Miscellaneous: FMD, other aneurysms, AV malformation, peds, tumours Flashcards
(198 cards)
1
Q
What vessels does fibromuscular dysplasia affect?
A
Most medium sized arteries - e.g. internal carotid and renals
2
Q
What layer of the vessel does fibromuscular dysphasia affect?
A
Media
3
Q
What is the prevalence of fibromuscular dysphasia?
A
Unknown
4
Q
Who gets fibromuscular dysphasia? (Age, sex)
A
Young females
5
Q
What is the theory for pathogenesis of fibromuscular dysplasia?
A
Ischemia of vasovasorum. Arteries affected are internal carotid, vertebral and renals that have long segments without branch points thus less vasovasora.
6
Q
What is the definition of nutcracker syndrome?
A
Compression of left renal vein by SMA. Nutcracker syndrome = constellation of symptoms. NC phenomenon if asymptomatic.
7
Q
What are risk factors of nutcracker syndrome?
A
Low BMI, young female
8
Q
How can low BMI lead to nutcracker syndrome?
A
Paraspinal muscle wasting and low mesenteric/retroperitoneal fat means that SMA is at a more acute angle <16 degrees vs normal 35-40 degrees
9
Q
What are the 3 anatomical types of nutcracker syndrome?
A
Anterior and posterior. Also “atypical” circumaortic renal vein or congenital malformations e.g. left IVC
10
Q
What is posterior nutcracker syndrome
A
Usually with retroaortic vein, compression of left renal vein against vertebral body
11
Q
What are symptoms of nutcracker syndrome?
A
Hematuria and left flank pain. Also genital varices, pain with menses/sex/urination, protienuria and abdo pain
12
Q
What is the duplex criteria for nutcracker syndrome?
A
Flow velocities/diameter proximal and distal to stenosis ratio > 5
13
Q
What is the most common open operation for nutcracker syndrome?
A
Renal vein transposition onto the IVC more caudally. Gonadal and adrenal veins often have to be ligated.
14
Q
What are open options for anterior nutcracker syndrome?
A
Left renal vein transposition +/- patch or saphenous vein cuff. Gonadal vein transposition. Saphenous vein bypass from LRV to IVC.
15
Q
What open operations can be used for both anterior and posterior nutcracker syndrome?
A
Gonadal vein transposition, saphenous vein bypass from LRV to IVC
16
Q
What is the rate of migration for renal vein stenting?
A
7%. Can possibly migrate to right atrium and require open heart surgery!
17
Q
What are the differences between hemangiomas and congenital malformations?
A
Hemangioma = tumour, grows rapidly, involutes by age 12, originates from proliferative endothelium
Congenital vascular malformations - inborn error, grow steadily in parallel with childs growth, never disappear or regress, quiescent endothelium
18
Q
What are the 6 types of congenital malformations according to the Hamburg classification?
A
1-Arterial
2- Venous
3-Arteriovenous shunting
4-Lymphatic
5-Combined hemolymphatic
6-Capillary
19
Q
What are the congenital vascular malformation embryologic subtypes?
A
- Extratruncular:
- infiltrating/diffus,
- limited/localized
- Truncular:
- stenosis or obstruction (hypoplasia, aplasia, hyperplastia, membrane congenital spur),
- dilation (localized = aneurysm, diffuse = ectasia)
20
Q
What abnormality is depicted here?
A
Portwine stain - Klippel Trenaunay syndrome. Limb swelling and leg length discrepancy. Hemolymphatic malformation.
21
Q
Who gets congenital vascular malformations more? Men or women?
A
1:1, M:F
22
Q
What is the most common type of congenital vascular malformation?
A
Venous
23
Q
What are 5 types of risk factors during pregnancy that are associated with congenital vascular malformations?
A
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
24
Q
When do extratruncular congenital malformations arrest development?
A
Early embryonic life while vascular system is in the reticular stage.
25
What stmulates extratruncular CVMs to grow and proliferate?y,
Internal stimulation: menarche, pregnancy hormonal release
External: trauma, surgery
26
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.
27
What has a higher rate of recurrence - extratruncular or truncular CVM?
Extratruncular
28
What type of CVMs are associated with more serious hemodynamic consequences? Truncular or extratruncular?
Truncular
29
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.
30
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)
31
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
32
What type of CVM is this? Soft bluish swelling. Easily compressible. Enlarge with dependency/valsalva. Respond to compression therapy.
Venous
33
What type of CVM is this? Ischemic changes, distal gangrene, high output heart failure
Arteriovenous
34
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.
35
What is the name of the prominent vein on the back of this patients leg?
Marginal vein (embryonic remnant failed to involute). Parker weber syndrome.
36
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
37
Which type of congenital vascular malformations need to be treated aggressively?
Extratruncular. They grow and will recur unless the lesion nidus is completely destroyed
38
Which type of congenital vascular malformations can be handled conservatively unless they become hemodynamically significant?
Truncular lesions. They lack the potential for proliferation.
39
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
40
What is congenital vascular bone syndrome?
