Multidisciplinary Evaluation and Management of Complex Arteriovenous Malformations ·...
Transcript of Multidisciplinary Evaluation and Management of Complex Arteriovenous Malformations ·...
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Multidisciplinary Evaluation Multidisciplinary Evaluation and Management of Complex and Management of Complex
Vascular MalformationsVascular Malformations
Cynthia K. Shortell, MDCynthia K. Shortell, MDVascular ConferenceVascular Conference
October 30, 2009October 30, 2009
http://vascular.surgery.duke.edu
••
Vascular Vascular malformations?malformations?–
“Embryologically
developed, inborn errors of vascular morphogenesis leading to true structural anomalies”1
http://vascular.surgery.duke.edu
Arterio venous
Capillary
Venous
Lymphatic KTS
1. Mulliken JB, Glowacki J (1982) Hemangiomas and vascular malformations in infants and children. A classification based on endothelial characteristics. Plast Reconstr Surg 69:412–422.
•• Etiology:Etiology:–
Unknown, but genetic predilection
–
Molecular studies: •
VMs
are caused by dysfunctions in the signaling process regulating proliferation, differentiation, apoptosis, maturation and adhesion of vascular cells2
http://vascular.surgery.duke.edu
2. Cohen, M.M., Jr., Vasculogenesis, angiogenesis, hemangiomas, and vascular malformations. Am J Med Genet, 2002. 108(4): p. 265-74.
•
Spectrum of disorders ranging from minimal to significantly disabling conditions impacting patient’s anatomic, functional and emotional integrity
••
Incidence Incidence VMs
= 1.21.2--1.5% 1.5%
http://vascular.surgery.duke.edu
••
Obstacles to treatment and Obstacles to treatment and misconceptions about misconceptions about VMsVMs
• Expectation that lesions will regress• Lack of knowledge around diagnosis and
therapies• Fear/patients considered too high risk to treat
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••
Importance of multidisciplinary Importance of multidisciplinary approach approach –
Diversity of lesions and treatment options
–
The management of VMs
exceeds
the level of expertise of any single medical specialty
–
Consequently, many patients have been discouraged by the lack of correct diagnosis
and proper treatment
despite numerous visits to different clinics
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••
Multidisciplinary approach advantagesMultidisciplinary approach advantages–
Lessens
the need for multiple office visits
–
Streamlines
and expedites
patient care–
Gets all caregivers and staff on the same page
–
Best results
if patient evaluation and indication for treatment are based on the assessment of team members
–
Important to establish treatment strategy and TREATMENT GOAL(S) for each patient
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••
Duke Multidisciplinary Vascular Duke Multidisciplinary Vascular Malformation Team:Malformation Team:––
Vascular SurgeryVascular Surgery
––
Plastic SurgeryPlastic Surgery––
Pediatric SurgeryPediatric Surgery
––
Diagnostic Radiology Diagnostic Radiology ––
Interventional Radiology Interventional Radiology
––
Dermatology Dermatology ––
OphthalmologyOphthalmology
––
OrthopedicsOrthopedics
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Management ChallengesManagement Challenges
Nomenclature & ClassificationDiagnosis
Treatment Planning
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Inconsistent, archaic terminologyInconsistent, archaic terminology
• hemangioma
simplex• cavernous hemangioma
• birthmarks (naevi)• port-wine stains
• angiomas
/ angiomatas
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Nomenclature & Classification:Nomenclature & Classification:
The greatest confusion exists around the difference between “hemangiomas”
and
vascular malformations
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••
Numerous classification attempts in the Numerous classification attempts in the past:past:
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Mulliken and Mulliken and GlowackiGlowacki
Jackson et al.Jackson et al.
