Miscellaneous: FMD, other aneurysms, AV malformation Flashcards Preview

PGY 4 > Miscellaneous: FMD, other aneurysms, AV malformation > Flashcards

Flashcards in Miscellaneous: FMD, other aneurysms, AV malformation Deck (57):
1

What vessels does fibromuscular dysplasia affect?

Most medium sized arteries - e.g. internal carotid and renals 

2

What layer of the vessel does fibromuscular dysphasia affect?

Media

3

What is the prevalence of fibromuscular dysphasia?

Unknown

4

Who gets fibromuscular dysphasia? (Age, sex)

Young females

5

What is the theory for pathogenesis of fibromuscular dysplasia?

Ischemia of vasovasorum. Arteries affected are internal carotid, vertebral and renals that have long segments without branch points thus less vasovasora. 

6

Which renal artery is more affected by fibromuscular dysphasia?

Right more than left. Longer, therefore less vasovasorum/prone to vessel wall ischemia. 

7

What is the definition of nutcracker syndrome?

Compression of left renal vein by SMA. Nutcracker syndrome = constellation of symptoms. NC phenomenon if asymptomatic. 

8

What are risk factors of nutcracker syndrome?

Low BMI, young female

9

How can low BMI lead to nutcracker syndrome?

Paraspinal muscle wasting and low mesenteric/retroperitoneal fat means that SMA is at a more acute angle <16 degrees vs normal 35-40 degrees

10

What are the 3 anatomical types of nutcracker syndrome?

Anterior and posterior. Also "atypical" circumaortic renal vein or congenital malformations e.g. left IVC

11

What is posterior nutcracker syndrome

Usually with retroaortic vein, compression of left renal vein against vertebral body

12

What are symptoms of nutcracker syndrome?

Hematuria and left flank pain. Also genital varices, pain with menses/sex/urination, protienuria and abdo pain

13

What is the duplex criteria for nutcracker syndrome?

Flow velocities/diameter proximal and distal to stenosis ratio > 5

14

What is the most common open operation for nutcracker syndrome?

Renal vein transposition onto the IVC more caudally. Gonadal and adrenal veins often have to be ligated. 

A image thumb
15

What are open options for anterior nutcracker syndrome?

Left renal vein transposition +/- patch or saphenous vein cuff. Gonadal vein transposition. Saphenous vein bypass from LRV to IVC. 

16

What open operations can be used for both anterior and posterior nutcracker syndrome?

Gonadal vein transposition, saphenous vein bypass from LRV to IVC

17

What is the rate of migration for renal vein stenting?

7%. Can possibly migrate to right atrium and require open heart surgery!

18

What are the differences between hemangiomas and congenital malformations?

Hemangioma = tumour, grows rapidly, involutes by age 12, originates from endothelial cells

Congenital vascular malformations - inborn error, grow steadily in parallel with childs growth, never disappear or regress

19

What are the 6 types of congenital malformations according to the Hamburg classification?

1-Arterial 

2- Venous

3-Arteriovenous shunting

4-Lymphatic

5-Combined hemolymphatic

6-Capillary

20

What are the congenital vascular malformation embryologic subtypes?

  • Extratruncular: 
    • infiltrating/diffus,
    • limited/localized
  • Truncular:
    • stenosis or obstruction (hypoplasia, aplasia, hyperplastia, membrane congenital spur),
    • dilation (localized = aneurysm, diffuse = ectasia)

21

What abnormality is depicted here?

Q image thumb

Portwine stain - Klippel Trenaunay syndrome. Limb swelling and leg length discrepancy. Hemolymphatic malformation.

22

Who gets congenital vascular malformations more? Men or women?

1:1, M:F

23

What is the most common type of congenital vascular malformation?

Venous

24

What are 5 types of risk factors during pregnancy that are associated with congenital vascular malformations?

1) Exposure to chemicals

2) Infections: rubella, herpes, toxoplasmosis

3) Drugs: thalidomide, anticonvulsants, quinine

4) Rec Drugs: EtOH, Cocaine, smoking

5) Diseases: DM, thyroid disease, TB, hypoxia, lead/carbon monoxide

25

When do extratruncular congenital malformations arrest development?

Early embryonic life while vascular system is in the reticular stage. 

26

What stmulates extratruncular CVMs to grow and proliferate?y, 

Internal stimulation: menarche, pregnancy hormonal release

External: trauma, surgery

 

27

Why do extratruncular CVMs behave like an infiltrating process/invade surrounding structures?

Arrested growth in early embryonic life. Bones, muscle, soft tissue and blood vessels all originate from same mesoderm and no plane of partition between them. 

28

What has a higher rate of recurrence - extratruncular or truncular CVM?

Extratruncular

29

What type of CVMs are associated with more serious hemodynamic consequences? Truncular or extratruncular?