Long bone abnormal growth caused by congenital vascular malformation
41
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
42
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.
43
How does the marginal vein relate to congenital vascular bone syndrome?
It causes angio-osteo hypertrophy and limb overgrowth
44
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)
45
What type of congenital vascular malformations do Klippel Trenanay patients get? Arteriovenous, venous or lymphatic?
All KTS patients get venous, lymphatic and combined hemolymphatic
46
How do you treat the majority of Klippel Trenaunay Syndrome patients?
Compression stockings (35-45) and sclerotherapy
47
48
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.
49
What is "Localized Intravascular Coagulopathy"?
Extratruncular venous malformations associated with coagulopathy due to stasis of blood within abnormal structures
50
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.
51
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
52
What are "high flow" congenital vascular malformations?
arteriovenous fistulas
53
What are "low flow" congenital vascular malformations?
Venous, lymphatic, capillary
54
How do you treat capillary malformations for cosmesis?
Flashlamp pulse dye laser therapy
55
How are macrocystic lymphatic malformations treated differently from microcystic?
56
Name 3 signs and symptoms of large vessel vasculitis
1. Bruit
2. Asymmetrc BP
3. Claudication
57
Name 5 signs and symptoms of medium vessel vasculitis
1. Cutaneous nodules
2. Livedo reticularis
3. Digital gangrene
4. Mononeuritis multiplex
5. Renovascular HTN
58
Name 4 signs or symptoms of small vessel vasculitis
1. Palpable purpura
2. Pulmonary renal syndromes (glomerulonephritis, alveolar hemorrhage)
3. urticarial skin rashes
4. scleritis.
59
Name 2 large vessel vasculitis
"Giant Samurai"
1. Giant cell arteritis
2. Takayasu
60
Name 3 medium vessel vasculitis
"Obliterating your cow in a medium sized pan"
1. Thromboangitis obliterans
2. Kawasaki
3. Polyarteritis Nodosa
61
Name 5 small vessel vasculitis
Small German men who Cryo
1. Henoch Schonlen Purpura (IgA vasculitis)
2. Wegners (Granulomatosis w Polyangitis)
3. Cryoglobunimeic vascultis
4. Churg Strauss (Eosinophilic granulomatosis w polyangitis)
5. Microscopic polyangitis
62
Is giant cell arteritis more common in men or women?
Women (life time risk 1% for women, 0.5% for men)
63
What are the diagnostic criteria for giant cell arteritis?
3 of 5:
1. Elevated ESR
2. Age \> 50
3. Temporal abnormality (e.g. tenderness or decreased pulsation)
4. New headache
5. Abnormal biopsy (predominance of mononuclear cell infiltration or granulomatous inflammation with multinucleated giant cells)
64
What is the treatment for giant cell arteritis?
Give Cortocosteroids Always! (GCA)
IV methylprednisolone 1000 mg/day x 3 days if visual loss.
Otherwise 40-60 mg po daily.
65
What are classic CT findings of Ormond Disease (Idiopathic retroperitoneal fibrosis)?
Homogenous mass around the aorta following the iliac bifurcation with associated medial deviation of the mid part of the ureter and secondary hydronephrosis.
66
What are typical symptoms of Ormond Disease (idiopathic retroperitoneal fibrosis)?
Pain in lower back, abdomen, flank and scrotum
HTN
Systemic symptoms (fever, anorexia, malaise)
67
Which vasculitis is associated with a "hairy kidney"?
Erdheim Chester Disease
68
What feature distinguishes Erdheim Chester Disease from Ormond and IgG4 related systemic disease?
Bony lesions often of the lower extremeties that present with juxta-articular pain
69
What are the diagnostic criteria for polyarteritis nodosa?
3 of 10: ABCDEFGHI
1. Arteriographic abnormality (aneurysms or occlusions, string of pearls)
2. Biopsy of small or medium arteries showing polymorphic neutrophils.
3. Cr or BUN elevated
4. dBP \> 90
5/6. Extremity pain (myalgia) incl. testicular pain
7. Florid Livedo reticularis
8. Gross weight loss \> 4kg
9. Hep B infection
10. Impaired sensation (Mono or poly neuropathy)
70
Name 5 conditions that mimic polyarteritis nodosa
1. Segmental arterial mediolysis (SAM)
2. Pseudoxanthoma elasticum
3. Neurofibromatosis
4. Vascular type Ehlers Danlos (defect in Type 3 Collagen)
5. Adenosine deaminase 2
71
What are arteriographic findings consistent with Thromboangiitis obliterans?
"Corkscrew collaterals" around areas of occlusion with normal proximal arteries without evidence of atherosclerosis or emboli
72
What are 2 histologic features of thromboangiitis obliterans that distinguishes it from other vasculitis?
1. IEL is preserved
2. Venulitis also often seen
73
What are treatments for thromboangiitis obliterans?
1. Total cessation of all nicotine
2. Calcium channel blockers
3. IV iloprost
4. Sympathectomy
74
What percentage of patients with thromboangiitis obliterans that continue to smoke require an amputation?
