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LYMPHATIC DISORDERS
DR.NADIR MEHMOOD
PROFESSOR OF SURGERY, RMU.
S.U.1, BBH, RAWALPINDI
Learning Objectives
1. Describe the structure and function of lymphatic
cells, tissues and organs
2. Define and classify lymphedema
3. Describe the sign and symptoms of lymphatic
diseases
4. Enumerate the type of lymphatic obstruction
5. Enumerate the factors leading to lymphedema
6. Enlist the therapeutic options for lymphedema
7. Describe the sec l edema in ca breast
8. Describe diagnostic investigations in a case of
lymphatic obstruction
9. Enlist treatment options for lymphedema
Pretty much ignored, the lymphatic system is not
considered part of the circulatory system per se, but
is included as part of the body’s immune and
defense system.
While it collects and returns excess interstitial fluid,
it is also responsible for the transport of
lymphocytes, macrophages and large molecules.
It may also be the conduit for the dissemination of
neoplasia, as in the metastasis of carcinoma.
ANOTOMY OF THE
LYMPHATICS
Lymphatic system consist of:-
1) Peripheral lymph vessels ( consist of single
layer of attenuated endothelial cells, no
basement membrane)
2) Lymph nodes
3) Major lymphatic trunks
FUNCTION:- primary function is to transport
extracellular fluid from the interstitial space back to
blood stream, act as protective filter against foreign
body invasion
Total lymph return to the venous circulation in 24
hours about 2-4 liters.
 The lymphatic system
 Contains cells, tissues, and organs responsible
for defending the body
 Lymphocytes resist infection and disease by
responding to
 Invading pathogens such as bacteria or viruses
 Abnormal body cells such as cancer cells
 Foreign proteins such as toxins
lymphatic system
The Components of the Lymphatic
System
 Lymph
 Lymphatic vessels
 Lymphoid tissues and organs
 Lymphocytes and supporting phagocytic
cells
The lymphatic system consists
of
 Lymphatic capillaries
 Small lymphatic vessels
 Major lymph-collecting vessels
Lymphatic vessels include
 Superficial and deep lymphatics
 Thoracic duct
 Cisterna chyli
 Right lymphatic duct
Major lymph-collecting vessels
The Relationship between the
Lymphatic Ducts and the Venous
System
Lymphatic Flow
 Lymph ducts
 Right lymphatic duct
 about ½ inch long
 drains lymph from
upper right side of
body (arm & head)
– Thoracic (left) duct
• main collecting duct of
the lymphatic system
• 38-45 cm long
• drains 75% of body
• begins as a dilation
known as the cisterna
chyli located anterior
to lumbar disk #2
Mechanisms of Lymph Flow
 Lymph flows at low pressure and speed
 Moved along by rhythmic contractions of lymphatic
vessels
 stretching of vessels stimulates contraction
 Flow aided by skeletal muscle pump
 Thoracic pump aids flow from abdominal to thoracic
cavity
 Valves prevent backward flow
 Rapidly flowing blood in subclavian veins, draws
lymph into it
 Exercise significantly increases lymphatic return
Lymphedema
 Excessive and persistent accumulation of
extravascular and extracellular fluid and
proteins in tissue spaces
 Caused by a disturbance of the water and
protein balance across the capillary
membrane
 Increased concentration of proteins draws
greater amount of water into interstitial
spaces
 Exceeds transport capacity of the
lymphatic system, leading to lymphedema
Disorders of the Lymphatic System
Leading to Lymphatic Insufficiency
 Primary (Congenital) Malformation
 Infection and Inflammation
 Obstruction or Fibrosis
Trauma, Surgery, Neoplasms
Radiation Therapy
 Surgical Dissection of Lymph Nodes
 Chronic Venous Insufficiency
Clinical Signs and Symptoms of
Lymphedema
 Edema of the dorsum of the foot or hand
 Decreased range of motion, flexibility and
function
 Usually unilateral
 Worse after prolonged dependency
 No discomfort or a dull, heavy sensation; sense
of fullness
Clinical Manifestations of
Lymphatic Disorders
 Lymphedema
 Increased girth and weight of the limb
 Sensory disturbances
 Stiffness and limited range of motion
 Decreased resistance to infection
Types of Lymphedema
 Pitting edema
Short duration edema
Finger indentation of the skin
 Brawny edema
Tissue feels hard upon palpation
Indicates fibrotic changes
 Weeping
Fluid leaks, wound healing is impaired
Occurs mostly in the lower extremities
Examination and Evaluation of
Lymphatic Function
Special Considerations
 History, systems review
 Daily activities and position of limb
 Functional assessment
 Skin integrity
 Girth measurements
 Volume measurements
SECONDARY
LYMPHOEDEMA
This is the most common
form of lymphoedema; there
are several well- recognized
causes of secondary lymph
edema.
