15/03/2021
Lymphedema
Dr Vanitha
Dr H V Shivaram
Plan for talk
History Investigations
Anatomy Medical management
Pathophysiology Surgical Management
Staging of Lymphedema
Differential Diagnosis
History
Hippocrates – 3 lymphatic temperaments : phlegm
(Lymph and chyle), yellow bile & black bile.
Aselli pointed the differences between lymph vessels
and veins and was the first to describe the lacteals.
"William Hunter wrote that the Lymphatic System
created a " grand system for absorption, in men and
quadrapeds".
Anatomy of
Lymphatic System
Three elements :
1. Initial or terminal lymphatic capillaries
2. Collecting vessels
3. Lymph nodes
Pathophysiology
Inability of existing lymphatic system to
accommodate the protein and fluid entering the
interstitial compartment at the tissue level.
Three stages:
Stage 1 : Impaired lymphatic drainage results in
protein rich fluid accumulation in the interstitial
compartment ,clinically manifested as soft,
pitting edema.
Stage 2 : Accumulation of fibroblasts,
adipocytes and macrophages ,in the
affected tissues --> Local inflammatory
response.
Structural changes at skin and
subcutaneous level
Clinically, tissue edema is more
pronounced and non-pitting and has
a spongy consistency
Stage 3 : Advanced stage
Affected tissues sustains injuries due to local inflammatory
response and recurrent infectious episodes because of minimal
subclinical breaks in skin.
Repeated episodes injures the incompetent ,remaining
lymphatic channels worsening the lymphatic insufficiency.
Subcutaneous fibrosis and scarring + skin changes--
> Lymphostatic elephantiasis.
Lymphatic Causes
Primary Lymphedema Secondary Lymphedema
✓ Milroy disease Infections
✓ Meige disease Cancer treatment
✓ Lymphedema precox Trauma
✓ Lymphedema tardum Inflammation
✓ Lymphangiomas
✓ Lymphatic malformations
Classification
Primary – no known cause
Secondary – Cause is a known disease or a disorder
Primary lymphedema is classified based on age of onset and history of
familial clustering.
Congenital – Onset is before first year of life
Familial version of congenital lymphedema – Milroy disease, dominant
inheritence
Classification …..
Primary lymphedema – b/n 1-35 years – Lymphedema Praecox
Familial version – Meige disease
After 35 years – Lymphedema Tarda
Incidence of primary lymphedema – 1 in 10,000
Most common – Praecox (80%)
Congental and tarda – 10% each
Secondary lymphedema
Most common cause – Filariasis (infestation of lymph nodes by the parasite
Wuchereria Bancrofti)
Resection or ablation of regional lymph nodes by surgery
Radiation therapy
Tumor invasion
Direct trauma
Infection (less common)
Lymphatic malformations
Chronic Venous Insufficiency
Hypoalbuminemia
Non Drug induced edema
Lymphatic
Inflammatory diseases
Causes
Factitious
Congestive heart failure
Clinical features
Edematous limb - firm or hard in consistency
Loss of peri-malleolar shape--> "Tree-Trunk" pattern
Dorsum of foot is swollen-->buffalo-hump appearance
Toes – thick and squared
Positive Stemmer's sign
Skin changes in advanced disease:
Lichenification
Peau d' orange appearance
Hyperkeratosis
H/o Recurrent cellulitis & Lymphangitis after trivial trauma
Fungal infections of forefoot and toes
Complications
Slow wound healing
Lymphangitis , Lymphadenitis
Ulceration and thickening of skin
Malignancy – Lymphangiosarcoma (Sterwart Treves Syndrome)
Retiform Haemangioendothelioma
Differential diagnosis
Chronic Venous Insufficiency
Central organ failure (Heart/Renal)
Isolated lymphedema - No hyperpigmentation/ulceration
Lymphedema does not respond to overnight elevation
Other causes to be evaluated
Hypoproteinemia secondary to Cirrhosis
Nephrotic Syndrome
Malnutrition
Lipedema (excessive subcutaneous fat,b/l,non – pitting and greatest at
ankles and legs,feet are spared,family history)
Differentiation b/n venous and
lymphatic cause
Venous insufficiency
Pitting type of edema
Ankles and legs, sparing feet
Responds promptly to overnight elevation
Advanced stage – Atrophic skin with brawny pigmentation
Ulceration – above/posterior and beneath the malleoli
Investigations
Routine tests Special tests
TLC,DLC RA
LFT,RFT Antinuclear antibody
Blood Sugar levels,Thyroid
function tests
Blood smear for Microfilariae
Total plasma proteins and serum
albumin
Chest x ray
Ultrasound
Diagnosis
Second and third stages – diagnosis is relatively easy
First stage (mild, pitting edema, relieves with simple elevation) - Difficult
Suspected secondary – CT & MRI
Suspected primary – Lymphoscintigraphy
However if lymphoscintigraphy shows delayed lymphatic drainage,
diagnosis of primary lymphedema should never be made until neoplasia
involving the regional and central lymphatic drainage is excluded though
CT or MRI.
