This document discusses lymphedema, beginning with an introduction defining it as abnormal collection of lymph fluid due to congenital or secondary lymphatic dysfunction. It is a chronic disease often misdiagnosed or untreated. The document then covers lymph vessel anatomy, physiology, primary and secondary lymphedema causes, clinical features including elephantiasis, staging systems, diagnostic tools like lymphoscintigraphy and ICG lymphangiography, and various surgical management techniques for lymphedema including lymph node-venous anastomosis and vascularized lymph node transfer. It concludes with a section on lymphatic filariasis, the most common cause of secondary lymphedema.

1. LYMPHEDEMA
Presenter: Dr Sanjay Natarajan, JR3, General Surgery
Guides: Dr.Ravi HS, Associate Professor, Plastic Surgery
Dr.Harish Kumar P, Assistant Professor, General Surgery

2. INTRODUCTION
• Abnormal collection of interstitial lymph fluid due to
either congenital maldevelopment of the lymphatic
system or to secondary lymphatic dysfunction
• A chronic debilitating disease that is frequently
misdiagnosed, treated too late, or not treated at all

3. ANATOMY
• Lymph vessel begins as a blind ended sac at
the lymphatic capillary below the epidermis or
mucosa, draining into the precollectors
• The Precollectors have a valvular structure
connecting the dermal lymphatics to the
collectors in the fat plane
• The collectors are lined by smooth muscle
and have valves
• Between two valves of the collector is a
functional unit called lymphangion
• Lymphangions are innervated by ANS and
contract rhythmically

4. LYMP
H
FLOW

5. LYMPHATIC PHYSIOLOGY
• Lymph movement occurs despite low pressure
due to smooth muscle action, valves, and
compression during contraction of adjacent
skeletal muscle and arterial pulsation.
• When the pressure in the lymphangion becomes
high enough the lymph fluid will push through
the semilunar valve into the next lymphangion
and the valve closes
• Lymph vessels are similar to blood vessels but
they can function without a central pump due to
the unidirectional flow due to the valves
• Rhythmic contractions of smooth muscle in the
walls of the lymphatic vessels play a major role
in lymph circulation.

6. PRIMARY LYMPHEDEMA:
• Cause is unknown or unproven
• Can be classified by age at onset, morphology, or clinical setting.
• Based on age of onset
1.Congenital lymphoedema(Milroy’s disease) –onset < 2 yrs
old
2.Lymphoedema praecox (Meige’s disease) – onset 2-35 yrs
old
3.Lymphoedema Trada – onset after 35 yrs

7. PATHOLOGY- PRIMARY LYMPHEDEMA
Aplasia – the lymphatic channels have not been developed at all and are
absent. This is usual in Milroy’s Disease.
Hypoplasia – underdeveloped lymphatics with the vessels and lymph
nodes being few in number and/or with a smaller diameter than normal.
Hypoplasia is the most common type of abnormality of the lymphatics in
primary lymphedema.
Hyperplasia– there is an excessive number of lymphatics with impaired
function due to them often being enlarged or distorted (similar to varicose
veins) so that normal lymph flow is hampered.

8. SECONDARY LYMPHEDEMA

9. CLINICAL
FEATURES OF
LOWER LIMB
ELEPHANTIASIS

10. CLINICAL FEATURES
• Edema: Initially the interstitial space is filled with excess protein rich fluid
The edema is soft initially and displaced easily with pressure -pitting edema
When edema extends to distal aspects of feet, “square toes”are formed
When the dorsum of forefoot is involved, “buffalo hump” appearance is seen
In later stages, surrounding tissue becomes indurated and fibrotic
• Skin changes: In early stages, skin is pink and warm – due to increased capillary blood flow
Long standing cases show thickening, hyperkeratosis, lichenification and peau
d’orange appearance
Recurrent chronic eczematous dermatitis may occur
In some patients with chronic lymph stasis, there is hyperplasia of lymphatics and
valvular incompetence leading to formation of small vesicles draining lymph -
lymphorrhea

11. • The other clinical features include:
• Heaviness of limb
• Recurrent lymphangitis
• Fungal infections

12. PHYSICAL
EXAMINATION

13. PHYSICAL
EXAMINATION

14. Prominent involvement of digits with squaring of to
Kaposi Stemmer Sign: Inability to pinch skin
at the base of second toe. It is
pathognomonic of lymphedema