50%
75
Which vasculitis involves small vessels, associated with pANCA and associated with pulmonary renal syndrome?
Microscopic polyangitis
76
What is the diagnostic criteria for Eosinophilic Granulomatosis with Polyangiitis (Churg Strauss)
APE MEN (4 of these 6)
1. A - asthma
2. Paranasal sinusitis
3. Eosinophilia
4. Migratory pulmonary infiltrates on CXR
5. Extravascular accumulation (e.g. nasal polyps)
6. Neuropathy
77
What are clinical features of Bechets Disease (focus on vascular symptoms)?
BOOGIE PANTS
1. Budd chiari
2. Oral ulcers
3. Ocular ulcers
4. Genital ulcers
5. Intracranial aneurysms
6. Extremity aneurysms (popliteal/femoral/brachial/iliac)
7. Pulmonary aneurysms - can have fatal hemoptysis
8. Aortic aneurysm
9. Neurologic symptoms
10. Thrombosis of veins (mesenteric, hepatic, renal)
11. Stomach/GI inflammation
78
What percentage of rheumatoid arthritis patients develop vasculitis?What percentage of rheumatoid arthritis patients develop vasculitis?
10-15%
79
Which vasculitis is a rare autoimmune disease manifested with recurrent inflammation of cartilaginous tissues? What vessels are involved?
Relapsing polychondritis, large-vessel (thoracic and AAA)
80
Which condition is an inherited systemic disease that can cause early and diffuse arteriopathy in absence of traditional risk factors?
Pseudoxanthoma Elasticum
81
Which size vessels does pseudoxanthoma elasticum affect?
Small and medium
82
How do patients with pseudoxanthoma elasticum present?
Lower extremity claudication
83
How can you treat pseudoxanthoma elasticum patients with claudication?
Surgical bypass and angioplasty has been effective. NB higher risk of bleeding with antiplatelet and anticoagulation due to risk of arterial rupture in GI and GU tracts.
84
What is the mechanism behind exercise related external iliac arteriopathy?
Usually in competitive cyclists. Repetitive bending and compression of EIA between psoas branch, circumflex iliac and internal epigastric arteries leads to vessel wall fibrosis, smooth muscle proliferation and intimal thickening.
85
Name 4 different vasculitis mimics.
1. Fibromuscular dysplasia
2. Radiation arteritis
3. Neurofibromatosis type 1
4. Drug induced/hypersensitivity vasculitis
86
Which vessels do Buerger's disease/thromboangiitis obliterans primarily affect?
Small and medium sized infrapopliteal and infrabrachial arteries and veins
87
Why did the 2012 Chapel Hill consensus guidelines remove thromboangiitis obliterans from list of vasculitides?
Absence of inflammation markers and antibodies makes TAO unique from other forms of vasculitis
88
Which condition is characterized by segmental inflammatory cell infiltration of vessel wall and arterial or venous thrombotic occlusion. The thrombus formation tends to be hypercellular and the vessel wall architecture including the IEL is preserved.
Buerger's (TAO)
89
What is the diagnostic criteria for Buergers (5)
Diagnosis of exclusion. No universally accepted criteria.
Shionoya Criteria:
1. smoking history
2. age of onset \< 50
3. infrapopliteal arterial occlusive lesions
4. either upper limb involvement or phlebitis migrans
5. no other atherosclerotic risk factors.
90
What is this image depicting? Which diagnosis is this a hallmark feature?
Corkscrew collateral ("dot sign") - Buerger's disease
91
Which investigations should you order for Buerger's patients?
1. Vasc lab: BP/ABI/Toe pressures, tcPO2, arterial duplex, digital hypothermic challenge
2. Angio: MRA/CTA or XA
3. Labs (r/o other conditions): CBC Lytes, Bun Cr, Liver enzymes, Glc. ,
* coags, thyroid, lipid profile, homocysteine, vit b12, coags: PTT, aPTT, protein C&S, ATIII, Factor V leiden, APLA, ACLA,
* autoimmune: ESR, CRP, ANCA, RF, ANA, cryoglbulins
* serologic markers for CREST
* Hep B&C serology
* Tox screen: cocaine, amphetamine, cannabis
4. Exclude embolic sources: echo, tele, abdo ultrasound, angio
5. Consider biopsy if unusual features (e.g. age \> 50)
92
What are 5 angiographic findings in Buerger's disease?
1. Small and medium sized arteries, digital arteries
2. Segmental occlusive lesions (normal appearing segmeets interspersed)
3. More severe distally
4. Tapering or abrupt occlusion with corkscrew collateral around occlusion
5. Normal proximal arteries (no aneurysms, atherosclerosis or sources of emboli)
93
What is the rate of major amputation with Buerger's disease at 5, 10 ad 20 years? If you stop smoking, how long until risk of amputation is eliminated?
5 years - 10%
10 years - 20%
20 years - 23%
Risk eliminated after 8 years of no smoking
94
Name 8 pharmcologic treatments for Buerger's. Do they work?