LYMPHOSCINTIGRAPHY
ISOTOPE LYMPHOGRAPHY
Test of choice for suspected
lymphedema
Cannot differentiate between primary
and secondary lymphedemas
Sensitivity – 70% and Specificity –
nearly 100% in differentiating
lymphedema from other causes
Continue....
Assess lymphatic function by quantitating the rate of clearance of a
radiolabelled macromolecular tracer.
Advantages :Simple, safe and reproducible, with small exposure to
radioactivity(approx 5mCi).
Method: Injection of a small amount of radio-iodinated human albumin or
Tc 99m- labelled sulfur colloid in to the first interdigital space of foot or
hand.
Migration of radiotracer with in the skin and subcutaneous lymphatics
monitored with wholebody gamma camera
Images of major lymphatic channels in the leg + amount of radioactivity at
the inguinal lymph nodes after 30 and 60min of injection
An uptake of <0.3% of total injected dose at 30min+/- dermal backflow –
Lymphedema.
Normal range of uptake is b/n 0.6 - 1.6%
Edema secondary to venous disease – isotope clearance is abnormally
rapid---> >2% ilioinguinal uptake.
Variations in the degree of edema doesn’t significantly change the rate of
clearance of isotope.
Direct contrast lymphangiography provides finest details of lymphatic
anatomy
Contrast Lymphangiography
Invasive method
Exposure and cannulation of superficial lymph vessel at dorsum of forefoot
Slow injection of contrast medium (ethiodized oil)
Procedure is tedious,cannulation often requires aid of operating
microscope and dissection requires anesthesia
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A total of 7-10ml of contrast is ideal for lower extremity
4-5ml – upper extremity
Complications: Damage of visualised lymphatics, allergic reactions,
pulmonary embolism if oil based contrast agents enters venous system
through lymphovenous anastamoses.
Infrequently used, reserved for selected patients, candidates for direct
operation on their lymph vessels.
New diagnostic test
Contrast magnetic resonance
lymphangiography :
Intracutaneous injection of Gadobenate
dimeglumine
Capable of visualising the anatomy and
functional status of lymph flow, transport in
lymphatic vessels and lymph nodes of
lymphedematous limbs.
Treatment
Non – operative :
General measures
Limb elevation
Compression garments
Decongestive physical therapy
Compression pump therapy
Operative :
General therapeutic measures
Patient education about skin care & avoidance of injuries
Signs of infection - treated aggressively with antibiotics directed at gram
positive cocci.
Eczema at the level of forefoot and toes – Hydrocortisone-based creams
Basic range of motion exercises for all limbs
Maintain ideal body weight
Elevation & Compression garments
All stages of disease – high quality elastic garments, all the times except when
the legs are elevated above the heart
Ideal compression garment – Custom fitted and delivers pressure in range of 30-
60 mm Hg
Additional benefit – protection from injuries and insect bites
Avoid standing for prolonged periods
Overnight elevation of legs supporting the foot of bed on 15-cms blocks.
Complex decongestive lymph therapy
Intensive treatment Self – management
Manual lymph drainage Skin care
Skin care Manual lymph drainage as
needed
Bandaging
Day time compression garments ,
Exercise
night time bandaging
Compression garments
Exercise
Support groups
Manual Lymph drainage
Bandaging
Compression pump therapy
Intermittent , sequential compression devices
Reduced edema, by decreasing capillary filtration
Effective in Venous insufficiency , dependent edemas , lympho-venous
stasis, palliative care
Drug therapy
Benzopyrones – Coumarin reduces lymphedema by
Stimulation of proteolysis by tissue macrophages
Stimulation of peristalsis
Pumping action of collecting lymphatics
First randomised crossover trial of Coumarin in patients with
lymphedema of arms and legs was reported in 1993.
