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R9 > Chapter 142 - Fibromuscular dysplasia > Flashcards

Chapter 142 - Fibromuscular dysplasia Flashcards

1
Q

Fibromuscular dysplasia definition

A

1) Nonatheromatous noninflammatory proliferative process

2) medium sized conduit (long) arteries most common (renal, ICA)

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2
Q

First description of FMD

A

1938 String of beads appearance

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3
Q

Epidemiology of FMD

A

1) women > 90%
2) age 20-60
3) 10% familial
4) caucasians 95%

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4
Q

FMD and renal stenosis key points

A

1) 2nd most common renal stenosis

2) most common renal HTN in children

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5
Q

FMD associations with other factors and conditions

A

1) smoking
2) estrogen
3) ACE polymorphism
4) phenochromocytoma
5) Marfan
6) Alport syndrome
7) Takayasu

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6
Q

Pathogenesis of FMD

A

1) Vasovasorum damage from motion
2) long arteries with less vasovasorum more likely
3) Right renal > left renal to be affected and have ptosis

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7
Q

Differential diagnosis of FMD

A

1) Neurofibromatosis 1
2) EDS4
3) Williams syndrome
4) vasculitis

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8
Q

Differentiating NF1 and FMD

A

NF1 has characteristic skin lesions

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9
Q

Differentiating EDS4 from FMD

A

1) acrogeric dysmorphism
2) skin elasticity
3) distal joint laxity

in EDS4

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10
Q

Differentiating williams syndrome from FMD

A

1) facial dysmorphism
2) supra-aortic stenosis
3) behaviour changes

in Williams

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11
Q

Angiographic classification of FMD

A

1) Multifocal string of beads

2) focal with single area of stenosis

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12
Q

Histopathologic scheme of FMD

A

1) Intima fibroplasia (5-10%)
2) medial fibroplasia (80-85%)
3) periarterial/adventitial fibroplasia (<1%)

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13
Q

Secondary events from dysplastic lesions in FMD

A

Aneurysm 17% (1/3 in renal)

Dissection 20% (1/5 in renal)

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14
Q

Medial fibroplasia of FMD subdivided classes

A

1) Medial fibroplasia
2) perimedial fibroplasia
3) medial hyperplasia

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15
Q

Intimal fibroplasia key points

A

1) young no gender difference
2) collagen deposition in intima
3) disrupted internal elastic lamina
4) focal ring-like stenosis

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16
Q

Medial fibroplasia key points

A

1) Age 20-70
2) female:male 5-9:1
3) thinned media alrternating with thickened fibromuscular ride with collagen
4) can have secondary intimal hyperplasia
5) string of beads with beads larger than native vessel
6) distal 2/3 of main renal and branches 25%

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17
Q

Perimedial fibroplasia key points

A

1) young female < 50
2) patchy collagen deposition
3) intact external elastic lamina
4) string of bead but diameter less than native

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18
Q

Adventitial fibroplasia

A

1) no gender difference
2) collage replace normal adventitia
3) unifocal to long stenosis

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19
Q

Multiple vascular bed rate in FMD

A

2 vascular bed: 35%

3 vascular bed: 22%

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20
Q

difference in FMD and atherosclerotic lesions affecting renal function

A

FMD is mainly RAAS driven

Atherosclerosis can also be inflammatory driven

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21
Q

FMD natural history from asymptomatic in 4 years

A

1) 25% get HTN

2) 40% get FMD progression angiographically (overestimate)

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22
Q

Percentage of secondary hypertension in all HTN

A

5%

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23
Q

Indications for the evaluation of secondary HTN

A

1) require 3+ drugs
2) sudden elevation Cr and worsening HTN
3) <50 years old
4) spontaneous hypokalemia
5) bruit
6) flash pulmonary edema

24
Q

Limitations of MRA and CTA in size of vessels

A

2mm

25
Q

Gold standard for FMD diagnosis

A

Catheter DSA

26
Q

Indication for treating FMD

A

1) refractory HTN
2) intolerance/noncompliance to meds
3) impaired renal function
4) renal size decreased > 1cm