Known Causes of Lymphoedema
1) Bacterial infection.
2) Parasitic infestation (Filariasis).
3) Fungal infection (tinea pedis).
4) Exposure to foreign body material (Silica
Particles).
5) Primary lymphatic malignancy.
6) Metastatic spread to lymph nodes.
7) Surgical excision of lymph nodes.
8) Trauma (Particularly degloving injuries).
9) Superficial thrombophlebetis (acute Cellulites).
10) Deep venous thrombosis.
11) Factitious lymphoedema- tourniquet or
hysterical misuse.
Pathology (PATHOPHYSIOLOGY)
Due to any cause  out flow due to
sclerosis or obliterations of lymph
channel  lymphatic hypertension 
decrease in contractility valvular
impairment lymph stasis accumulation of
fluid, proteins, GF, and other active
peptides including bacteria, production of
fibroblasts and accumulation of
inflammatory cells (macrophages and
lymphocytes) and activation of
keratinocytes.  Proteins rich oedema,
sub dermal fibrosis, dermal thickening
and proliferations.
 HISTORY
Onset of painless swelling of limb.
 SIGNS
Unlike other types of oedema it involves the
foot “Square foot”.
Dorsum of the foot cannot be pinched
(Stammer’s sign). Proximally spread to knee and
it is unusual to climb up to thigh.
• Chronic eczema.
• Fungal infection of skin and nails fissuring,
ulcers (rare).
• Complications:
Lymphangiasareoma (Stewart – Treves
syndrome – “rare”.
CLINICAL PRESENTATION
Clinical grades (Brunners)
of lymphedema
 Subclinical/ latent
 I. Edema pits on pressure and swelling
disappears on rest and elevation
 II. Edema does not pit nor reduce on
elevation
 III. Edema with irreversible skin changes-
fibrosis and papillae
Secondary Lymph oedema
after radical mastectomy
INVESTIGATIONS:
 Routine Tests:
Blood C/P, urinalysis, urea and
electrolytes LFT, CXR, midnight
blood smear for micro filarial.
 Contrast Lymphangiography
(Now reserves for preoperative
investigations for lymphvenous
bypass)
Isotope Lymphoscintigraphy
Computerized Tomography
MRI
MANAGEMENT OF
LYMPHOEDEMA
 Physical Methods
 Elevation.
 Compression therapy by stockings(GCS).
 Intermittent pneumatic compression.
 Drugs
 Exercise & Weight reduction.
