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

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

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