Chapter 168 - Lymphedema introduction Flashcards
Two types of lymphedema and causes
High-input failure = venous edema exceeding maximum transport capacity of lymphatic conduit
low-output failure = compromised lymphatic flow
Consequence of lymph stasis
protein and cellular metabolite build up in extracellular space –> osmotic pressure increas e–> increase hydraulic pressure
Accumulation of hyaluronan and glycoprotein in extracellular space –> increase fibroblast, keratinocyte and adipocyte –> collagen deposition
Clinical classification of lymphedema
Primary lymphedema
Secondary lymphedema
Primary lymphedema subclassification
Genetic: familial vs sporadic
Onset: congenital (at birth), praecox (at puberty), tarda (after age 35)
Epidemiology of primary lymphedema
1) female 2-10x more often
2) incidence peak age 12-16
3) 1/6000-10000 livebirth
Chance of bilateral lymphedema
25%
Congenital lymphedema type of transmission
Autosomal dominant
with sporadic penetrance
Association of congenital lymphedema with genetic chromosomal abnormalities and syndromes
1) Turners
2) Klinefelters
3) trisomy 21
4) Noonan’s
5) neurofibromatosis
6) yellow nail syndrome
7) intestinal lymphagiectasia
8) lymphagiomatosis (generalize lymphatic anomaly
9) AVM
Symptoms of congenital lymphedema
Unilateral or bilatera leg edema
Swelling of genitalia, face
Lymphedema Praecox frequency of all primary lymphedema
94%
most common form
Meige’s disease
specific familial form of lymphedema praecox
Gender epidemiology of lymphedema praecox
10:1 more female
Clinical presentation of lymphedema praecox
unilateral limited to foot and calf in most patients
Lymphedema tarda frequency of all primary lymphedema
10%
after age 35
Lymphedema classification by morphology
Aplasia: no collecting vessel
Hypoplasia: less number of vessels
Numerical hyperplasia: increase number of vessels only
Hyperplasia: increase number but also tortuous and dilated - megalymphatic and lymphagiectasia
Hyperplasia patterns
more in male
less common than aplasia/hypoplasia
worse prognosis
can also have angioma and chylous reflux
usually unilateral
Ways to classify primary lymphedema
1) time of onset
2) morphology of lymphatic
3) anatomical location of defect
4) clinical setting
Primary lymphedema by anatomy
Distal obstruction (1/3) = agenesis/hypoplasia/obstruction of distal lymphatic with normal proximal vessels
- bilateral and mild swelling
- more in female
- extent of disease usually maxed in first year but can still grow in size
Proximal obstruction (1/2): obstruction of proximal lymphatic/nodes only
- intranodal fibrosis
- unilateral, severe and worsens with time
- more in females if unilateral
- more in male if bilateral hyperplasia