1. Calcium channel blockers - no evidence written in chapter
**2. Prostacyclin - iloprost infusion showed better ulcer healing and relief of ischemic rest pain compared with ASA**
3. Prostaglandin E1 - limaprost - no significant difference
4. Phophodiesterase inhibitors: cilastozol (PDE-3), viagra (PDE-5) - few reported cases showing some benefit
5. Endothelin receptor antagonists - RCTs still needed
6. Thrombolytics - high bleeding rate, RCT needed
7. Folate - no difference in amputation rate
8. Statins - RCT needed
95
Do men or women typically get Takayasu's? Old or young?
Young women - often of childbearing age
96
Which layers of the vessel wall are affected by Takayasu's?
Panarteritis affecting all 3 layers
97
SLE diagnostic criteria?
PRUNE RASH (4 of 11)
1. Photosensitivity
2. and 3. Rashes (ie, 2. Discoid rash; 3. Malar rash)
4. Ulcers in mouth
5. Neurologic (ie, seizures, psychosis)
6. and 7. Elevated blood tests (ie, 6. raised antinuclear antibody; 7. positive SLE cells seen, positive anti-double-stranded DNA antibody, positive antismooth muscle antibody, false-positive VDRL test)
8. Renal (ie, proteinuria, hematuria, cellular casts)
9. Arthritis (non-erosive)
10. Serositis (ie, pleuritis, pericarditis, peritonitis)
11. Hematologic (ie, hemolytic anemia, leukopenia, thrombocytopenia)
98
Which condition is this CTA depicting?
Takayasu's arteritis - skip lesion with inflammation of aortic wall (low attenuation ring)
99
How is Takayasu's classified?
6 types (1994 Tokyo Conference)
1. Aorotic branches
2a. Aortic arch + branches
2b. Aortic arch + branches + thoracic aorta
3. Descending thoracic and abdo aorta + branches
4. Abdo aorta + branches
5. Entire aorta + branches
Add C or P for coronary or pulmonary involvement
100
What is the first line treatment for Takayasu's?
Glucococorticoids - oral can be started. Many will relapse and require additional agents (cyclophosphamide, azathioprine, methotrexate, or mycophenolate mofetil).
101
Name 7 indications for surgical revascularization for Takayasu's
1. HTN due to renal artery stenosis
2. Lifestyle limiting claudication
3. Cerebral ischemia or \> 70% symptomatic stenosis of cerebral vessels
4. Moderate aortic regurg
5. Cardiac ischemia with coronary artery stenosis
6. Severe aortic coarctation
7. Progressive aneurysmal enlargement and dissecting aneurysm
102
What is the locus for marfans syndrome? What mutation? What is its genetic transmission pattern?
Locus: 15q21.1
Mutation: FNB1
Autosomal dominant with complete penetrance
103
How do Marfan's patients die?
Cardiovascular mortality ~90%. Aortic root aneurysm risks life threatening aortic dissection and rupture. Advances in surgery has returned life expectancy of marfans to near normal (before ~37 years).
104
Which condition is associated with aortic root aneurysm?
Marfan's
105
What are 7 features of Marfan's syndrome?
Mitral prolapse
Aortic root dilatation/dissection
Retinal detachment
Fibrillin abnormality
Arachnodactyly
pNeumothorax/blebs
Skeletal abnormalities
106
Name 4 differential diagnoses for Marfans
Other conditions associated with FBN1 mutations:
1. MASS phenotype - usually meets 1 but not other Ghent criteria for Marfan's
2. Shprintzen-Goldberg syndrome - more phenotypic heterogeneity. Still associated with aortic root enlargement but also mental retardation.
3. Homocystinuria - also have mental retardation, history of VTE, and CAD
4. Congenital contractural arachnodactyly - shares skeletal features with Marfans but no ocular or cardiovascular manifestations
107
How do you survey Marfans patients?
Life long optholomagy, cardiology and orthopedic assessments. Lifelong transthoracic echo monitoring.
108
When should you surgically intervene on Marfans?
Children: \>1cm growth per year or 2-3 SD above nomogram "z score"
Adults: \>50 mm
109
How do you medically manage Marfans?
Avoid burst exercise (sprinting, weightlifting etc)
B-blockers or Losartan equally effective.
110
Which type of ehlers danlos is vascular subtype? How is it inherited?
4, autosomal dominant
111
Which mutation do vascular Ehlers Danlos patients have? What does it code for?
COL3A1 gene, which codes for type 3 procollagen. The abnormal type 3 collagen molecule cannot fold into a triple helix, and is slowly degraded. "protein suicide".
112
What are the 9 minor diagnostic criteria for Vascular EHDS?
CHEAT FANG:
1. Club foot
2. Hypermobility of small joints
3. Early onset varicose veins
4. AV carotid-cavernous fistula
5. Tendon/muscle rupture
6. Family history (suddent death in one or more close relatives)
7. Acrogeria (taut thin skin)
8. pNeumothorax
9. Gingival recessession
113
What are the 4 major diagnostic criteria for Vascular EHDS?
