Download MBBS Diseases of lymphatic system Lecture PPT

Diseases of lymphatic
system

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Lymphatic system
Lymphatics commences from tissue

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spaces and empty into veins
Lymph nodes
Epitheliolymphoid tissues ? walls of GIT ,
thymus spleen
Function ? return of protein rich fluid into

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circulation
? Includes albumin, globulin, coagulation factors,
growth factors , cholesterol, fat soluble vitamins
etc

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Development
Four cystic spaces .. Neck and groin
Cisterns

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Lower limb abdomen ? through cisterna
chyli ? thoracic duct - left jugular vein
Head and neck to right
Lymphatics follow vein
No lymphatics in brain and bone cortex

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Lymphatic system includes lymphatic
channels, lymphoid organs and
circulating elements

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Starlings law plays a role
Lymph flows against a pressure gradient
? Helped by muscle contractions
? Contactions and relaxations of lymphangions
? Valves

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Diseases
Acute lymphangitis

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? Staph/streptococcus
? Lymphadenitis/abscess
? Fever pain etc
? Red streak
? Rest, elevation, IV antibiotics

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? Incision and drainage
? Recurrences --- acute inflammatory episode

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Chronic lymphangitis

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? Repeated attacks of acute lymphangitis
? May be a cause for lymphoedema

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Neoplasms
Benign neoplasms
? From birth
? Neck, axilla, shoulder, groin
? Localised clustures of dilated lymph sacs not

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connected to lymphatic system ?
lymphangiomas
? Three types ? capillary/simple, cavernous,
cystic hygroma

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Simple / capillary lymphangioma
lymphangioma circumscriptum
Dilated dermal lymphatics that blister on

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to skin
Contains clear fluid or blood stained
Inner thigh, shoulder or axilla
Lymphangiography diagnostic ?
separate from main lymphatic trunk

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Treatment excision ? after confirming the
dignosis

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CAVERNOUS
(DIFFUSE)LYMPHANGIOMA
Bigger swelling

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Face, mouth, lips, tongue
Intramuscular sometimes
Very difficult to remove
recurrence

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Cystic hygroma
Most common
75% neck, 20% axilla, 5% in mediastinum,

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pelvis, groin, retroperitoneum sometimes
combinations
Children from birth
Painless, infection ?produce pain
Complete surgical excision

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Sclerotherapy ?
Recurrence

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Malignant neoplasm
Lymphangiosarcoma
? Rare
? Long standing lymphoedema
? Post mastectomy

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? Upper limb common
? Bluish/purple discoloration- nodule- ulcer ?
diffuse
? Treatment ineffective ? fatal

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Lymphoedema
? defined as abnormal limb swelling caused
by the accumulation of increased amounts of
high protein ISF secondary to defective

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lymphatic drainage in the presence of
(near) normal net capillary filtration.

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1 in 6000 births
Physical , emotional , psychological

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Unilateral lymphoedema

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? Mild - < 20% limb volume
? Moderate ? 20 ? 40%
? Severe - > 40%
Pathophysiology of edema
? Capillary filtration abnormality

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? Lymphahtic abnormality ( true lymphoedema)
? Both

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Classification
Primary
? Cause unknown
? Congenital lymphatic aplasia/hypoplasia
Secondary

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? Definite cause

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Risk factors

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Primary lymphoedema
Familial or sporadic

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? Familial ? milroy's disease and Meige's disease
Age at onset
? Congenita ? 10% by birth
? Praecox ? 75% adolescence
? Tarda ? beyond 35 yrs ? 15%

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There is some developmental anomaly
Unilateral in 50%
Family history 1/5th cases

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Lymphangiographically three types
? Aplasia ? 15% , congenital type
? Hypoplasia ? solitary lymph channel- 75%
? Varicose/dilated lymph trunks in 12%-
associated AV malformations

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Prognosis bad for aplasia and varicose
type

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Secondary lymphoedema
Most common

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Infection/inflammation/trauma/tumor
Filariasis commonest cause
Certain conditions can mimic
lymphodema like ? factitious, immobility,
lipoedema

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Investigations
Routine
Lymphangiography

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? Direct
? Indirect
Isotope lymphoscintigraphy
CT scan ? Mass lesions
MRI ? LN

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USS ? Venous system /DVT

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Management
Initial evaluation

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Relief of pain
Control of swelling
? Bedrest, elevation, compression, bandaging,

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exercise
? Manual lymphatic drainage (MLD)
? Multilayer lymphoedema bandaging (MLLB)
Skin care

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Treat acute infective episodes
Exercises
Drugs

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? Flavanoids / benzpyrones
? Diuretics ?
Surgery

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Surgeries
Rare
Minimal benefit
Three types

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Bypass procedures
? Gille's ? Omental/skin bridge
? Kinmonth ? ileal mucosal patch
? Neibulowitz ? LN to vein
? Lymphaticovenular Anastomosis (LVA)

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Liposuction
Limb reduction procedures
? Sistrunk ? wedge excision and suturing

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? Homans
? Thompson's
? Charle's

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