Lymphatic disorder

1. LYMPHATIC SYSTEM

2. SURGICAL ANATOMY
• Primordial lymphatic system begins to develop
from 6th
week of development.
• Peripheral lymphatic system develops from
these lymph sacs.
• Lymph sac has three components-
-Terminal lymphatic capillaries
-Lymphatic vessels
-Lymph nodes
Lymph vessels run adjacent to main blood vessels.

3. SURGICAL ANATOMY CONTD..
• Cisterna chyli is formed in the abdomen,
continues as thoracic duct in the thorax.
• Thoracic duct has got initial main course
towards the rt side of mediastinum but later
towards left side entering IJV at its joining point
with lt SC vein.
• Lymphovenous communication occurs at LN
level of Iliac, Subclavian and Jugular levels.
• Lymphatics are absent in epidermis, cornea,
CNS, cartilage, tendons and muscles.

4. SURGICAL ANATOMY CONTD..
• Great LYMPH DUCTS are-
1. THORACIC DUCT
2. RIGHT LYMPHATIC DUCT
3. SUBCLAVIAL AND JUGULAR TRUNKS
4. BRONCHOMEDIASTINAL TRUNKS
THESE CONTAIN VALVES TO PREVENT
BACKFLOW.

5. CISTERNA CHYLI
• Formed by joining of rt and lt lumbar
sympathetic trunks and intestinal lymphatic
ducts.
• Lumbar LT are short lymph vessels arising from
paraaortic lymph glands and recieves lymph from
lower limb, pelvis and pelvic viscera, kidney,
adrenals and deep lymphatics of abdominal wall.
• Intestinal lymphatic ducts arises from preaortic
nodes and recieves lymph from stomach,
intestines, liver, spleen and pancreas

6. CISTERNA CHYLI CONTD…
• CC is a lymph sac lying in front of L1 & L2
vertebrae between aorta and crus of the
diaphragm. From its upper end it continues as
thoracic duct in the chest.

7. THORACIC DUCT
• Passes through aortic crus of the diaphragm.
• From there it runs medial to azygos vn and rt of the
aorta in the post mediastinum.
• Related in front to esophagus, diaphragm and
pericardium.
• Behind it is related to IC arteries, hemiazygos and
accessory hemiazygos vn.
• Crosses towards lt side at level of T7 and reaches lt side
at level of T5.
• In the neck it passes in front of vertebral vessels and
sympathetic chain and behind carotid system.

8. THORACIC DUCT CONTD..
• It ends as a single vessel at the junction of IJV and SCV
with a valve.
• Single termination is common.
• 45 cm in length and 5 mm in width maximum being at the
ends.
• Tributaries include-
1. Trunk from post mediastinal nodes
2. Lateral IC nodes of upper six spaces
3. Lt jugular lymph trunk
4. From lt upper limb
5. Lt bronchomediastinal trunk.

10. RIGHT LYMPH DUCT
• About 2.5 cm in length
• Formed by rt jugular, rt subclavian and rt
bronchomediastinal trunks
• Joins at junction of rt IJV and SCV.
• About 450-600 LN in the body.
-200 in neck
-100 in thorax
-50-60 in axilla
-250 in abd & pelvis
-50 in groin

11. LYMPHATIC WATERSHEDS OF SKIN

12. FUNCTIONS OF LYMPHATICS
• FILTRATION
• ABSORBTION
• Cholesterol, long chain fatty acids& fat soluble
vitamins are TRANSFERRED through
lymphatics into cisterna chyli
• IMMUNITY

13. INVESTIGATIONS
•LYMPHANGIOGRAPHY
•ISOTOPE
LYMPHOSCINTIGRAPHY

14. LYMPHANGIOGRAPHY
• PATENT BLUE DYE OR 1 ML OF ISOSULFAN BLUE
IS USED.
• Radiographs are taken to visualize both
lymphatic and LN
• Secondaries in LN cause filling defect where as
lymphoma shows enlarged nodes which have
foamy appearance.
• Not performed these days as it is technically
difficult, invasive , time consuming and may not
reach the desired area.

15. ISOTOPE LYMPHOSCINTIGRAPHY
• Radioactive Tc labelled sulphide colloid particles
or radioiodinated human albumin is injected into
web space which are specifically taken up by
lymphatics.
• Gamma cameras are used to visualize lymphatics
and inguinal LN.
• Radioactivity of inguinal LN is measured at 30 &
60 min
• In 3 hrs it reaches paraaortic LN, other
abdominal LN and liver.

16. ISOTOPE LYMPHOSCINTIGRAPHY
• More sensitive
• Technically easier and faster
• Simple & safe
• High sensitivity and specificity
• Thoracic duct, other LN and liver can be
imaged.