 Antibiotics Penicillin’s / Erythrocin,
Cotrimazole/ Grisflovin
Components of a Decongestive
Lymphatic Therapy Program
 Elevation
 Manual lymphatic drainage
 Compression
 Exercise
 Skin care
 Daily living precautions
Elevation
 Elevate the involved limb when using a
sequential compression pump
 Elevate limb when sleeping, resting, and
during sedentary activities
 Compressive bandages or garment should
be worn during periods of elevation
Manual Lymphatic Drainage
 Slow, very light, repetitive stroking and circular
massage movements performed in a specific
sequence, limb elevated whenever possible
 Proximal congestion in the trunk, groin, buttock,
or axilla is cleared first
 Direction of massage is towards specific lymph
nodes
 Usually involves distal to proximal stroking
Manual
Lymphatic
Drainage
Manual Lymphatic Drainage
 Labor and time intensive
 Specialized training needed
Continuing education
Certification
Manual Lymphatic Drainage
Manual Lymphatic Drainage
Exercise
 Active range of motion, stretching, and
low-intensity resistance exercise is
incorporated with manual drainage
techniques
 Exercises should be performed with
compressive bandages or garment
Exercise
 Exercises are performed in a specific
sequence, often with the limb elevated
 Low-intensity cardiovascular/pulmonary
endurance activities included
 Deep breathing and relaxation also
incorporated
Exercise
 Exercises performed in a specific sequence
can assist lymph flow
 Awareness of other medical conditions
 Patient education
 Please read pages 842 through 847 in
Kisner and Colby for further information
on exercise precautions and guidelines
Exercise
Compression
 No-stretch, non-elastic or low-stretch elastic
bandages are used
 Sports bandages, such as ACE(All Cotton Elastic)
wraps, are NOT recommended in the treatment
of lymphedema
 Compressive garments are available
 Use of a sequential, pneumatic compression
pump on a daily basis may be recommended
Compressive Bandages
Bandages
Compression Garments
Sequential, Pneumatic Pumps
Intermittent Compression
Devices
Contraindications
 Deep vein thrombosis
 Local superficial infection
 Congestive heart failure
 Acute pulmonary edema
 Displaced or acute fractures
Skin Care and Hygiene
 Lymphedema increases risk of skin
breakdown, infection, and delayed wound
healing
 Proper skin care
 Inspection
 Protection
SKIN CARE
 PROTECT HANDS
 AVOID BARE FOOT WALK
 USE ELECTRIC RAZOR TO DEPILATE
 AVOID SKIN MACERATION
 ANTI MOSQUITO PRECAUTIONS
 SEEK MED TREAT EARLY IN CASE OF
REDNESS, SWELLING, PAIN ETC
 AVOID TAKING BLOOD SAMPLES FROM
AFFECTED LIMB
 USE OF SUNBLOCK WHEN NEEDED
SURGERY
 BYPASS Procedures.
 Limb REDUCTION Procedures.
 Sis trunk Operation
(Skin + S/C)  PRIMARY Closure
 Homan Operation
Skin flaps are elevated and subcutaneous
tissue is excised.
 Thompson Operation: - buried dermal flap.
 Charles Operation: - all skin /S/C tissue is
excised to deep fascia with coverage SSG.
CYSTIC HYGROMA
 Abnormal lymph – filled, often
multilocular, soft, brilliantly
transluminable, located at the base
of the neck, it is also found in head
and inguinal region.
 Clinically behaves like benign
tumor and grow gradually in size.
 Leading to cosmetic problem and
compression of surrounding
organs.
CYSTIC HYGROMA
 MANAGEMENT
 Simple aspiration.
 Sclerotherapy.
 Surgical Excision (may present a
problem because of its extension to the
surrounding structures).
Primary chylous disorders
 Essential of diagnosis
Protein rich fatty lymph accumulation in a
body cavity
High specific gravity of the chylous
Incompetent lymphatic valves
Dilatation of lymphatic
Chylorrhea from lymph filled vesicles in
lower extremity or perineum
Severe metabolic and
nutritional derangement
Immunosupression
Chylous ascites and
chylothorax
Diagnosis:-
Lymphangiography
CT scan
MRI
Prevention of Lymphedema
Prevention of Lymphedema should be a
priority of patient management!!!!!