15. PEROMETERY
• Infrared optoelectronic technology is used to
detect changes in limb volume. It uses 360
degrees of infrared light and takes surface
measurements at 0.5 cm increments. Volume
is then calculated from this information.
• The measurement is rapid and precise but
the machine is bulky and expensive making it
difficult for widespread use
• Similar to tape measurements, it is also
limited in its inability to distinguish volume
changes from weight gain versus edematous
changes

16. CIRCUMFERENCE TAPE MEASUREMENT
• Most frequently used technique due to the
low expense and ease of use.
• Circumferential measurements are either
taken at boney landmarks or established
locations along the limb
• Operator dependant as the relative tension
placed on the tape measure may affect the
accuracy.
• It is ideal to perform as many measurements
as is practically possible (in 4–9 cm intervals
along the length of the limb)
• Volume of limb can be calculated using
modified cone equation

17. LYMPHOSCINTIGRAPHY
• Technecium-99 m sulfur colloid, is injected
subdermally into the affected, and usually the
unaffected control, limb.
• Lymphoscintigraphy relies on the lymphatic system’s
ability to transport large radiolabeled protein or
colloid molecules from the interstitial space, through
nodal basins, back to the vascular compartment.
• The radiolabels can be followed using the gamma
camera to detect the radioactivity.
• Provides a poor resolution image
• Time consuming and does not help in surgical
planning
B/L congenital Lower extremity
Lymphedema Tarda

18. ICG LYMPHANGIOGRAPHY
• Indocyanine green (ICG) lymphangiography
involves the injection of a contrast agent
into the interstitial fluid and then
monitoring flow of protein bound dye in
the superficial lymphatic channels below
the dermis.
• This uses near infrared cameras to detect
the fluorescence from protein bound,
excited ICG molecules.
• It allows real time visualization of lymphatic
flow without exposure to radiation
• Used in lymphatic microsurgery to identify
lymph vessels

19. THE INTERNATIONAL SOCIETY
OF LYMPHOLOGY STAGING SYSTEM
Stage 0
Latent lymphedema.
Lymph flow impairment
after injury without
measurable signs of
edema or swelling
Stage 1
Spontaneously
reversible
lymphedema.
Measurable
swelling or edema
that resolves with
elevation or
compressive
Stage 2
Spontaneously
irreversible
lymphedema.
Progression of
edema that does
not fully respond
to conservative
therapies
Stage 3
Lymphostatic
elephantiasis. The
final stage in which
severe irreversible
swelling, fibrosis,
and fatty
deposition result in
thickened, firm

20. CAMPISI STAGING OF LYMPHEDEMA
• Stage IA: No clinical edema despite the presence of lymphatic
dysfunction as demonstrated on lymphoscintigraphy.
• Stage IB: Mild edema that spontaneously regresses with elevation.
• Stage II: Persistent edema that regresses only partially with elevation.
• Stage III: Persistent, progressive edema; recurrent erysipeloid
lymphangitis.
• Stage IV: Fibrotic lymphedema with column limb.

21. MD ANDERSON CANCER CENTER ICG LYMPHANGIOGRAPHY STAGING
I 11 111 1V
• Stage 1: many patent lymphatic vessels,
with minimal, patchy dermal backflow.
• Stage 2: moderate number of patent
lymphatic vessels, with segmental dermal
backflow
• Stage 3: few patent lymphatic vessels, with
extensive dermal backflow involving the
entire arm.
• Stage 4: no patent lymphatic vessels seen,
with severe dermal backflow involving the
entire arm and extending to the dorsum of
the hand

22. SURGICAL MANAGEMENT
• PHYSIOLOGICAL PROCEDURES
• Lymph vessel- Lymph vessel
Anastomosis
• Lymphnode -Venous Anastomosis
• Lymph vessel – Venous Anastomosis
• Vascularized Lymphnode Transfer
• Lymphatic grafting
 EXCISIONAL PROCEDURES
 Sistrunk Procedure
 Charles Procedure
 Homan Procedure
 Thomson Procedure
 Suction Assisted Lipectomy

