LYMPHOEDEMA
LECTURE BY
DR OKORIE G.
CONSULTANT PLASTIC SURGEON
INTRODUCTION
• DEFINITION:
Abnormal accumulation of Protein–rich fluid in the interstitial spaces
with resultant swelling, inflammation & irreversible tissue changes.
• Typically affects the limbs esp lower limbs
• Chronic , Progressive & Debilitating condition
• Lymphatic system has 3 vital roles: GIF
G = GI Lipid absorption
I = Immune system support
F = Fluid balance
INTRODUCTION Contd
FLUID BALANCE:
• Lymphatic system structure Unidirectional, circulatory network responsible for
REABSORPTION & RETURN of ~ 90% of interstitial ultrafiltrate to circulatory system
• Derangement of fluid balance LYMPHOEDEMA
• Underlying mechanism : DRAINAGE DYSFUNCTION resulting from
1. Malformation Primary Lymphoedema
Insufficient
reabsorption of
ultrafiltrate & transport
back to circulatory system
2. Disruption Secondary Lymphoedema
INTRODUCTION Contd
IMPACT OF LYMPHOEDEMA
• Physical function
• Psychosocial well being
• Quality of life
PATHOPHYSIOLOGY OF
LYMPHOEDEMA
STARLING’S HYPOTHESIS:
• Fluid movt due to filtration across capillary wall is DEPENDENT on the
balance btw the HYDROSTATIC PRESSURE GRADIENT & the ONCOTIC
PRESSURE GRADIENT across the capillary
• STARLING FORCES: 4 forces
1. HYDROSTATIC PRESSURE:
A. Capillary (Pc)
B. Interstitial (Pi)
2. ONCOTIC PRESSURE:
A. Capillary (pc)
PATHOPHYSIOLOGY Contd
• Hydrostatic Pressure Oncotic Pressure
FLUID ACCUMULATION IN THE
INTERSTITAL SPACES
INFLAMMATION FIBROSIS
FURTHER FLUID FURTHER DISRUPTION OF LYMPHATIC
ACCUMULATION CHANNELS
CLASSIFICATION
BASED ON AETIOLOGY:
• PRIMARY UNKNOWN AETIOLOGY
MALFORMATION of Lymphatic channels
• SECONDARY KNOWN AETIOLOGY
DISRUPTION of lymphatic channels
PRIMARY LYMPHOEDEMA
CLASSIFICATION: TIME OF ONSET
1. CONGENITAL LYMPHOEDEMA : BIRTH – 1YR
• Familial Type MILROY’S DISEASE
• Non Familial Type
2. LYMPHAOEDEMA PRAECOX: 2YRS – 35YRS
Familial Type MEIGE’S DISEASE
Non Familial Type
3. LYMPHOEDEMA TARDA: ABOVE 35YRS
CLASSIFICATION Contd
CONGENITAL LYMPHODEMA :
• Incidence : 6-12% of all cases
• Sex Ratio M:F = 1:2 LYMPHOEDEMA TARDA
• Location UL: LL = 1:3 Incidence : ~ 11%
Sex Ratio : F > M
LYMPHOEDEMA PRAECOX:
• Incidence : 77-94% of cases
• Sex Ratio M:F = 1:4
PRIMARY LYMPHOEDEMA &
ASSOCIATED GENETIC DISORDERS
• Diatichiasis lymphoedema syndrome
• Yellow nail syndrome
• Trisomy 21
• Trisomy 13
• Neurofibromatosis type 1
• Haemangioma
• Turner syndrome
• Klinefelter syndrome
SECONDARY LYMPHOEDEMA
• AETIOLOGY KNOWN
1. PARASITIC INFESTATION :
• Wuchereria bancrofti ( Filarial nematode)
• Other Parasites : Brugia malayi, Brugia timori (All cause lymphatic filariasis)
• Commonest cause in Low income countries
2. MALIGNANCY
a. BREAST CANCER RELATED LYMPHOEDEMA (BCRL)
• Commonest cause in middle and high income countries
b. Sentinel Node Mapping / Lymphadenectomy
c. Radiotherapy
3. TISSUE TRAUMA eg Surgery
4. INFECTION
CLINICAL FEATURES
• Chronic swelling of the extremity…..Often painless, initially pitting but
later becomes non pitting
• Fatigue
• Impairment of daily activity
• Psychosocial problems
• Fever, chills
• Generalised weakness
• Recurrent cellulitis