Summary
The lymphatic system helps maintain homeostasis
of fluids, and also helps remove antigen from
the body
The immune system consists of barriers (physical
and chemical) and specific and nonspecific
mechanisms to eliminate antigen
“Immune cells” are blood cells. Some circulate in
the blood and can then migrate into tissues
at site of injury. These include neutrophils
and macrophages.
Any Questions???
THANK YOU

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Understanding Lymphatic Disorders and Lymphedema

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  • 2. LYMPHATIC DISORDERS DR.NADIR MEHMOOD PROFESSOR OF SURGERY, RMU. S.U.1, BBH, RAWALPINDI
  • 3. Learning Objectives 1. Describe the structure and function of lymphatic cells, tissues and organs 2. Define and classify lymphedema 3. Describe the sign and symptoms of lymphatic diseases 4. Enumerate the type of lymphatic obstruction 5. Enumerate the factors leading to lymphedema 6. Enlist the therapeutic options for lymphedema 7. Describe the sec l edema in ca breast 8. Describe diagnostic investigations in a case of lymphatic obstruction 9. Enlist treatment options for lymphedema
  • 4. Pretty much ignored, the lymphatic system is not considered part of the circulatory system per se, but is included as part of the body’s immune and defense system. While it collects and returns excess interstitial fluid, it is also responsible for the transport of lymphocytes, macrophages and large molecules. It may also be the conduit for the dissemination of neoplasia, as in the metastasis of carcinoma.
  • 5. ANOTOMY OF THE LYMPHATICS Lymphatic system consist of:- 1) Peripheral lymph vessels ( consist of single layer of attenuated endothelial cells, no basement membrane) 2) Lymph nodes 3) Major lymphatic trunks FUNCTION:- primary function is to transport extracellular fluid from the interstitial space back to blood stream, act as protective filter against foreign body invasion Total lymph return to the venous circulation in 24 hours about 2-4 liters.
  • 6.  The lymphatic system  Contains cells, tissues, and organs responsible for defending the body  Lymphocytes resist infection and disease by responding to  Invading pathogens such as bacteria or viruses  Abnormal body cells such as cancer cells  Foreign proteins such as toxins lymphatic system
  • 7. The Components of the Lymphatic System
  • 8.  Lymph  Lymphatic vessels  Lymphoid tissues and organs  Lymphocytes and supporting phagocytic cells The lymphatic system consists of
  • 9.  Lymphatic capillaries  Small lymphatic vessels  Major lymph-collecting vessels Lymphatic vessels include
  • 10.  Superficial and deep lymphatics  Thoracic duct  Cisterna chyli  Right lymphatic duct Major lymph-collecting vessels
  • 11. The Relationship between the Lymphatic Ducts and the Venous System
  • 12. Lymphatic Flow  Lymph ducts  Right lymphatic duct  about ½ inch long  drains lymph from upper right side of body (arm & head) – Thoracic (left) duct • main collecting duct of the lymphatic system • 38-45 cm long • drains 75% of body • begins as a dilation known as the cisterna chyli located anterior to lumbar disk #2
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  • 14. Mechanisms of Lymph Flow  Lymph flows at low pressure and speed  Moved along by rhythmic contractions of lymphatic vessels  stretching of vessels stimulates contraction  Flow aided by skeletal muscle pump  Thoracic pump aids flow from abdominal to thoracic cavity  Valves prevent backward flow  Rapidly flowing blood in subclavian veins, draws lymph into it  Exercise significantly increases lymphatic return
  • 15. Lymphedema  Excessive and persistent accumulation of extravascular and extracellular fluid and proteins in tissue spaces  Caused by a disturbance of the water and protein balance across the capillary membrane  Increased concentration of proteins draws greater amount of water into interstitial spaces  Exceeds transport capacity of the lymphatic system, leading to lymphedema
  • 16. Disorders of the Lymphatic System Leading to Lymphatic Insufficiency  Primary (Congenital) Malformation  Infection and Inflammation  Obstruction or Fibrosis Trauma, Surgery, Neoplasms Radiation Therapy  Surgical Dissection of Lymph Nodes  Chronic Venous Insufficiency
  • 17. Clinical Signs and Symptoms of Lymphedema  Edema of the dorsum of the foot or hand  Decreased range of motion, flexibility and function  Usually unilateral  Worse after prolonged dependency  No discomfort or a dull, heavy sensation; sense of fullness
  • 18. Clinical Manifestations of Lymphatic Disorders  Lymphedema  Increased girth and weight of the limb  Sensory disturbances  Stiffness and limited range of motion  Decreased resistance to infection
  • 19. Types of Lymphedema  Pitting edema Short duration edema Finger indentation of the skin  Brawny edema Tissue feels hard upon palpation Indicates fibrotic changes  Weeping Fluid leaks, wound healing is impaired Occurs mostly in the lower extremities
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  • 21. Examination and Evaluation of Lymphatic Function Special Considerations  History, systems review  Daily activities and position of limb  Functional assessment  Skin integrity  Girth measurements  Volume measurements
  • 23. This is the most common form of lymphoedema; there are several well- recognized causes of secondary lymph edema.