FABS:
1. Facial characteristic appearance (thin delicate nose, thin lips, hollow cheeks)
2. Arterial/uterine/GI rupture
3. Bruising
4. Skin thin/translucency
114
How can you confirm the diagnosis of ED IV?
1. Collagen testing - structurally abnormal collagen 3
2. Serum/blood testing of COL3A1 gene
115
Where do ruptures and dissections of ED IV patients occur? What about Marfan's?
ED IV = medium sized vessels
Marfan's = aorta
116
Name 3 differentials for ED IV?
Disorders of bruisability and woundhealing e.g. "battered child"
1. Von Willebrand's disease
2. Platelet disorders
3. Scurvy
117
How can you mitigate the risk of suture line bleeding when repairing ED IV vessels?
1. Felt pleged
2. Bioglue
118
Name 2 non-vascular but common complications of ED IV
1. GI perforation - 25% of all ED IV complications. Sigmoid colon most common location
2. Uterine and pregnancy related complications.
119
What medical treatment options are available for ED IV?
1. Celiprolol (controversial study) - 5 year decreased risk of rupture from 50% to 20%
2. Factor 7 transfusion for spontaneous abdo bleed
120
What 3 features are associated with Loeys Dietz, "classic triad"?
1. Aortic aneurysm/vascular tortuosity
2. Craniofacial abnormality - bifid uvula or cleft palate
3. Hypertelorism
121
How many types of Loeys Dietz are there?
1. Severe craniofacial and aortic aneurysms
2. Less severe craniofacial (only a bifid uvula or high palate) and aortic aneurysms
3. Aneurysms and early osteoarthritis
4. Aneurysm of aorta and cerebral tortuoustiy - aortic root can dissect or rupture at small diameter in childhood unlike EDSIV and Marfan's
122
What type of LDS dies earliest?
Type 1 - 23 years old (type 2 = 32, other types later onset)
123
What type of connective tissue disorder is associated with ectopia lentis
Lens dislocation - Marfan's
124
When should aneurysms of the thoracoabdominal and infrarenal aorta be repaired prophlyactically in LDS patients?
4 cm +.
Lower threshold compared with EDS IV because tissue fragility are dramatically more fragile in EDS IV. Loeys Dietz has much better tissue handling and can make better anastomoses.
125
What is the conservative/medical management of Loeys Dietz patients?
1. Beta blockade
2. Avoid "burst" activities
Similar to Marfan's. No RCT data to support initiating Losartan.
126
What is the difference between primary and secondary Raynaud's phenomenon?
Which is more common?
What is Raynaud's disease?
Primary - idiopathic
Secondary - associated with an underlying disease like rheumatoid arthritis, systemic sclerosis
Primary more common.
Raynaud's disease = old nomenclature
127
What is the "hunting response"?
Maximum vasoconstriction in responce to cold happens at 10-20 degrees Celsius. Beyond that there is a rhythmic fluctuation in finger flow caused by periods f vasoconstriction and vasodilation q 30s to 2 mins. The cold induced dilation protects the fingers from freezing in a cold environment and is impaired in secondary Raynaud's phenomenon because of occlusive arterial disease.
128
What are medical treatment options for Raynauds (5)?
1. Calcium channel blockers (nifidepine)
2. Prostaglandins (prostacyclins, iloprost)
3. Alpha adrenergic blockers (prazosin)
4. PDE5 inhibitors (sildenafil)
5. Endothelin inhibitors (bosentan)
6. Others: fluoxetine, ACE, nitrates, ARB (losartan)
129
Name 6 surgical/invasive therapies for Raynaud's phenomenon.
1. Botox
2. Sympathetic block
3. Thoracoscopic sympathectomy
4. Lumbar sympathectomy
5. Digital sympathectomy
6. Spinal cord stimulators.
130
Which renal artery is more affected in FMD - right or left? Any theories as to why?
Right - it's longer.
Theory that FMD may be caused by vessel wall ischemia due to vasa vasora damage because it happens in long vessels away from branch points which are prone to stretching and further away from where vasa vasorum originate. Right is longer than left renal artery.
131
Name 4 differential diagnoses for FMD and distincitive features that separate them?
1. Neurofibromatosis-1: characteristic skin lesions
2. ED IV: acrogeric dysmorphism, skin elasticity, distal joint laxity
3. Williams syndrome: facial dysmorphism, supraaortic stenosis, behaviour
4. Vasculitis: FMD is not inflammatory and has no associated increase in acute phase reactnatns, anemia, or thrombocytopenia
132
How is FMD classified?
1. Angiographically: multifocal - "string of beads" or focal
2. Histologically: intimal, medial or adventitial - not used as frequently since FMD patients undergo less surgery
133
When should you investigate for secondary causes of hypertension? 7 indications
1. \> 3 antihypertensive agents
2. Flash pulmonary edema
3. Suddem acceleration of serum Cr
4. Spontaneous hypokalemia
5. Bruit
6. Young \< 50 yrs
7. Worsening of previously well controlled hypertension
134
What is a hemodynamically significant renal artery stenosis?