Belov (Malan, Degni) : Belov (Malan, Degni) :
trunculartruncular
and and extratruncularextratruncular
International workshop on International workshop on
Vascular Malformations Vascular Malformations Hamburg, GermanyHamburg, Germany
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Vascular Anomalies
Tumors Malformations
High Flow (AVM)
Low Flow
Venous Lymphatic
Combined
Hemangioma Other
Hemangiomas Hemangiomas Vascular MalformationsVascular Malformations
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vs.vs.
••
Vascular tumors:Vascular tumors:–
Infantile hemangiomahemangioma
the
most common type––
Proliferative phase during Proliferative phase during the first year of lifethe first year of life
–
Spontaneous involution–
Treatment often not needed
–
Therapy: usually medical (corticosteroids, interferon α)
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1 year 6 years
Retroauricular
infantile hemangioma:
••
Vascular Vascular malformationsmalformations–
Arterial, capillary, lymphatic, venous, combined
–
Do NOT regress–
Treatment indicated if symptomatic
–
Therapy: surgery, or interventional
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•
Hemangiomas are true neoplastic disorders and pathohistologically
they demonstrate increased endothelial cell turn over rate
•
Vascular malformations arise by dysmorphogenesis
without increased
endothelial proliferation
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http://vascular.surgery.duke.edu
Histopathology:Histopathology:
•Normal endothelial cell turn over rate (Hypertrophy)
••Increased endothelial cell turn Increased endothelial cell turn over rate (Hyperplasia)over rate (Hyperplasia)
Malformations Tumors
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Malformations vs. Tumors
••Infantile hemangiomas are Infantile hemangiomas are characterized by proliferative characterized by proliferative phase during the first year of life phase during the first year of life followed by followed by involutionalinvolutional
phase phase of slow, spontaneous of slow, spontaneous regressionregression
in most casesin most cases
••Vascular malformations Vascular malformations DO DO NOTNOT
regressregress
proliferative phasemid involutionlate involution
Histopathology:Histopathology:
Infantile hemangioma
Vascular malformation
Onset Infancy or childhood Anytime during lifetime
Course Proliferating, involuting, involuted
Commensurate growth or slow progression
Histology Increased cellular turnover
Normal cellular turnover
Triggering factors Unknown Hormones, trauma, spontaneous
Treatment
Spontaneous involution, corticosteroids, interferon α, surgery
Sclerotherapy, surgery, laser, embolization
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VS.
Vascular MalformationsVascular Malformations
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Management ChallengesManagement Challenges
Nomenclature & ClassificationDiagnosis
Inadequate treatment
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••
Diagnosis:Diagnosis:–
H&P
–
Duplex scan–
MRI
–
Arteriogram
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••
Physical Exam:Physical Exam:–
Assess presence and characterization of mass
–
Color / temperature of skin–
Thrill / bruit
–
Dilated veins and variation of mass with Valsalva
–
Presence of limb asymmetry and presence of lymphedema
http://vascular.surgery.duke.edu
••
Clinical FeaturesClinical Features–
Present at birth
–
May not be clinically apparent until later in life–
Growth and appearance may be stimulated by trauma, the effects of hormones (during puberty and/or pregnancy)
–
May occur in the absence of any identified triggering factors
http://vascular.surgery.duke.edu
In some cases, it may be challenging to distinguish
between low flow and high flow lesions….