Side effects : liver toxicity.
Drug therapy
Antifilarial medications :
Broncroftian filariasis : DEC 6mg/Kg/day orally for 12 days
Brugian filariasis : DEC 3-6mg/kg/day for 12 days
Given in divided doses after meals
Single dose of Albendazole 400mg
Single dose of Ivermectin 150mcg/kg
Molecular Lymphangiogenesis
Concept involves the factors responsible for lymphatic system development
Activation of VEGFR-3 pathway by administration of cognate ligands VEGF-C
and VEGF-D using various methods.
Tested in animal models with promising results
Former clinical trials are needed to evaluate the therapeutic potential
Possibility of stimulation of dormant tumor cells as a consequence of increased
angiogenesis of therapeutic lymphangiogenesis.
Operative treatment
Indications :
Stage II & III patients with severe functional impairement,recurrent
lymphangitis and severe pain and edema despite CDT.
Two categories of operations :
Reconstructive
Excisional
Reconstructive methods
Considered when proximal obstruction noted with preserved and dilated,
lymphatics peripheral to the obstruction.
Residual dilated lymphatics are anastamosed to near by veins or to
transposed healthy lymphatic channels (mobilised/harvested)
Most commonly done in patients with
Upper extremity lymphedema secondary to axillary lymphadenectomy
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Leg lymphedema secondary to inguinal or pelvic lymphadenectomy
Objective improvement in 30-80% of patients with an average initial
reduction in excess limb volume of 30%-84%.
Primary lymphedema - not considered
b/o hypoplastic and fibrotic distal lymph vessels
Transfer of lymphatic bearing tissue ( portion of greater omentum/segment
of ileum)
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Intention : To connect residual hypoplastic lymphatic channels to competent
lymphatics in the transferred tissue.
Omental flap – poor results
Segment of ileum with its mucosa stripped, mobilised and sewn on to the cut
surface of residual ilioinguinal lymph nodes
To bridge the lower extremity lymphatics with mesenteric lymphatics
Out of 8 selected patients,6 showed sustained clinical improvement
Excisional operations
Considered in patients without residual lymphatics of adequate size
Recalcitrant stage II &III patients with moderate edema and relatively
healthy skin
Excisional operation involves removal of large segment of lymphadematous
subcutaneous tissue and overlying skin.
Palliative procedure, introduced by Kontoleonin 1918
Popularised by Homan as "Staged subcutaneous excision underneath
flaps".
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Procedure : Medial incision from the level of medial malleolus through the
calf in to the midthigh.
Flaps of 1-2 cms thickness are elevated anteriorly and posteriorly
All subcutaneous tissue beneath the flaps + underlying medial calf deep
fascia + redundant skin excised
Sural nerve is preserved
Second stage Operation
Second stage operation – after 3-6 months
Done by lateral incision
Recent long term follow up study – 80% of patients have significant and
long lasting reduction in extremity size with improved function and extremity
contour.
Wound complications – 10% of patients
Alternate surgical procedures
Minimally invasive version of Kontoleon
Liposuction through small incision
Safe, on short term basis for advanced stage
Surgeons with experience recommends initial conservative treatment of
pitting edema f/b liposuction
Charles Operation
Pronounced lymphedema + infected and unhealthy skin
Classical excisional operation - 1912
Procedure : Complete and circumferential excision of skin, subcutaneous
tissue and deep fascia of involved leg and dorsum of foot.
Single stage operation and coverage is provided by full thickness skin
grafting from the excised skin.
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Follow up :
Immediate reduction in circumference and volume
Skin graft take was 88%
Complications : wound infection,hematomas and necrosis of skin flaps,10-
15% of segmented grafts donot take,localised sloughing,scarring and focal
recurrent infections,hyperkeratosis,dermatitis
Hospital stay – 21-36 days
Complications were worse in patients in whom leg resurfacing was
performed with split thickness skin grafts from opposite extremity
Advanced cases : Exophytic changes within the grafted skin,chronic
cellulitis and skin breakdown ---> leg amputation
References
Sabiston Text book of Surgery
Lymphedema , Diagnosis and treatment ,Collection of journals , Springer
publication
Thank you