1. Pressure gradient \> 10 mm Hg
2. Renal to aortic pressure ratio \< 0.9
135
What is the treatment of choice for FMD patients with uncontrollable hypertension due to renovascular hypertension?
Balloon angioplasty for renal vessels. Stenting is usually not required. Vessels respond well to angioplasty because the septae are broken. Also patients are young.
136
How are aortorenal bypasses approached (type of incision)?
Transverse subcostal - kidney can be exposed retroperitoneally or transperitoneally depending on extent of aorta needed to be exposed.
137
At what size should renal artery aneurysms be excluded/treated in patients with FMD?
\> 2 cm
138
What is the treatment of choice for carotid FMD?
PTA with flow reversal for distal protection. Stenting not ideal because of kinked arteries. Also difficult to find landing zone for other distal embolic protection devices.
139
What are the 2 most common causes of popliteal artery disease that is not atherosclerotic?
1. Popliteal entrapment
2. Adventitial cystic disease
140
What are the 6 types of popliteal entrapment syndrome?
1. Popliteal completes development before migration of medial head of gastroc. Gastroc is situated normally but pushes popliteal artery medially.
2. Popliteal artery is displaced medially because the medial head of gastroc has an abnormal insertion site on the femoral condyle.
3. Abnormal muscle slip or fibrous band from either medial or lateral femoral condyle
4. Persistence of axial artery as the mature distal popliteal artery - remains in its embryologic position deep to the popliteus.
5. Both popliteal artery and vein are entrapped by any of the type 1-4
6. Anatomic variant not known, possible gastroc hypertrophy
141
Name 7 non-atherosclerotic causes of peripheral artery disease
1. Popliteal entrapment
2. Adventitial cystic
3. Iliac artery endofibrosis
4. Fibromuscular dysplasia
5. Buerger's disease
6. Vasculitis (Takayasu, Giant Cell Arteritis, Behcets)
7. Chronic exertional compartment syndrome
142
How can you tell angiographically between popliteal entrapment syndrome and popliteal adventitial cystic disease?
Adventitial cystic disease popliteal stenosis should be visible when foot in a neutral position. Provacative maneuvers should unmask a popliteal entrapment.
143
Which types of popliteal entrapment are best approached from a medial vs. posterior approach?
Medial - I and II
Posterior - III and IV
144
When should you replace the popliteal artery vs. just release muscle/fibrous bands in popliteal entrapment syndrome?
Abnormal artery:
- PSV 250-275
- Velocity ration \> 2
- Occluded
- Stenoses
- Post steotic dilatation/aneurysm
145
Name 4 classes of infected aneurysms
1. Microbial arteritis - noncardiac source bacteremia or local spread
2. Septic emboli
3. Post-traumatic (e.g. illicit drugs)
4. Bacteremia of pre-existing atheroscerlotic aneurysm
146
Name 5 ways of dealing with an infected femoral pseudoaneurysm
1. Ligate & wide debridement (illicit drug use can ligate the femoral if SFA/profunda intact, test occlusion and see if you maintain a pedal doppler signal first)
2. In-situ reconstruction with vein or cryopreserved allograft and sartorius muscle flap
3. Obturator bypass
4. Latera femoral bypass from external to distal SFA
5. Axillary to distal SFA bypass.
147
Name 8 conditions associated with secondary Raynauds
COLD HAND
1. **C**ancer: multiple myeloma, leukemia
2. **O**ccupational: hypothenar hammer, vibration
3. **L**upus and other connective tissue diseases: scleroderma, rheum arthritis, Sjogrens
4. **D**rugs: vasopressors, cocaine, ergot
5. **H**ematologic: polycythemia rubra vera, thrombocytosis
6. **A**rterial: TAO, atherosclerosis, GCA
7. **N**asty infections: Hep B & C
8. **D**iabetes
148
Why do you need to exercise caution in administering ASA to children?
Potential for Reye syndrome - a rapidly progressing encephalopathy
149
Name 6 causes for pediatric aneurysms
1. Infection
2. Aortitis - takayasu
3. Arteritis - Behcet, SLE, kawasaki, PAN
4. Genetic - EDIV, LDS, Tuberous sclerosis, Marfans
5. Developmental/idiopathic
6. Traumatic
150
Name 4 major types of pediatric vascular tumours
1. Infantile hemangioma
2. Congenital hemangioma
3. Kaposiform hemangioendothelioma
4. Pyogenic granuloma
151
What anomalies can lower body infantile hemangiomas be associated with?
LUMBAR
1. Lumbar/spinal
2. Urogenital
3, Myelopathy
4. Bone deformities
5. Anorectal/arterial anomalies
6. Renal
152
What anomalies can a facial infantile hemangioma be associated with?