• Skin changes Fissuring, ulcerations, verrucous changes
• KAPOSI STEMMER’S SIGN
CAMPISI STAGING
1a Impaired lymphatic function without evidence of gross
lymphoedema
1b Appearance of Limb swelling reducible with limb elevation
2 Marked swelling not completely reducible by limb elevation
3 Vol of swelling with features of lymphangitis
4 Fibrosis of Lymphatics + Warts
5 Elephantiasis
INTERNATIONAL SOCIETY OF LYMPHOLOGY
LYMPHOEDEMA STAGING CLASSIFICATION
From International Society of Lymphology. The diagnosis and treatment of peripheral lymphedema:
2016
consensus document of the International Society of Lymphology. Lymphology. 2016;49(4):170-184_)
STAGE DESCRIPTION PATHOPHYSIOLOGY CLINICAL FEATURES
O Subclinical Impaired lymph transport Swelling not evident
I Spontaneously Early lymph accumulation Swelling relieved by Limb
reversible elevation
Pitting may be present
II Not spontaneously Fat hypertrophy & Swelling not improved by
reversible Deposition with tissue limb elevation
fibrosis
III Lymphostatic Chronic lymphatic stasis & Swelling not improved by
elephantiasis inflammation. Further Limb elevation
fibrosis & Fatty deposition Oedema nonpitting &
wooden
Hyperkeratotic &
Verrucous skin changes
INVESTIGATIONS
1. IMAGING :
• Lymphoscintigraphy
• Lymphoangiography
• U/S
• Fluorescence microlymphography
• CT Scan
• MRI
2. Biopsy
INVESTIGATIONS C ont’d
3. Serial limb measurement:
A. Tape measurement
B. Perometer
C. Water Displacement
4. BMI
5. FBC
6. Skin snip for microfilariae
TREATMENT
1. NON OPERATIVE
[Link] PHARMACOLOGIC
B. PHARMACOLOGIC
2. OPERATIVE
NON PHARMACOLOGIC
1. PHYSICAL TREATMENT & ADJUVANTS
A. COMPLEX DECONGESTIVENTHERAPY (CDT)
• AKA Combined Physical Therapy(CPT), Complex Decongestive Physiotherapy(CDP)
• Has 2 Phases:
PHASE 1:
• Consist of Skin Care, Manual Lymphatic Drainage(MLD), Muscle Pumping exercises,
Compression
PHASE 2:
• Compression
• +/- MLD
• N/B: Most important Tx option
NON PHARMACOLOGIC Cont’d
B. Compression Garment alone
C. Massage alone
D. Intermittent Pneumatic Compression
E. Thermal Therapy
F. Elevation
G. Low Level Laser
H. DIET
PHARMACOLOGIC
A. BENZOPYRONES:
• Bioflavonoids eg COUMARIN
• Mode Of Action:
• Hydrolyses tissue protein & Facilitate their absorption
• Stimulate lymphatic collectors
• Role of benzopyrones NOT CLEAR
B. DIURETICS:
• Not significant
PHARMACOLOGIC Cont’d
C. ANTIMICROBIALS:
• If there is cellulitis or lymphangitis
D. ANTIPARASITIC DRUGS
PSYCHOSOCIAL REHABILITATION
• Psychosocial support
• Quality Of Life Assessment- Improvement program
• Patient self- efficacy assessment
• All must be integral part of Tx
OPERATIVE TREATMENT
• AIM: Improve lymph return
• Palliative/Curative
• 2 TYPES:
1. Decongestive
2. Physiologic
DECONGESTIVE
1. SISTRUNK:
• Excision of skin down to fascia & primarily closed the incisions
2. HOMANS:
• Thinning of tissues on both sides of the affected limb
3. THOMPSON:
• Similar to Homans but thinning is via one side
4. CHARLES:
• Excision + STSG
5. LIPOSUCTION
PHYSIOLOGIC
• Mainly Microvascular techniques
• Include :
• Lymphatic-Venous shunt
• Lympho-Lymphatic shunt
• Omental flap Transposition