23. PATIENT SELECTION
• Failure to obtain satisfactory control of lymphedema after an year of
vigourous non surgical treatment is a major criterion for considering surgery
• Patients with commitment to a lifetime of Complex Decongestive Therapy as
the initial results seen in the immediate postoperative period cannot be
maintained without therapy.
• Patients with BMI of more than 30 should not be offered physiological
procedures as obesity leads to impaired lymphatic function.
• Patients with pre-existing venous insufficiency should not be offered
physiologic procedures as they require a low pressure venous outflow
system

24. CHARLES PROCEDURE
• Total excision of all skin and
subcutaneous tissue from the
affected extremity. The underlying
fascia is then grafted, using the skin
that has been excised.
• This technique is extreme and is
reserved for only the most severe
cases.
• Complications include ulceration,
hyperkeratosis, keloid formation,
hyperpigmentation, weeping
dermatitis, and severe cosmetic
deformity

25. THOMSON PROCEDURE –
BURIED DERMIS FLAP
• The procedure combines both physiological and excisional
methods
• Anterior and posterior skin flaps raised medially
• Subcutaneous tissue, deep fascia excised
• Posterior skin flap sutured to the muscles after removing the
epidermis as a split skin graft
• Anterior flap is sutured over the posterior flap
• How does this procedure work:
• Excision of tissue causes reduction in size
• Increased tension promotes lymph drainage
• Diversion of lymphatics from superficial to deep
compartment as deep fascia is excised
• Possible development of anastomosis between superficial
and deep lymphatics

26. SISTRUNK
PROCEDURE
• A wedge of skin and
subcutaneous tissue is
excised and wound is closed
primarily

27. HOMAN PROCEDURE
• Originally introduced by Kontoleon in 1918,
popularized by Homan later
• “Staged subcutaneous excision beneath the flaps”
• In first stage, Flaps are raised anteriorly and
posteriorly along the medial aspect of the lower
limb and the underlying tissue including
subcutaneous fat and fascia are removed upto
the muscle.
• If further excision is required, second stage is
performed after 3-6 months through an incision
over lateral aspect of the limb

28. SUCTION ASSISTED LIPECTOMY
• Common misconception is that lymphedema
involves accumulation of lymph fluid only
• Lymphostasis causes impaired clearance of lipids
which are taken up by macrophages
• Chronic inflammation also causes the deposition
of cytokine mediated adipocyte
• Using Liposuction non pitting upper limb
lymphedema of >4L can be effectively removed
without any compromise in lymph transport
• Liposuction should never be performed in non
pitting edema which is dominated by
accumulated lymph
• Pitting edema must be converted to non pitting
edema using Controlled Compression Therapy
(CCT) first

29. SURGICAL TECHNIQUE: SAL
• Made to measure compression garments
(normal limb as template) must be worn
from 2 weeks before surgery
• Anaesthesia: Tumescent anaesthesia with
infiltration of 1-2 L of saline containing low
dose adrenaline and lignocaine
• Tourniquet application along with
tumescence reduces blood loss
• Using 15-20 incisions of length 3mm,
liposuction is done
• 15cm and 25cm long cannulas with 3mm
and 4mm diameter are used
• Power assisted liposuction with vibrating
cannulas is helpful

30. LYMPH NODE -VENOUS
BYPASS
• Nodovenous shunt was developed by
Nielubowicz in artificial lymphedema
produced in dogs
• Mostly used in cases of lymphatic filariasis
before cytoreductive surgery
• Used in cases where afferent lymphatics
are also destroyed like filariasis, post
traumatic, post inflammatory lymphedema
• It is a physiological procedure

31. SURGICAL TECHNIQUE:
NODOVENOUS SHUNT
• Vertical 3cm incision made medial to the femoral artery,
exposing the GSV
• The distal end is ligated and there should not be any
retrograde flow in proximal segment ( No SFJ incompetence)
• Identify a viable lymph node along vertical group
• No dissection done around lymph node to preserve the
lymphatics
• Shave the upper capsule of the lymph node and observe the
lymph ooze
• The proximal segment of vein is anastomosed to the cut
surface of the capsule of the node using 7-0 Nylon
continuous sutures
• Similarly, end to side anastomosis can be done by making a
stab over the vein

32. LYMPH VESSEL –
VENOUS ANASTOMOSIS
• Introduced by O’Brien (1977)
• Lymphaticovenular anastomosis is a surgical
procedure where lymphatic vessels in a
lymphedematous limb are connected to
nearby small veins and venules using
microsurgical techniques
• It requires anastomosis between healthy
lymphatic vessels and a healthy nearby vein
• In Lower limb, it can be performed at
inguinal or popliteal level
• Contraindicated in venous hypertension