PHACES syndrome
1. Posterior fossa brain malformation
2. Hemangioma
3. Arterial abnormalities
4. Coarctation of aorta/Cardiac deficits
5. Eye abnormalities
6. Sternal clefting, supraimbulical raphe
153
What is the difference between a congenital hemangioma and infantile hemangioma?
Congenital hemangiomas are fully formed at birth and does not have the postnatal growth seen in infantile hemangioma
154
What is the hallmark histopathologic feature of infantile hemangiomas?
GLUT-1 positivity
155
How do you treat infantile hemangiomas that cannot be treated with observation alone or with topical agents?
Propanolol. Systemic steroids are given if patients cannot tolerate or have a contraindication to beta blockers.
156
Name the Neves and Zincke classification of this tumour thrombus
Level 3 - retrohepatic but still below the diaphragm without extension into the right atrium
157
Name the Neves and Zincke classification of this tumour thrombus
Level 2 - cephalad extent is more than 2 cm above the left renal vein but still infrahepatic
158
Name the Neves and Zincke classification of this tumour thrombus
Level 1 - cephalad extent is confined to less than 2 cm above the left renal vein
159
During an “en bloc ” resection of a colon cancer fecal contamination occurs and the IVC is resected 5 cm longitudinally and 50% circumferentially. List 5 ways to repair the IVC:
1. Lateral venorapphy with 3-0 or 4-0 Prolene if less than 50% stenosis
2. Patch repair (saphenous; prosthetic if no contamination). Patch repair preferred over interposition graft
3. Interposition graft (deep femoral vein or spiral saphenous vein graft; 16 mm PTFE if no fecal contamination)
4. Ligation of infrarenal IVC (unstable patient with severe injuries or when repair produces major stenosis or cancer patients with chronically obstructed IVC and developed collaterals)
5. Reconstruction of IVC with cuff of abdominal aorta and subsequent aortic reconstruction with ax-fem graft or rifampin coated graft (if no fecal contamination then regular Dacron tube graft repair)
160
Why should you reimplant the renal vein when doing IVC reconstruction/interposition grafting?
Maintains patency of IVC. (Usually doesn't affect renal function that much).
161
What are 2 methods to improve IVC reconstruction patency?
1) Renal vein reimplantation
2) Create AV fistulas (renal art/vein, SFA/GSV, renal art/gonadal vein)
162
When performing IVC reconstructions - which patients should you consider creating an AV fistula in?
High risk of graft thrombosis:
1) Infrarenal IVC (lower flow than suprarenal)
2) Long grafts/multilevel IVC recon
3) Small diameter grafts \< 16 mm in diameter
163
When do you need to use veno-venous bypass for IVC reconstructions? (5)
1. Pt unable to tolerate IVC clamping due to tenuous cardiopulmonary status or old age
2. Level 3 extension of tumour thrombous
3. Complex multilevel IVC involvement
4. Need full graft replacement
5. Adequate oncologic resection cannnot be achieved without bypass support
164
If you plan to embolize a splenic artery aneurysm, which vaccines do you need to give patients?
1. Pneumococcal
2. Meningococcal
3. Hemophilus influenza B
165
When repairing splanchnic aneurysms open, when can you ligate the celiac and common hepatic artery (what needs to be open for collaterals?)
Gastroduodenal artery, pancreaticoduodenal arteries, portal vein
166
When ligating vessels for a hepatic artery aneurysm, when do you also need to perform a cholecystectomy?
If the proper or right hepatic artery is ligated, a cholecystectomy should also be performed
167
What type of vascular injury is associated with pediatric fall on outstretched hand?
Brachial artery injury in context of supracondylar fracture
168
How should you manage pediatric patients with a pulseless pink hand?
Controversial - Rutherford's authors recommend exploring in stable patients and repair vessel as needed
169
What are the KDOQI guidelienes for when to place permanent HD access in pediatric patients?
KDOQI guidelines recommend placeing permanent HD access in all patients aged 0 to 19 who are greater than 20 kg and are not expected to receive a kidney transplant within 1 year
170
What are the 4 most common causes of ESRD in pediatric patients?
1. Glomerular sclerosis
2. Renal hypoplasia/dysplasia
3. Congenital obstructive uropathies
4. Lupus nephritis
171
What is the difference between chronic regional pain syndrome type 1 and type 2?
CRPS type II is distinguished by evidence of peripheral nerve injury.
172
Name 5 major theories about the pathogenesis of chronic regional pain syndrome
1. Exaggerated inflammatory response - inflammatory mediators TNF-a and IL-6 are elevated in affected limbs. Red/hot/swollen affected limb are all signs of inflammation. Some benefit of IVIG.