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•
Every effort should be made to differentiate differentiate low flow from high flow
malformations
•
This process is the most critical
step in the evaluation and management of VM
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••
Duplex scanDuplex scan–
Useful to confirm the diagnosis, as it is rapid, easy and shows the low flow velocity and vascularization
–
Frequently inadequate to demonstrate the extent of the lesion
http://vascular.surgery.duke.edu
••
MRIMRI
is the imaging modality of choice in the evaluation of VM: –
Shows the lesion’s flowflow
characteristicscharacteristics
–
Provides good soft tissue definitiontissue definition–
Delineates the extent
of the malformation
throughout the involved tissues –
Determines relationship to normal circulation
and surrounding anatomic
structures–
Requires exact and expert timing and protocol
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••
ARTERIOGRAMARTERIOGRAM–
When MRI suggests the presence of a high flow/arterial component, the diagnostic workup should include an arteriogramarteriogram
–
If the arteriogram confirms a high flow component, treatment can be done at the same time
–
If the arteriogram excludes a high flow component, treatment can safely be designed for a low flow lesion
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High FlowHigh Flow
Vascular MalformationsVascular Malformations
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••
High FlowHigh Flow
Vascular Malformations Vascular Malformations ((ArterioArterio--Venous Malformations)Venous Malformations)–
Direct connection between arterial and venous system caused by congenital malformations resulting in fistulous A-V tracts
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••
PE: PE: –
Skin discoloration
–
Elevated cutaneous temperature
–
Dilated veins –
Thrill / bruit
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•
Can be aggressive; cutaneous
ischemia with ulceration, infection or hemorrhage
•
Can be painful•
High output cardiac failure when extensive
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•
Duplex:–
Multidirectional flow and high-amplitude arterial waveform with spectral broadening
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••
MRIMRI–
Best to evaluate extent of AVM and relationship to adjacent structures
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••
Arteriogram Arteriogram –
Used to evaluate the extent of lesion and to plan treatment
–
Allows precise evaluation of feeding arteries and draining veins-feasibility of embolization
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This arteriogram demonstrates AVM supplied by infraorbital
and ophthalmic artery
••
Therapeutic mainstaysTherapeutic mainstays––
Multimodal treatmentMultimodal treatment
including
preoperative embolization
and complete surgical resection, is usually necessary for the management of high flow vascular malformations
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••
Treatment of high Treatment of high flow vascular flow vascular malformationsmalformations–
Coil embolization
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http://vascular.surgery.duke.edu
••
Treatment of high Treatment of high flow vascular flow vascular malformations malformations –
Glue embolization
••
Treatment of high Treatment of high flow vascular flow vascular malformationsmalformations–
Glue embolization
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••
Surgical treatment Surgical treatment of high flow of high flow VMsVMs––
Surgical resectionSurgical resection
•
Pulsatile
mass at the base of long and ring fingers
•
Severe ischemia of the long finger
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•
Angiogram demonstrates a lesion originating at the palmar
arch and
extending into the long finger
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•
Resection and revascularization with an autogenous
vein graft
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•
One year after surgery–
All motion and sensation had returned to all the digits
–
The color and turgor of the long finger
pulp are dramatically improved
http://vascular.surgery.duke.edu
Low FlowLow Flow
Vascular MalformationsVascular Malformations
http://vascular.surgery.duke.edu
••
Clinical PresentationClinical Presentation–
Affects both superficial and deep underlying anatomic structures (skin, muscles, abdominal viscera, CNS)
–
Sx
severity variable based on size and extent –
Can be isolated or part of a syndrome
http://vascular.surgery.duke.edu
••
Presenting symptoms:Presenting symptoms:–
