Vascular Anomolies Flashcards
What is the first order classification of vascular anomalies?
Vascular tumors
vascular malformations
What is the sub-classification of vascular tumours?
infantile hemangioma
hemangioendothelioma
angiosarcoma
Misc
What is the sub-classification of vascular malformations?
Slow-flow
capillary malformation CM
lymphatic malfomation LM
venous malformation VM
Fast flow
arterial malformation
Combined
What is the most common location for infantile hemangiomas?
head and neck
Describe what slow flow CM are and treatment.
Red macular lesions seen at birth and persist throughout life
Laser tx for cosmesis
Names three diseases associated with SF CM.
sturge weber
cutis marmorata telangiectatica congenita
Macrocephaly-CM
Describe what SF VM look like and treatment.
Bluish compressible swellings
Can be painful
Can be extensive and lead to leg length discrepancy
At risk for systemic coagulopathy after trauma
Tx OR can have significant blood loss
tourniquet
Names three diseases associated with SF VM.
Glomuvenous malformation
Cutaneomucosal venous malformation
Blue rubber bleb nevus syndrome
Describe what SF LM are and treatment.
Microcystic cmor combined
Tx lenticular excision
Dissect off skin, not malignant need not excise everything
May need to remove associated viscera
Post op, suction drainage
Name types of fast flow VM.
AVF
AVM
What are symptoms of AVF?
LE—edema, hypertrophy, trophic changes
UE—pain, digital ischemia, discoloration of the digits
What is an AVM?
Center call nidus has feeding vessels, micro and macro AVFS and ecstatic veins
Where are AVM most commonly found?
Intracranial, followed by limbs, trunk and viscera
How do you stage AVM?
I quiescence
Cutaneous blush, warmth (av shuntung on Doppler)
Stage II expansion
Bruit, audible pulsation, expanding lesion
Stage II destruction
Pain ulceration, bleeding, infection
Stage IV decompensation
Cardiac failure
What are indications to treat AVM?
pain ulceration, functional impairement, soft tissue and bony destruction and bleeding.
CHF
What is treatment for AVM?
Embolization alone or in combo
Embo nidus then resection next few days
What are variation of combine VM?
fast or slow
any combo of C, L, V, A
What is klippel-trenaunay syndrome?
slow flow Capillary-lymphaticovenous malformation
Large and small CM extremities and buttock
Lymphedema and LM in LE
Limb hypertrophy present at birth
What are side effects of KTS?
Infections, PE, thrombophleb, GI, constipation, bladder outlet obstruction , hematuria
What is parks weber?
Fast flow capillary arteriovenous malformation CAVM or CLAV<
Usually LE and trunk
Cutaneous flushing wih underlying multiple micro AVFS
large, flat, pink stains on the skin, and because of their color are sometimes called “port-wine stains
What is most common tumour of infancy?
infantil hemangioma
What is infantile hemangioma?
benign tumor of the endothelium
most common neoplasm of infancy
gros rapidly in first year then growth plateaus then shrinkage
bright color fades becomes less tense.
last phase complete by 4 yo
What is most common site of infantile hemangioma?
liver
can cause hepatomeg, IVE compression
How to diagnose infantile hemangioma?
US or MRI
What is treatment of infantile hemangioma?
conservation
topical steroid
laser
embolic only for CHF
excision only if at risk for injury and blood loss
What is the cause of primary raynauds?
idiopathic
What are connective tissue causes of secondary raynauds?
systemic sclerosis
inflammaroty muscle disease
Lupus
Sjogren’s syndrome
Vasculitis
What drugs can cause secondary raynauds?
BB
clonidine
ergotamine
vinyl chloride
What occupational cause can cause raynauds?
