatedSubcutaneous tissue excisedSkin flap trimmed & closedUsually staged procedure with lateral & medial separated by 3-6 months to avoid necrosisMostly 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
لله الحمد
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