Skin discoloration
–
Varicosities–
Pain
–
Decreased mobility –
Swelling
–
Bleeding –
Osteomuscular
hypertrophy
http://vascular.surgery.duke.edu
••
Venous malformationsVenous malformations–
Typically bluish
–
Soft and easily compressible lesions –
Usually enlarge when affected extremity is dependent or after Valsalva
maneuver
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••
Venous malformationsVenous malformations–
No increase in local skin temperature
–
No thrill or bruit
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Venous malformation affecting fronto-temporal part of the face
••
Venous Venous malformationsmalformations–
Extensive extratruncular
malformation causing swelling of the upper extremity
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••
Venous Venous malformationsmalformations––
Malformation Malformation causing swelling of causing swelling of digit digit
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••
DuplexDuplex
of venous malformation reveals mixed venous waveform
••
DuplexDuplex
is inadequate to demonstrate the
extent of the lesion
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••
MRIMRI
gives a bright hypersignal
on T2-
weighted spin-echo sequences that delineates the extent of the malformation throughout the involved tissues
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••
Lymphatic malformationsLymphatic malformations–
Usually noted at birth or before the patient reaches the age of 2 years
–
Sponge-like lesions –
Skin overlying lymphatic malformations is usually normal in color
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••
Lymphatic malformations Lymphatic malformations –
Microcystic
–
Macrocystic–
Combined
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••
Duplex Duplex scan of lymphatic malformation shows complete absence of Doppler signal
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••
Lymphatic malformationsLymphatic malformations–
Most common complications:
• Infection • Swelling/compression of vital structures
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••
Capillary malformations Capillary malformations –
Present at birth and grow in proportion to the growth of a child
–
Appear as localized pink or red lesions
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••
Capillary malformationsCapillary malformations–
Commonly associated with enlargement and anomalies of underlying structures
–
Midline occipital capillary malformations can herald the presence of an encephalocele
or
ectopic meninges
http://vascular.surgery.duke.edu
••
Capillary Capillary malformationmalformationassociated with venous malformations and osteomuscular
hypertrophy in patient with KTS
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Vascular malformations can be isolated or as a part of a syndrome (ie
Klippel
Trenaunay
Syndrome, Proteus, Maffucci, Parkes-Weber)
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••
KlippelKlippel--TrenaunayTrenaunay
SyndromeSyndrome:–
Rare congentital
anomaly
–
No arterial component (w/arterial
comp = Parkes
Weber
syndrome)
http://vascular.surgery.duke.edu
••
KlippelKlippel--TrenaunayTrenaunay Syndrome Syndrome Triad:Triad:
–
Capillary malformations (“port wine stain”)
–
Venous/lymphatic malformation
–
Pathognomonic osteomuscular
hypertrophy
http://vascular.surgery.duke.edu
••
Special diagnostic considerations:Special diagnostic considerations:–
Aplasia or hypoplasia
of deep venous trunks
–
Localized Intravascular Coagulopathy
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••
Special diagnostic consideration ISpecial diagnostic consideration I––
AplasiaAplasia
or or hypoplasiahypoplasia
of deep venous trunksof deep venous trunks
• Present in 8 %8 %
of VM patients (with venous predominance)3
http://vascular.surgery.duke.edu
3. Eifert
S, Villavicencio JL, Kao TC, Taute
BM, Rich NM. Prevalence of deep venous anomalies in congenital vascular malformations of venous predominance. J Vasc
Surg
2000;31:462–71
•
Prevalence of deep venous anomalies is even higher in patients with KTS
•
Ascending phlebography
of
patient with KTS: absence of the left iliofemoral
vein
segment
http://vascular.surgery.duke.edu
(18%)(18%)
••
MRI reconstruction of MRI reconstruction of patient with KTS: patient with KTS: ––
Absent left common Absent left common iliac vein iliac vein
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•
Evaluation of patency and anatomic variations of the ENTIRE ENTIRE venous system (deep and superficial)
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••
Special diagnostic consideration IISpecial diagnostic consideration II––