Truncular

30

When do truncular CVMs arise?

Arrested development later, during vascular trunk formation stage when truncular lesions have lost the characteristics of mesenchymal cells. All appear as formed vessels with various degrees of development. 

31

What are the differences between a vascular birthmark and capillary malformations?

1- Colour: CM = port wine, birthmark = pinkish

2- Location: CM = anywhere on body, birthmark = nuchal "stork bite" or face "angel's kiss"

3- CM accompanied by other abnormal findings beneath the skin (e.g. CM of face = warning sign of intracranial CVM Sturge Weber Syndrome with ipsilateral ocular and leptomeningeal vascular malformation)

32

What is Sturge Weber syndrome?

Neurologic syndrome that presents as port wine stain of face. Ipsilateral ocular and leptomeningeal vascular malformation may result in seizures, increased pressure in the eye (glaucoma), developmental delays, and weakness on one side of the body

33

What type of CVM is this? Soft bluish swelling. Easily compressible. Enlarge with dependency/valsalva. Respond to compression therapy.

Venous

34

What type of CVM is this? Ischemic changes, distal gangrene, high output heart failure

Arteriovenous

35

What is the marginal vein?

An embryonic tissue remnant that fails to involute - usually on lower leg and looks like a large dilated lateral vein. Often heralds coexisting deep venous anomalies which should be investigated. 

36

What is the name of the prominent vein on the back of this patients leg?

Q image thumb

Marginal vein (embryonic remnant failed to involute). Parker weber syndrome. 

37

What is transarterial lung perfusion scintigraphy (TLPS)?

A scan with radioisoptope tagged albumin microspheres to measure the percentage of blood shunted by AVMs. Can be used to detect microshunting common in Park weber syndrome

38

Which type of congenital vascular malformations need to be treated aggressively?

Extratruncular. They grow and will recur unless the lesion nidus is completely destroyed

39

Which type of congenital vascular malformations can be handled conservatively unless they become hemodynamically significant?

Truncular lesions. They lack the potential for proliferation. 

40

What are 10 indications to treat extratruncular vascular malformations?

1-Hemorrhage

2-High output heart failure

3-Cosmetically deforming

4-Vascular bone syndrome

5-Near vital structures: airway, sight/eat/hear regions

6-Recurrent sepsis

7-Disabling pain

8-Lymph leak

9-High risk regions: hemartrhrosis, DVT, PE

10-Secondary ischemic complications

41

What is congenital vascular bone syndrome?

Long bone abnormal growth caused by congenital vascular malformation

42

What do congenital vascular malformations contribute to leg length descrepency to make the affected limb longer?

AV shunts cause arterial hypertrophy and hypervascularization of the bone

43

What do congenital vascular malformations contribute to leg length descrepency to make the affected limb shorter?

Bone compression from vascular mass or reduction of arterial inflow from arterial hypoplasia will cause bone shortening. 

44

How does the marginal vein relate to congenital vascular bone syndrome?

It causes angio-osteo hypertrophy and limb overgrowth

45

What are 4 clinical features of Klippel Trenaunay Syndrome?

1-port wine stain localized to a small area

2-limb overgrowth/gigantism

3-large cluster of varicose veins

4-large lateral venous collector (Marginal Vein)

46

What type of congenital vascular malformations do Klippel Trenanay patients get? Arteriovenous, venous or lymphatic?

All KTS patients get venous, lymphatic and combined hemolymphatic

47

How do you treat the majority of Klippel Trenaunay Syndrome patients?

Compression stockings (35-45) and sclerotherapy

48

49

What is the difference between park weber syndrome and klippel trenaunay syndrome?

Very similar. PWS associated with arteriovenous malformations that require embolization in symptomatic patients. 

50

What is "Localized Intravascular Coagulopathy"?

Extratruncular venous malformations associated with coagulopathy due to stasis of blood within abnormal structures

51

What are phleboliths?

Microthrombi from localized intravascular coagulopathy associated with venous malformation that take on a "stone like" structures that you can see on plain x-rays. May be an indication to anticoagulate. 

52

Why do congenital malformation patients need to be followed life long?

Menarche, pregnancy, hormonal therapy, trauma, or surgery can trigger activity in previously quiet lesions and they can grow rapidly 

53

What are "high flow" congenital vascular malformations?

arteriovenous fistulas

54

What are "low flow" congenital vascular malformations?

Venous, lymphatic, capillary

55

Name 3 vascular tumours

  1. Infantile hemangioma,
  2. kaposiform hemangioendothelioma,
  3. tufted angioma

56

How do you treat capillary malformations for cosmesis?

Flashlamp pulse dye laser therapy

57

How are macrocystic lymphatic malformations treated differently from microcystic?