17. LYMPHEDEMA
• Accumulation of lymph in extracellular &
extravascular fluid compartment commonly in
the sub cutaneous tissue.
• Primarily due to defective lymphatic drainage
• KINMOTH classified lymphedema as-
1. Primary
2. Secondary

18. PRIMARY LYMPHEDEMA
• Commonly affects females.
• Common in lower limbs and on left side
• It can be-
1. Familial
2. Syndromic (Turner’s, Down’s, Kleinfelter’s)

19. PRIMARY LYMPHEDEMA CONTD..
• It can be classified as –
1. Lymphedema Congenital
2. Lymphedema Praecox
3. Lymphedema Tarda

20. LYMPHEDEMA CONGENITAL
• Accounts for about 10% of cases
• Present at birth to less than 2 yrs of age
• Familial type is called as Nonne Milroy’s
disease
• Type 1 familial AD disorder related to
chromosome 5
• 1:6000 of live births
• B/L upper and lower limbs, genitalia and face
may be involved.

21. LYMPHEDEMA PRAECOX
• Usually present at puberty
• Familial type is called as Letessier
Meige’s Syndrome
• Type II familial AD
• Occurs between puberty and middle
age

22. • Lymphedema tarda presents in
middle life after 35 yrs of age.
• Radiologically it can be classified
as-
1. Hypoplasia (70%)
2. Aplasia (15%)
3. Hyperplasia (15%).

23. PATHOPHYSIOLOGY
Decreased lymphatic contractility, valvular insufficiency
and lymphatic obliteration
Lymphatic hypertension and dilatation
Accumulation of proteins, GF, glycosamines
Increased collagen formation
Deposition of proteins, fibroblasts- fibrosis
Involves s/c tissue outside deep fascia
v

24. SECONDARY LYPHEDEMA
• It can be due to-
1.Trauma
2.Surgery
3.Filariasis
4.TB/ Syphilis
5.Fungal infections
6.Advanced malignancy
7.Post radiotherapy
8.Rare causes like RA, Snake bites, DVT, CVI
Sec. lymphedema develops rapidly

25. FILARIASIS
• Caused by W. bancrofti
• It was also called as Malabar leg.
• Shows nocturnal periodicity as it is related to night biting
habits of the vector and sleeping habits of the host.
• Human being is the definitive host & female mosquito is the
int. host
• Development or multiplication of microfilaria never occurs in
human blood
• Life span of microfilaria in human blood is 3 mths and it is
infective to female mosquito
• A density of approx 15 microfilariae/ drop of blood are
needed to make it infective.

26. PATHOLOGY
Recurrent lymphangitis
Dermal lymphatics backflow
Retrograde obliteration
Recurrent Cellulitis
Accumulation of protein, GF,
Glycosaminoglycans

27. PATHOLOGY…
Activation of Collagen & keratinocytes
Protein rich lymphedematous tissue
formation
Sub dermal fibrosis
Dermal thickening and dermal proliferation
Cracks- ulceration abscess formation

28. EFFECTS OF W. bancrofti INFECTION
• Carrier stage
• Immune and allergic reactions
• Filarial fever, urticaria, pruritus, epididymoorchitis as
acute presentations
• Occult filariasis
• Lymphadenitis & lymphangitis
• Lymphorrhagia, lymph scrotum, lymphocele, chyluria,
chylous diarrhea, RP lymphangitis, chylous ascites,
chylothorax
• Disease in the limb is confined to skin and s/c tissue as
only superficial lymphatics are involved.

29. SITES OF LYMPHEDEMA
• Lower limbs- M/C
• Upper limbs
• Scrotum and penis [Ram’s Horn
Penis]
• Breast
• Labia and eyelids

30. CLINICAL FEATURES
• Foot swelling extending progressively in the leg- Tree Trunk
Pattern
• Buffalo hump on dorsum of foot
• Squaring of toes
• +ve Stemmer’s sign
• Edema- initially pitting
• Pain of varying intensity
• Debility/ immobility
• Eczema, fissuring, papillae formation, ulceration,
lymphorrhea, elephantiasis
• Recurrent lymphangitis, cellulitis, abscess formation
• Pathological and social discomfort causing severe morbidity

33. BURNER’S GRADING
• Grade I- pitting edema which more or less disappears
on elevation
• Grade II- non pitting edema which does not reduce
on limb elevation
• Grade III- non pitting edema with skin changes
• Lymphedema can again be classified as-
1. Mild- < 20% of excess limb value
2. Moderate- 20-40%
3. Severe- > 40%

34. COMPLICATIONS
• Skin thickening, abscess and maggot’s
formation
• Recurrent cellulitis, non healing ulcers,
septicemia
• Recurrent Streptococcal infection
• Lymphangiosarcoma [Stewart Treves
syndrome]- biopsy to confirm- chemo or
radiotherapy, later even amputation may be
required.
• Usually seen after mastectomy