Chapter 168 - Lymphedema introduction Flashcards

1
Q

Two types of lymphedema and causes

A

High-input failure = venous edema exceeding maximum transport capacity of lymphatic conduit

low-output failure = compromised lymphatic flow

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2
Q

Consequence of lymph stasis

A

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

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3
Q

Clinical classification of lymphedema

A

Primary lymphedema

Secondary lymphedema

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4
Q

Primary lymphedema subclassification

A

Genetic: familial vs sporadic
Onset: congenital (at birth), praecox (at puberty), tarda (after age 35)

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5
Q

Epidemiology of primary lymphedema

A

1) female 2-10x more often
2) incidence peak age 12-16
3) 1/6000-10000 livebirth

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6
Q

Chance of bilateral lymphedema

A

25%

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7
Q

Congenital lymphedema type of transmission

A

Autosomal dominant

with sporadic penetrance

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8
Q

Association of congenital lymphedema with genetic chromosomal abnormalities and syndromes

A

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

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9
Q

Symptoms of congenital lymphedema

A

Unilateral or bilatera leg edema

Swelling of genitalia, face

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10
Q

Lymphedema Praecox frequency of all primary lymphedema

A

94%

most common form

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11
Q

Meige’s disease

A

specific familial form of lymphedema praecox

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12
Q

Gender epidemiology of lymphedema praecox

A

10:1 more female

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13
Q

Clinical presentation of lymphedema praecox

A

unilateral limited to foot and calf in most patients

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14
Q

Lymphedema tarda frequency of all primary lymphedema

A

10%

after age 35

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15
Q

Lymphedema classification by morphology

A

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

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16
Q

Hyperplasia patterns

A

more in male
less common than aplasia/hypoplasia

worse prognosis

can also have angioma and chylous reflux

usually unilateral

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17
Q

Ways to classify primary lymphedema

A

1) time of onset
2) morphology of lymphatic
3) anatomical location of defect
4) clinical setting

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18
Q

Primary lymphedema by anatomy

A

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
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19
Q

First description of inheritable lymphedema

A
Milroy 1892
Milroy disease (when inheritable)
20
Q

Diseases associated with heritable lymphedema

A

1) Lymphedema-cholestasis (Aagenaes’ syndrome) - 15q realted to VEGFR3
2) lymphedema distichiasis - supplement row of eyelashes from meibomian glands - FOXC2 mutation
3) hypotrichosis lymphedema telangiectasia - SOX18 mutation

21
Q

Most common cause of secondary lymphedema

A

Breast cancer 6-30%

22
Q

Most common cause of secondary lymphedema in 3rd world countries

A

Filariasis

23
Q

Filariasis define

A

1) parasite Wuchereria bancrofti (90%), Brugia malayi, Brugia timori
2) transmitted by mosquitoes
3) perilymphatic inflammation and fibrosis, sclerosis of lymph nodes by adult warms
4) worm products cause reactive hyperplasia and dilation of lymphatic collecting channel

24
Q

Diagnosis of filariasis

A

1) eosinophilia

2) microfilariae in peripheral nocturnal blood, urine, lymph

25
Q

Outcome of filariasis

A

Filarial lymphedema –> incapacitating elephantiasis

difficult to treat

26
Q

Other causes of secondary lymphedema

A

1) trauma - burns, wounds
2) pregnancy
3) infection
4) contact dermatitis
5) RA

27
Q

Clinical staging from the Working Group of the 10th International Congress of Lymphology

A

Latent phase: fluid and fibrosis, no apparent edema
Grade I: pitting edema reduced by elevation, no clinical fibrosis
Grade II: non-pitting edema, no improve with elevation, moderate-severe fibrosis
Grade III: irreversible fibrosis, sclerosis, lymphostatic elephantiasis