HAVS
TOS
What illness that impair digital perfusion can cause secondary raynauds?
paraproteinemia
cryoglobulinemia
malignancy
What are some large vessel disease that can cause secondary raynauds?
athero
thromboangitis obliterans
What are proposed pathogenic cases of raynauds?
vascular impaired vasod (impaired NO and prostacyclin) increased vasoconstriction (overproduction of ATII, ET-1) structural vascular abnormalities
Neural
impaired vasod (calcitonin gene related peptide)
increased vasoc (abnormal adrenergic function)
central (stress induced)
Intravascular factos (plt WBC activation, increased thrombin generation)
Genetic
What are important questions on hx?
tissue loss
CTD (difficulty swallowing, photosensitivity, mouth ulcers)
asymmetry
what drugs
occupational hx
fam hx
RF for athero
bruits, pulses
What investigations to do for raynauds?
nailfold cappilaroscopy (abnormalities)
angio/NIV for large vessel
Thermography (normal thermal gradients at room temp for PRP but not for sclerosis)
What is medical therapy for raynauds?
remove triggers
CCB
ACEi
phosphodiesterase inhibitors
topical NO, nitrate
When to do sx? what sx options for raynauds?
when medical treatment fails
ulcer debridement
cervical sympathectomy (symptoms may return)
periarterial symapthectomy
Balloon angio
arterial reconstruction
excision of calcific deposits
amputation
botox injections
What is the angio anatomic classification of raynauds?
type I: occlusion of R or U artery, decreased flow in level 2-3 arteries
type II: as I but with stenosis of R or U artery
type IIIa: main disorder in common digital or digital arteries
type IIIb: rare, occlusion of the digital arteries to index finger caused by vibration
type IV: all level stenotic
Type V: global ischemia, paucity of vessels, scant flow on angio
What is the technique for digital sympathectomy?
bruner Z-shaped incision
stripp adventitia of common digital artery
How many neurone are in the motor sympathetic route?
3
Where is the thoracic sympathetic trunk located?
in the middle of the intercostal space either bottom edge of top rib or top edge of bottom rib
Name indications for cervicothoracic sympathectomy
essential hyperhidrosis
ischemia of the hand
CRPS
Long QT syndrome
Raynauds
What are surgical approaches to sympathectomy?
paravertebral
transthoracic
supraclavicular
VATS (gold standard)
What is the target for CRPS vascular disease and raynauds?
stellate, G2 and G3
What are results of sympathectomy for raynauds?
50% dissatifaction
60% compensatory hyperhidrosis
may no longer be recommended
What are results of sympathectomy for CRPS?
Success 85% needs to be done early
What are common complications to sympathectomy?
Compensatory sweating
Segmental atelectasis
Penumo
Subcut emphysema
What are rare complications of sympathectomy?
Horners
Hemo
Effusion
Injry to vagus, phrenic, scla, sclv
What are causes of failure of sympathectomy?
Incomplete denervation
Regeneration
Functional reorganization
What arête appears to be preferentially affected in secondary raynauds?
Ulnar
What is FMD?
Non-athersclerotic noninflamm angiopathy of unknown cause. Medium sized vessels
What are complication of carotid FMD and how many get comps?
10%
Decreased lumen size
Formation distal embolization of thrombus
Dissection/rupture
What is the most common pathophys for cFMD?
medial fibroplasia 90%
What are most common concurrent lesion with cFMD?
ipsi bifurcation 20%
extracranial CA anerusy.
CA dissection
vert artery FMD
intracranial aneurysm/occlusive
RA FMD
What are diagnostic methods?
duplex-may miss distal dz
angio–best, string of beads
CT-better then MRA
MRA-signal dropout may appear to be beads
What are therapeutic challenges for cFMD?
Difficult to quantify severity of disease
If concurrent lesion, difficult to determine which is causing symptoms
If ipsi sympto and contra asympto, difficult to know what to do with contra side
May have nonfocal symptoms due to global ischemia
Intracranial aneurysms may alter surgical approach
HTN from RA FMD may complicate sx on carotid FMD
What is conservative therapy for cFMD?
Antiplt
Monitor q6 months
Rule out involvement in other arterial beds
Avoid chiro
What are invasive treatment strategies?
Open surgical dilatation
Rigid dilators disrupt obstructive webs
May need to ivied belly of digastric muscle
Start with 1.5mm
Back bleed
Stroke 3%
10 year patency, stroke-free and survival all about 90%
Dilatation mainstay of treatment
Stenting
Durability of PTA/stent unknown
May need to use external to get sheath/balloon up
Balloon should cover entire lesion and be slightly undersized to avoid dissection
Stent only if needed
What is the classification of FMD?
intimal fibroplasia
medial dysplasia
medial fibrosis
perimedial
medial hyperplasia
adventitial fibroplasia
What is the most common type of FMD?
medial
What are proposed cause of FMD?