2. Sympathetically mediated syndrome
3. Limb ischemia and reperfusion injury
4. Central sensitization theory
5. Secondary to nerve damage
6. Autoimmune disorder
7. Cortical reorganization theory
173
Name the Stanton Hicks diagnostic criteria for complex regional pain syndrome
1. Clinical symptoms:
* burning, hyperpathia, allodynia
* Temperature or color changes edema
* Hair or nail growth changes
2. Laboratory results
* Thermometry or thermography bone radiography
* 3 phase bone scan quantitative sweat test response to sympathetic block
Interpretation: \> 6 reflex sympathetic dystrophy probable, 3-5 RSD possible, \<3 RSD unlikely
174
Name the Budapest clinical diagnostic criteria for complex regional pain syndrome
**1. Ongoing pain out of proportion to inciting event**
**2. 1 symptom in 3/4 categories:**
i) vasomotor: tempreature asymmetry/skin colour
ii) sensory: hyperesthesia, allodynia
iii) Motor/trophic
iv) Sudomotor/edema
**3. One sign in 2+ categories:**
i) vasomotor: temp asymmery
ii) sensory: hyperalgesia to pin prick and/or allodynia to light touch
iii) motor/trophic: decreased ROM or motor dysfunction or trophic changes
iv) Sudomotor/edema
**4. No other diagnosis better explains signs/symptoms**
175
A 3 phase bone scan with accelerated blood flow in the disease limb combined with increased diffuse activity during the blood-pool phase and increased periartericular uptake in the delayed statis phase is pathognomonic for which condition?
Chronic regional pain syndrome. However a meta-analysis states bone scans have a sensitivity of 50% therefore many believe are not accurate for diagnosing CRPS.
176
Name 9 medications that can be used in the treatment of chronic regional pain syndrome
1. Opoids
2. Tricyclic antidepressants
3. GABA agonists (e.g. gabapentin)
4. Alpha adrenergic blocking agents (e.g. IV phentolamine)
5. Beta blockers - propanolol
6. Calcium channel blockers
7. Bisphosphonates
8. NSAID
9. Steroids
177
What is the timeline and treatment for stage 3 chronic regional pain syndrome?
Timeline: \>6 months
Treatment recommendation:
1. Steroid therapy or sympathetic blockade should be considered but may be unsuccessful.
2. Neuromodulation (e.g. spinal cord stimulation) may be used.
3. Manipulation of joint contractures under general anesthesia
4. Antidepressants
5. Vocational guidance
178
What is the timeline and treatment for stage 2 chronic regional pain syndrome?
Timeline: 3-6 months
Treatment recommendation:
1. Physical therapy, TENS and steroids combined.
2. If fails, consider sympathetic blockade and surgical sympathectomy
179
What is the timeline and treatment for stage 1 chronic regional pain syndrome?
Timeline: 0-3 months
Treatment recommendation:
1. Physical therapy +/- TENS.
2. Local nerve or sympathetic block for patients w severe pain unable to undergo PT.
3. If fails: give steroids.
180
What are the indications for cervicothoracic sympathectomy?
1. Essential hyperhidrosis
2. Selected cases of hand ischemia
3. Complex regional pain syndrome
4. Clinically refractory long QT syndrome
5. Rare cases of Raynaud syndrome
181
What are the indications for a lumbar sympathectomy?
1. Essential plantar hyperhidrosis
2. Selected cases of chronic critical leg ischemia with no conditions or revascularization options
182
What are the denervation level(s) for: Long QT syndrome
Stellate ganglion, G2 and G3
183
What are the denervation level(s) for: Raynaud syndrome
Stellate ganglion, G2 and G3
184
What are the denervation level(s) for: Vascular disease
Stellate ganglion, G2 and G3
185
What are the denervation level(s) for: Complex regional pain syndrome?
Stellate ganglion, G2 and G3
186
What are the denervation level(s) for: plantar hyperhidrosis?
L2 and L3
187
What are the denervation level(s) for: facial rubor?
G2
188
What are the denervation level(s) for: craniofacial hyperhidrosis?
G2
189
What are the denervation level(s) for: axillary hyperhidrosis?
G4
190
What are the denervation level(s) for: palmar hyperhidrosis?
G4 or G3
191
Name 6 rare (\< 1%) causes of complications associated with thoracoscopic sympathectomy
1. Horner syndrome
2. Hemothorax
3. Pleural effusions
4. Injury to vagus nerve
5. Injury to phrenic nerve
6. Injury to subclavian artery and vein
192
Name 4 common causes of complications associated with thoracoscopic sympathectomy
1. Compensatory sweating 70-100%
2. Segmental atelectasis 1-5%
3. Pneumothorax 1-5%
4. Subcutaneous emphysema 1-2%
193
What are 3 causes of failure of thoracic sympathectomy?
1. Incomplete denervation
2. Functional reorganization (collateral nerve sprouting)
3. Regeneration of new fibers from the ganglion cell
194
Name 7 secondary IVC tumours that may have tumour thrombus
195
Name 7 secondary IVC tumours
196
Name 7 secondary SVC tumours
197
What is the most common primary tumour of the IVC?
Primary leiomyosarcoma
198
If you are resecting an IVC tumour and planning to patch angioplasty, how large should the diameter of your patch be?
D x 3 x % of caval wall to be replaced
E.g. IVC 20 mm with 50% resected - 20 x .5 x 3 = 30 mm