28
Q

Mesenteric lymphangiectasia symptoms

A

Diarrhea

Weight loss

29
Q

Intermittent drainage of milky fluid from skin vesicles is

A

reflux of chyle

30
Q

Signs and symptoms of lymphedema

A

1) pitting edema
2) square toes
3) positive Stemmer’s sign
4) buffalo hump
5) red skin warm (early) skin
6) thick skin with hyperkeratosis, lichenification, peau d’orange = pigskin (late)
7) recurrent eczematous dermatitis and excoriation
8) verrucae +/- lymphorrhea and chylorrhea
9) yellow nails, clubbing, lack of nail growth
10) aching and heaviness of limb (not intense pain unless infected)

31
Q

Stemmer’s sign

A

positive = inability to pinch base of 2nd toe because of inelasticity

32
Q

Yellow nail syndrome

A

1) yellow nails from lymphedema

2) pleural effusion

33
Q

Infection rate in primary lymphedema

A

31%

34
Q

Reasons why lymphedema more prone to infection

A

1) malnutrition
2) impaired local immune response
3) systemic immunodeficiency
4) ample substrate for bacterial growth
5) infection further exacerbates lymphatic drainage

35
Q

Lymphangiosarcoma

A

1) multicentric lesions
2) blue nodules
3) sclerotic plaques
4) bullous change

36
Q

Cancers that can arise from lymphedema

A

1) lymphangiosarcoma
2) kaposi’s sarcoma
3) SqCC
4) malignant lymphoma
5) melanoma

37
Q

Lymphocintigraphy key points

A

1) radiolabelled macromolecular tracer
2) inject subdermal within interdigit webspace
3) gamma camera to trace

38
Q

Size of particles that get absorbed into lymphatics

A

larger in the 10 nm range like antimony trisulfide Sb2S3

Different size travel at different time
Sb2S3 - 30-60 min
rhenium - 30-60 min
Tc99m albumin - 10 min

39
Q

Parameters of lymphoscintigraphy

A

1) transport kinetics, speed
2) distribution pattern
3) lymph node appearance time
4) lymph node visualizability
5) lymph vessel visualizability
6) abnormal tracer accumulation

40
Q

Limitation of lymphoscintigraphy

A

cannot visualize any detail in the foot (injection site)

cannot distinguish between primary vs secondary lymphedema

41
Q

Signs of lymphedema on lymphoscintigraphy

A

1) dermal backflow
2) absent/delay transport
3) crossover filling with retrograde backflow
4) delay or absent visualization of lymph node

42
Q

Signs of lymphangiectasia on lymphoscintigraphy

A

1) mild or no delay in lymph transport

2) reflux to affected lymphedematous leg can be seen due to valve icnompetence

43
Q

CT and MRI differentiation of lymphedema from venous edema and lipedema

A

Lymphedema: confiend to epifascial space of skin and subcutaneous tissue
Venous edema: also affect subfascial muscles
Lipedema: fat accumulation no fluid

help rule out obstructive mass

44
Q

Direct contrast lymphangiography

A

1) identify subcu lymphatic with subcu methylene blue injection
2) Direct injection of iodine-based, lipid-soluble agent into subcu lymphatics once identified
3) limited to use before reconstructive surgery

45
Q

Lipedema

A

1) Lipodystrophy
2) symmetrical enlargement of lower extremities
3) causes venous insufficiency
4) spares feet
5) cause secondarily cause lymphedema
6) usually morbidly obese
7) normal lymphoscintigraphy and lymphangiography in most cases

46
Q

Workup for lymphedema

A

1) systemic work up to rule out other causes of edema (renal, hepatic, hypoproteinemia, eosinophilia)
2) bioimpedance spectroscopy in cancer patients to assess risk of futher lymphedema
3) Venous duplex to rule out venous incompetence
4) Lymphoscintigraphy

47
Q

Factors that are involved in lymphangiogenesis

A

VEGF-C and VEGF-D bind VEGFR3 on lymphatic endothelium