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TRENDS in
Lmphedema Management Dr. Mansoor Khan MBBS,
FCPS-I
Surgical “D” unit,
Khyber Teaching Hospital,
Peshawar
“Accumulation of abnormal amount of protein rich fluid in the interstitium
due to compromised lymphatic system with (near) normal net
capillary filtration”
In United States
Highest incidence is observed following breast cancer surgery with radiotherapy (10 – 40%).
Worldwide
140-250 million cases of lymphedema are estimated to
exist with filariasis as the most common cause
Lymphatic filariasis affects more than 90 million people
in the world
According to WHO
Lymphatic Filariasis is the 2nd leading cause of permanent & long term disability in the world
after leprosy
Basics of Lymphatic System
Develops from 4 primitive cystic spaces, 2 in the neck and 2 in the groin
Cisterns elongate & develop communications
Condensations along the connections are lymph nodes
* Persistence of primitive cisterns are cystic hygromas
Embryology of lymphatic system
Embryology
Lymphatic capillaries
Blind ended
Large intercellular & intracellular
fenestrations
Allowing macromolecular influx (1000 kDa)
Collagen fibers attachment on outer surface
Dermal papillae
Micronatomy of lymphatic system
Sub papillary pre-collectors
Sub-dermal collector lymphatics
Epifacial, valved, muscular lymphatics
with lymphangions
Subfascial lymphatics
Interconnections at inguinal, anticubital,
axillary levels
Microanatomy of lymphatic system
Capillaries Pre-collectors
Collectors
Deep lymphatic trunk
Anatomy
Pathophysiology
90% 10%
Pathophysiology
Collagen deposition
lymphostasis
Obstruction
Aplasiahypoplasia
Hypocon-tractility
Valvular incompetence
Dermal thickening
Sub dermalfibrosis
LYMPH-EDEMA
Pat
ho
ph
ysio
log
y
LYMPHEDEMA
Primary lymphedema Secondary lymphedema
Congenital Praecox Tarda
Etiology of lymphedema
Congenital lymphedema
< 1year of age
10-25% of all primary lymphedema
Sporadic or familial (Milroy's disease)
More common in males
Lower extremity is involved 3 times more frequently than the upper extremity
2/3 patients have bilateral lymphedema
Aplasia pattern without subcutaneous lymphatic trunks involvement
Evident after birth and before age 35 years
Most often arises during puberty
65-80% of all primary lymphedema cases
Females are affected 4 times
70% of cases are unilateral, with the left lower extremity being involved
Hypoplastic pattern, with the lymphatics reduced in caliber and number
Lymphedema Precox
Clinically not evident until 35 years or older
Rarest form of primary lymphedema
Only 10% of cases
Hyperplasic pattern, with tortuous lymphatics increased in caliber and number
Absent or incompetent valves
Lymphedema Tarda (Meige disease )
Secondary Lymphedema
Most common lymphedema having well recognized causes
Filariasis
Commonest cause worldwide
Endemic in 72 countries
Affecting 5-10% population Africa, India, South America
Endemic areas of Filariasis
Filariasis
Wuchereria Bancrofti (90%)
Brugia malayi
Brugia timori
Other causes of Secondary Lymphedema
Breast surgery with radiotherapy
Primary malignancy
Prostate, cervical cancer, malignant melanoma
Trauma to lymphatics
Surgical excision of lymph nodes
Presentation of lymphedema
Age of onset
Painless swelling
Presence or absence of family history
Coexistent pathology
Presentation of lymphedema
Characteristically foot involvement
Ankle contours are lost with infilling of the submalleolar depressions
Buffalo hump on foot dorsum
Square shaped toes
Stemmer’s sign
Skin changes
Chronic eczema
Dermatophytosis
Fissuring
Verrucae
Ulcerations
Stewart Treves syndrome
Presentation of lymphedema
Chyluria, chylous ascites, chylothorax,
Lymphorrhoea
MEGALYMPHATICS
Brunner Classification
0 Histological abnormalitiesNot clinical evident
I Pitting edema, Subsides with elevation
II Non pitting edemaNot relieved with elevation
III Irreversible skin changes,fibrosis, papillae
Investigations
Investigations
Infrequently required to establish the diagnosis
To determine residual lymphatic function
To establish treatment preferences
To evaluate therapy
Contrast Lymphangiography
Was gold standard for mapping
Damages the normal lymphatic channels due to inflammation
Very painful procedure and needs GA
Lymphangiogram
Lymphangiogram
Isotope Lymphoscintigraphy
Replaced the earlier
Technetium labeled antimony sulphide
Dye needs to be injected in toe web through a 27 G needle
Lymphoscintigram
An indication for CT scan or MRI
is suspicion of malignancy,
for which these tests offer the most information
MRI Scan
Blood slide (Microfilaria)
Blood slide
Adult worms in lymph nodes
Others
Eosinophilia
Increased IgE levels
Compliment fixation test
Antigens of filaria
Treatment
TREATMENT
Conservative Surgical
Conservative
Physical Medication
Complex Lymphedema Therapy (CLT)
Manual lymphatic drainage (MLD)*
(massage to make the flow to normal lymphatics)
Low stretch bandaging
(to prevent re-accumulation)
*Vodder and/or Leduc techniques
CLT
Intermittent pneumatic pump compression therapy
Effectively milking the lymph
from the extremity
Compression garment
To help prevent return of fluid
Skin care
(Examine, dry, moisturizers)
Exercises
Psychological support
& occupational therapy
Antiparasitic agents
Diethylecarbimazole 6mg/kg single dose or 1-3wk
(Don’t use in pregnancy, infants, elderly)
Ivermectin (400mcg/kg/d)
Tetracycline
Doxycycline (100mg/day for 6-8 wks)
Antibiotic
For skin infections
Penicillin V 500mg tds for streptococcal
Flucloxacilline 250mg qid for staphylococcal
Infections
Miconazole 1% skin ointment
Or systemic antifungal
Hydroxyrutosides/ coumadins
Binds wit proteins, engulfed by macrophages leading to proteolysis
Surgical Procedures for Lymphedema
Surgical
Ablative/reduction Bypass surgeries
Ablative surgeries
Sistrunk procedure
Homan procedure
Thompson procedure
Charles procedure
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
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
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
Bypass surgeries
Lymph node anastamosis with veins
Lymphovenous anastamosis
Lym
ph
edem
a
Thanks