33. SURGICAL TECHNIQUE: LVA
• Site of incision and lymph vessel identification made
out using ICG lymphography
• Local anaesthetic with adrenaline is infiltrated
• Isosulfan Blue or Lymphazurin dye is injected distal
to the incision site which is absorbed by the
lymphatics and helps in delineation
• End to Side anastomosis of the venule to the sub
millimeter lymphatic vessel is done using high
resolution microscope using monofilament 10-0 or
11-0 Prolene
• Patient can be discharged on the same day of
surgery
• At 1 year follow up, upto 61% reduction in limb size
has been reported
End-to-side anastomosis of
1.5 mm diameter venule into
0.6 mm diameter lymphatic
channel

34. VASCULARIZED LYMPH NODE TRANSFER
• Lymph nodes along with their vascular supply from a donor site is harvested and
transferred to the affected extremity as a free tissue transfer.
• A microsurgical anastomosis is performed between the blood vessels of the
lymph node flap and the recipient site vessels
• How does it work?
• Lymphangiogenesis: VEGF C promotes growth and connections between donor
and recipient site lymphatics
• Transplanted lymph node acts as lymph pump
• Soft tissue acts as a bridge between the proximal and distal lymphatics
• Adverse effects: Lymphedema of the donor site

35. VASCULARIZED OMENTAL
FLAP TRANSFER
• Omentum is a highly vascular, lymphatic organ
• It is freely available and does not produce
donor site lymphedema
• As early as 1960 omentum was used as a
pedicled flap based on gastro epiploic vessels
which was tunneled to be used in upper
extremity lymphedema
• After the discovery of free tissue transfer
techniques, omentum is now used as a free
flap
• Omental harvest can be done through
minimally invasive method

36. LYMPHATIC FILARIASIS
• Lymphatic Filarasis is a vector borne disease caused by:
• Wuchereria bancrofti (90%)
• Brugia malayi (10%)
• Brugia timori
• The genera of mosquitoes transmitting the disease:
• Culex
• Anopheles
• Aedes
• Globally, around 120 million people in 83 countries are affected
• Eradicable disease

38. PATHOGENESIS
Filarial worms
enter lymphatics
Vessel wall
dilatation
Inhibits
contractility
Lymph stasis
Repeated
bacterial infection
ADLA Lymphedema Elephantasis

39. CLINICAL FEATURES
• Asymptomatic microfilaremia
• Unilateral or bilateral asymmetric
swelling of limbs
• Acute ADLA attacks – fever, pain
• W. bancrofti - entire affected limb, the
genitals, or breasts.
• B. malayi - legs below the knee and
upper limbs below the elbow, without
any genital or breast involvement.
• Thickening of skin
(MC)

40. DIAGNOSIS
• History taking – evolution of disease
• Clinical examination
• Night blood examination to detect microfilariae
• Immuno-chromatographic-card test (ICT)
• Ultrasonography for locating the adult worms
• (NEGATIVE ONCE LYMPHEDEMA IS ESTABLISHED)

41. DIFFERENTIAL DIAGNOSIS
• Diseases other than filariasis can present with
lymphedema and elephantiasis
• Eg. Primary lymphedema due to congential lymphatic
disorders , secondary lymphedema due to malignancy
• Practically indistinguishable from Lymphatic filariasis
in advanced stage

42. MEDICAL MANAGEMENT- FILARIASIS
• DEC is very effective as a microfilaricidal agent but it kills only around 50% of
adult worms.
• Dose: 6 mg/kg daily for 12 days.
• Single dose of 6 mg/kg is also effective
• Not useful in advanced cases of lymphedema
• For management of ADLA: oral or parenteral antibiotics can be given
according to the culture/sensitivity reports
• In advanced cases, long term antibiotic therapy is indicated to prevent ADLA
and worsening of lymphedema

43. NON SURGICAL MANAGEMENT
• Forms the mainstray of management of lymphedema
• Surgery is reserved only for patients who fail non operative management

44. EDEMA PREVENTIVE MEASURES
Skin hygiene – wash, moisturize
Clothing- avoid synthetics and tight fits
Avoid trauma. Treat wounds immediately
Control fungal infections
Diet- low sodium, high protein
Exercise- aerobic, resistance, stretching
Compressive garment – correct fit