  • 24. Known Causes of Lymphoedema 1) Bacterial infection. 2) Parasitic infestation (Filariasis). 3) Fungal infection (tinea pedis). 4) Exposure to foreign body material (Silica Particles). 5) Primary lymphatic malignancy. 6) Metastatic spread to lymph nodes. 7) Surgical excision of lymph nodes. 8) Trauma (Particularly degloving injuries). 9) Superficial thrombophlebetis (acute Cellulites). 10) Deep venous thrombosis. 11) Factitious lymphoedema- tourniquet or hysterical misuse.
  • 25. Pathology (PATHOPHYSIOLOGY) Due to any cause  out flow due to sclerosis or obliterations of lymph channel  lymphatic hypertension  decrease in contractility valvular impairment lymph stasis accumulation of fluid, proteins, GF, and other active peptides including bacteria, production of fibroblasts and accumulation of inflammatory cells (macrophages and lymphocytes) and activation of keratinocytes.  Proteins rich oedema, sub dermal fibrosis, dermal thickening and proliferations.
  • 26.  HISTORY Onset of painless swelling of limb.  SIGNS Unlike other types of oedema it involves the foot “Square foot”. Dorsum of the foot cannot be pinched (Stammer’s sign). Proximally spread to knee and it is unusual to climb up to thigh. • Chronic eczema. • Fungal infection of skin and nails fissuring, ulcers (rare). • Complications: Lymphangiasareoma (Stewart – Treves syndrome – “rare”. CLINICAL PRESENTATION
  • 27. Clinical grades (Brunners) of lymphedema  Subclinical/ latent  I. Edema pits on pressure and swelling disappears on rest and elevation  II. Edema does not pit nor reduce on elevation  III. Edema with irreversible skin changes- fibrosis and papillae
  • 28. Secondary Lymph oedema after radical mastectomy
  • 29. INVESTIGATIONS:  Routine Tests: Blood C/P, urinalysis, urea and electrolytes LFT, CXR, midnight blood smear for micro filarial.  Contrast Lymphangiography (Now reserves for preoperative investigations for lymphvenous bypass) Isotope Lymphoscintigraphy Computerized Tomography MRI
  • 31.