May be hormonal factors
Occlusion of vaso vasorum
Long straight segs without branches are affected
CA and RA also get a lot of motion damaging VV.
Right renal longer so this may be why its affected more
Smoking
Familial
EDS
What are most common patterns of involvement?
Uni Right
Bilat renal
Uni left
Carotid/vert
Multiple
How common a cause of HTN is r FMD
2nd most common cause
What is the natural hx of r FDM?
About 25% of asympto devel HTN within 4 years
40% angio progression
decrease of renal funcition less frequent then with athererosclerotic
What are best tools for diagnosis?
DUS/CT/MRA are screening only because limited resolution for distal renal vessels
angio for diagnosis
What are indication for interventions for rFMD?
renal to aortic P gradient of 0.9
change in kidney size of 1 cm
refractory HTN
impaired renal function
size difference of >1.5cm
pressure gradient across stenosis of 10mmhg
What are indications for evaluation of secondary HTN?
>3 anti-HTN
sudden acceleration of CR or HTN
increased CR with ACEi
<30 yo
spontaneous hypoK
unexplained pulm edema
bruit
What are medical treatments for rFMD?
ASA, statins
Anti-htn treatment <140/90
What are surgical options for rFMD?
aortorenal bypass
autotransplantation
When to consider autotransplantation in rFMD?
if reop
failed endo
multiseg
single kidney with stenosis in several RA
What is Takayasu’s?
immune arteritis causing inflammation of aorta, its major branches, and pulmonary arteries.
What are proposed aetiologies of Takayasu?
genetic (HLA-Bw52) immune mediated (cell and antibody)
What is histology of TA?
panarteritis (3 layers) with skip area
excessive ground substance in intima
What are clinical features of TA?
claudication (UE>LE)
diminished pulses
MSK symptoms
constitutional symptoms
H/A
CVI symptoms’
HTN common
What are findings on duplex?
thickened wall
Describe the1994 Tokyo international conference angiographic classification for TA?
Type I aortic arch branches alone
type II ascending, arch, branches
Type III ascending, arch branches and descending
Describe the1994 Tokyo international conference angiographic classification for TA?
Type I aortic arch branches alone
type II ascending, arch, branches
Type III ascending, arch branches and descending
Type IV abdominal aorta and branches
Type V entire aorta
What are features of active disease?
fever, myalgias
elevated ESR
vascular ischemia (claudication)
typical angio features
What is medical treatment?
glucocorticoids first line treatment
methotrexate, cyclophosphamide, azathioprine
What is invasive treatment strategies?
dont operate on active disease
endo poor results
bypass good patency
What are RF for TAO?
male
smoking
What are features of the chronic phase of TAO?
organization of occlusive thrombus with recanalization
perivascular fibrosis
What are features of the intermediate/subacute phase phase of TAO?
partial recanalizations
disappearance of microabcess
deposition of immunoglobulins
What are features of the acute phase of TAO?
panvasculitis of small and medium vessels
occlusive, highly cellular arterial thrombus with microabcess
What are diagnostic criteria for TAO (shinoya criteria)?
smoking history/cocaine use
onset before 50
infrapop occlusive disease
upper limb involvement or phlebitis migranc
absence of other atherosclerotic RF
What are angiographic features of TAO?
Segmental occlusive lesions
more severe disease distally involvement of digital arteries,
normal proximal arteries without evidence of atherosclerosis
collateralization around areas of occlusion with corkscrew-shaped collaterals (Martorell’s sign, also described as “tree root” or “spider leg” collaterals)
What are angiographic features of TAO?
Segmental occlusive lesions
more severe disease distally involvement of digital arteries,
normal proximal arteries without evidence of atherosclerosis
collateralization around areas of occlusion with corkscrew-shaped collaterals (Martorell’s sign, also described as “tree root” or “spider leg” collaterals)
What are life style changes for TAO?
smoking cessation
exercise training
foot care
What are medical treatments for TAO?
ABX and NSAIDS for superficial phlebitis
CCB for vasospams
regional sympathetic block
statin
cilostazol
What are medical treatments for TAO?
ABX and NSAIDS for superficial phlebitis
CCB for vasospams
regional sympathetic block
statin
cilostazol
What are therapeutic options for TAO?
PCA
debride
flaps
amp