27
Q

BP target in fmd

A

BP < 140/80

in DM < 130/80

28
Q

First line antiHTN drugs 5 classes

A

1) ACEi
2) ARB
3) beta blocker
4) CCB
5) diureticsd

29
Q

Renal angioplasty in FMD key points

A

1) 0.15 mg nitroglycerin helps with vasospasm
2) cutting balloons not used unless IVUS shows adequate thickness of wall and not hypoplastic
3) stent usually not needed

30
Q

Complications following endo treatment of FMD

A

1) access 3-26%
2) dissection 1.4-6.7%
3) rupture 2-6%
4) branch occlusion 1-5%

31
Q

Factors that increase need for reintervention endo in FMD

A

1) branch vessel involvement

2) complex lesions

32
Q

Indications for treating FMD with open surgery

A

1) complication following endo

2) concurrent aneurysm disease

33
Q

Role of mannitol in renal protection

A

1) diuresis

2) hydroxyl free radical scavenger

34
Q

Dose of mannitol for renal protection

A

12.5-20g

35
Q

Open renal artery repair in FMD key points

A

1) cannot reimplant because too short
2) conduit GSV, IIA, prosthetic
3) renal protection

36
Q

Autotransplantation of kidney key points

A

1) cold perfusion with Saline, Ringers or Wisconsin (4C, 500 ml)
2) Take patch off IVC to ease in anastamosis

37
Q

Complication of open surgery in FMD

A

1) morbidity 19-28%
2) post-op occlusion 3.8-13%
3) restenosis 0-16%
4) vein graft dilatation late 20-44%

38
Q

Pediatric causes of renal artery stenosis

A

1) FMD
2) developmental RAS
3) NF1
4) Moyamoya
5) Takayasu
6) Alagille syndrome
7) Williams syndrome

39
Q

Percent of pediatric HTN due to RAS

A

8-10%

40
Q

Rate of bilateral carotid FMD

A

35-85%

41
Q

Epidemiology of carotid FMD

A

Female 40-60 make up 60-90% of all cases

42
Q

Rate of carotid FMD causing emboli, thrombosis, dissection leading to aneurysm

A

< 10%

43
Q

Location of lesion of carotid FMD compared to atherosclerotic

A

more distal in FMD

44
Q

Rate of kinking in carotid FMD

A

5%

45
Q

Rate of concurrent atherosclerotic disease in carotid FMD

A

20%

46
Q

Rate of carotid aneurysms in carotid FMD

A

21.1%

47
Q

Rate of carotid dissection in carotid FMD

A

5.6 - 75%

48
Q

rate of Concurrent vertebral FMD in carotid FMD

A

7-38%

49
Q

rate of Intracranial aneurysm and occlusive disease in carotid FMD

A

51%

80% solitary lesion, 20% multiple
usually same side as carotid lesion

50
Q

Rate of coexisting renal FMD in carotid FMD

A

8-40%

51
Q

Neurological symptoms on presentation in carotid FMD

A

1) stroke 12-27%
2) hemispheric TIA 31-42%
3) amaurosis fugax 22-28%
4) Horner 12.4%
5) cranial nerve abn 9.4%
6) focal neuro deficit 13.6%

52
Q

Sensitivity and specificity of carotid bruit for carotid FMD

A

Sen 45.4%

Spe 93.7%

53
Q

1st line treatment of asymptomatic carotid FMD

A

1) antiplatelet

2) monitor

54
Q

Special considerations for carotid FMD different from atherosclerotic

A

1) multiple lesions which one to treat
2) concurrent aneurysm intracranial
3) concurrent HTN
4) contralateral asymptomatic lesion what to do
5) cannot use existing evidence on degree of stenosis

55
Q

Surgical technique in carotid FMD

A

1) percutaneous angioplasty
2) Open dilation technique
3) open angioplasty
4) interpositional bypass

data limited for all

56
Q

Open dilation of carotid FMD stroke rate

A

1.4-2.6%
TIA 1.4-7.7%
Cranial nerve injury 5.1-16.7%

R9 (200 decks)

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