  • 32.  Physical Methods  Elevation.  Compression therapy by stockings(GCS).  Intermittent pneumatic compression.  Drugs  Exercise & Weight reduction.  Antibiotics Penicillin’s / Erythrocin, Cotrimazole/ Grisflovin
  • 33. Components of a Decongestive Lymphatic Therapy Program  Elevation  Manual lymphatic drainage  Compression  Exercise  Skin care  Daily living precautions
  • 34. Elevation  Elevate the involved limb when using a sequential compression pump  Elevate limb when sleeping, resting, and during sedentary activities  Compressive bandages or garment should be worn during periods of elevation
  • 35. Manual Lymphatic Drainage  Slow, very light, repetitive stroking and circular massage movements performed in a specific sequence, limb elevated whenever possible  Proximal congestion in the trunk, groin, buttock, or axilla is cleared first  Direction of massage is towards specific lymph nodes  Usually involves distal to proximal stroking
  • 37. Manual Lymphatic Drainage  Labor and time intensive  Specialized training needed Continuing education Certification
  • 40. Exercise  Active range of motion, stretching, and low-intensity resistance exercise is incorporated with manual drainage techniques  Exercises should be performed with compressive bandages or garment
  • 41. Exercise  Exercises are performed in a specific sequence, often with the limb elevated  Low-intensity cardiovascular/pulmonary endurance activities included  Deep breathing and relaxation also incorporated
  • 42. Exercise  Exercises performed in a specific sequence can assist lymph flow  Awareness of other medical conditions  Patient education  Please read pages 842 through 847 in Kisner and Colby for further information on exercise precautions and guidelines
  • 44. Compression  No-stretch, non-elastic or low-stretch elastic bandages are used  Sports bandages, such as ACE(All Cotton Elastic) wraps, are NOT recommended in the treatment of lymphedema  Compressive garments are available  Use of a sequential, pneumatic compression pump on a daily basis may be recommended
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  • 52. Intermittent Compression Devices Contraindications  Deep vein thrombosis  Local superficial infection  Congestive heart failure  Acute pulmonary edema  Displaced or acute fractures
  • 53. Skin Care and Hygiene  Lymphedema increases risk of skin breakdown, infection, and delayed wound healing  Proper skin care  Inspection  Protection
  • 54. SKIN CARE  PROTECT HANDS  AVOID BARE FOOT WALK  USE ELECTRIC RAZOR TO DEPILATE  AVOID SKIN MACERATION  ANTI MOSQUITO PRECAUTIONS  SEEK MED TREAT EARLY IN CASE OF REDNESS, SWELLING, PAIN ETC  AVOID TAKING BLOOD SAMPLES FROM AFFECTED LIMB  USE OF SUNBLOCK WHEN NEEDED
  • 55. SURGERY  BYPASS Procedures.  Limb REDUCTION Procedures.  Sis trunk Operation (Skin + S/C)  PRIMARY Closure  Homan Operation Skin flaps are elevated and subcutaneous tissue is excised.  Thompson Operation: - buried dermal flap.  Charles Operation: - all skin /S/C tissue is excised to deep fascia with coverage SSG.
  • 56. CYSTIC HYGROMA  Abnormal lymph – filled, often multilocular, soft, brilliantly transluminable, located at the base of the neck, it is also found in head and inguinal region.  Clinically behaves like benign tumor and grow gradually in size.  Leading to cosmetic problem and compression of surrounding organs.
  • 58.  MANAGEMENT  Simple aspiration.  Sclerotherapy.  Surgical Excision (may present a problem because of its extension to the surrounding structures).
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  • 72. Primary chylous disorders  Essential of diagnosis Protein rich fatty lymph accumulation in a body cavity High specific gravity of the chylous Incompetent lymphatic valves Dilatation of lymphatic Chylorrhea from lymph filled vesicles in lower extremity or perineum
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  • 74. Severe metabolic and nutritional derangement Immunosupression Chylous ascites and chylothorax Diagnosis:- Lymphangiography CT scan MRI
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  • 76. Prevention of Lymphedema Prevention of Lymphedema should be a priority of patient management!!!!!
  • 77. Summary The lymphatic system helps maintain homeostasis of fluids, and also helps remove antigen from the body The immune system consists of barriers (physical and chemical) and specific and nonspecific mechanisms to eliminate antigen “Immune cells” are blood cells. Some circulate in the blood and can then migrate into tissues at site of injury. These include neutrophils and macrophages.