45. MANUAL LYMPHATIC DRAINAGE
• Danish physicians Emil and Estrid Vodder developed manual techniques to aid
and improve lymphatic flow and coined the term “Manual Lymphatic
Drainage”’
• The body is divided into 6 areas corresponding to the drainage of B/L cervical,
axillary and inguinal lymph nodes
• The intact body part adjacent to the affected body part is massaged first. This
redirects the lymph towards functioning lymphatic territories
• Particularly useful in body parts where sustained compression is not possible –
Face
• MLD alone when used separately did not improve the symptoms. It has to be
combined with other modalities like compression, etc

46. The techniques stimulate the
lymph flow from distal to
proximal lymphatics

47. COMPLEX DECONGESTIVE PHYSIOTHERAPY
• Also known as Complete Decongestive Therapy (CDT)
• The four cardinal compoenents of CDT are:
• Skin care – Hygiene measures, anti-fungal, anti-bacterial measures which prevent
attacks of cellulitis
• Manual Lymphatic Drainage
• Compressive bandaging which prevents the reaccumulation of lymph fluid
• Exercises performed while wearing the bandages, enable muscle and joint
pumps to exert their lymphokinetic effects
• CDT is done in two phases:
• Phase I : Intensive phase – mobilizing the lymphatic load and initiate reduction of
fibrosis
• Phase II: Maintenance phase – Optimizing and preserving the achieved success

48. CONTRAINDICATIONS OF CDT
• Acute erysipelas
• Acute thrombophlebitis
• Decompensated heart failure
• Peripheral arterial occlusive disease

49. • CDT must be performed by a certified
lymphedema therapist
• Many patients lead a near normal life
after significant reduction in limb
volume

50. COMPRESSION BANDAGING
• Helps to maintain the tissue hydrostatic pressure
• This compression must be continued until volume reduction stabilizes
and tissues remodel with improved lymphatic capacity
• Following manual lymphatic drainage, specific combination of
padding, foam, gauze and short stretch bandages applied in layers
• Exercise is essential to optimize the efficacy of short stretch bandages
• In the intensive phase, the lymphedema therapist applies the bandages
but in the maintenance phase the patient can learn to apply the
bandages.

51. Bandaging of individual digits is
essential
Soft padding distributes the pressure
evenly
Multilayered inelastic
compression

52. COMPRESSION GARMENTS
• Limits the fluid build up in the limb
• Acts as counterforce to muscle contractions to improve
lymphatic drainage
• Effects enhanced when worn during exercise
• Can be worn in the night while using compression
bandaging in the day time
• Must be custom made for the individual patient

53. INTERMITTENT PNEUMATIC COMPRESSION
• IPC with multichamber pumps effectively
removes excess fluid from extremity
• Can be incorporated into a multidisciplinary
programme
• Reduces edema by decreasing capillary
filtration
• Leads to formation of tissue channels that
provide pathways for clearance of edema
fluid

54. LYMPHEDEMA AND BREAST CANCER
• As a consequence of breast cancer treatment, lymphedema can occur in the
extremity and trunk region
• Avoidance of ALND or SLNB does not prevent lymphedema
• The women are advised to avoid any medical procedures to the ipsilateral ‘’at
risk’’ limb
• Resistance exercise to the at risk limb prevents lymphedema
• Patients who undergo ALND or atleast 5 nodes in the axilla removed need
surveillance beyond immediate postop period
• Early identification of increase in limb volume and management with
compression garments is effective

55. LOWER LIMB LYMPHEDEMA AND CANCER
• Secondary Lower limb lymphedema is associated with surgical excision/
radiation of inguinofemoral lymph nodes
• The incidence varies depending on type of cancer and treatment regimen
• The most common timing of onset is within first year following the treatment
• It is one of the most common issues reported following treatment of
gynaecological cancers – Ovarian, cervical , vulval , endometrial cancers
• With the increase in the incidence of cancers the public health burden of
Lymohedema is expected to increase

56. REFERENCES
• 1) Lymphedema - A Concise Compendium of Theory and Practice
• 2) Rutherford Textbook of Vascular Surgery
• 3) Haimovisci’s Vascular Surgery

57. THANKYOU