Localized Intravascular Localized Intravascular CoagulopathyCoagulopathy
• Thrombosis of intralesional
blood• Usually latent and asymptomatic• If symptomatic: associated with painful
inralesional
thrombotic episodes • Patients can become severely
coagulopathic
even
during diagnostic procedures
http://vascular.surgery.duke.edu
••
Localized Intravascular Localized Intravascular CoagulopathyCoagulopathy–
Often erroneously labeled as Kasabach-
Merritt Syndrome (a distinct clinical entity characterized by profound thrombocytopenia associated with vascular tumors)
http://vascular.surgery.duke.edu
••
Localized Intravascular Localized Intravascular CoagulopathyCoagulopathy–
The platelet count in LIC is moderately diminished (100-150 x 103/ml)
–
The increased risk of bleeding in some of VM patients can be attributed to the increased consumption of coagulation factors4
http://vascular.surgery.duke.edu
4. Mazoyer, E., et al., Coagulation disorders in patients with venous malformation of the limbs and trunk: a case series of 118 patients. Arch Dermatol, 2008. 144(7): p. 861-7
•
Procoagulant
states are also associated with low flow VMs, including chronic intermittent PE
•
A hypercoagulabilityhypercoagulability
profile should be routinely performed in all VM patients prior to undergoing diagnostic evaluation, surgical intervention or sclerotherapy
•
Most PC states will not be detected however
http://vascular.surgery.duke.edu
••
Treatment of low flow vascular Treatment of low flow vascular malformationsmalformations–
Small lesions may be cured
–
Extensive lesions therapy palliative/goal oriented
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••
Treatment optionsTreatment options––
Surgical resectionSurgical resection
––
SclerotherapySclerotherapy
http://vascular.surgery.duke.edu
••
Treatment optionsTreatment options––
Surgical resection:Surgical resection:
•• Most effective for Most effective for encapsulatedencapsulated
and and microvascularmicrovascular
lesionslesions
• Diffuse, deep, and macrovascular
lesions are not amenable to surgical excision d/t
risk of
hemorrhage and damage to vital structures
http://vascular.surgery.duke.edu
••
Treatment optionsTreatment options––
Sclerotherapy:Sclerotherapy:
A) LiquidA) Liquid––
EthanolEthanol
B) FoamB) Foam––
PolidocanolPolidocanol
((AethoxysklerolAethoxysklerol®®))––
Sodium Sodium TetradecylTetradecyl
Sulfate (Sulfate (SotradecolSotradecol®®))
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••
Ethanol Ethanol sclerotherapysclerotherapy::–
Limitations:
• Use in pediatric patients • General anesthesia required for
all patients
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••
Ethanol Ethanol sclerotherapysclerotherapy
adverse effects:adverse effects:• EtOH
toxicity
• Severe pain• Ulceration and necrosis at injection site• Ischemic bullae• DVT and PE• Peripheral nerve injury• Pulmonary hypertension
http://vascular.surgery.duke.edu
••
Ethanol Ethanol sclerotherapysclerotherapy::–
Numerous side effects:
•• BB Lee et al Journal of Vascular SurgeryBB Lee et al Journal of Vascular Surgery
March March 20032003
––
87 patients / 399 sessions of ethanol 87 patients / 399 sessions of ethanol sclerotherapysclerotherapy––
51 acute and 7 chronic complications51 acute and 7 chronic complications
http://vascular.surgery.duke.edu
••
Foam Foam sclerotherapysclerotherapy–
Sodium Tetradecyl
Sulfate (USA)
• Used widely in US and Europe in the treatment of varicose veins and superficial reflux
• Not previously applied to the treatment of VMs–
Polidocanol
(Europe)
• has shown benefit in the treatment of VM but is not FDA approved in US
http://vascular.surgery.duke.edu
““Sodium Sodium TetradecylTetradecyl
Sulfate foam Sulfate foam sclerotherapysclerotherapy may be an effective alternative to may be an effective alternative to polidocanolpolidocanol in the treatment of LFVMin the treatment of LFVM55””
http://vascular.surgery.duke.edu
5. Markovic
JN, Shortell CK. Initial experience in the treatment of low flow vascular malformations with sodium tetradecyl
sulfate foam sclerotherapy. Presented at SVS Vascular Annual Meeting, San Diego June 2008
http://vascular.surgery.duke.edu
5. Markovic
JN, Shortell CK. Initial experience in the treatment of low flow vascular malformations with sodium tetradecyl
sulfate foam sclerotherapy. Presented at SVS Vascular Annual Meeting, San Diego June 2008
••
Foam Foam sclerotherapysclerotherapy––
Offers best visualization under USOffers best visualization under US
––
Prevents Prevents sclerosantsclerosant
dilution by dilution by intralesionalintralesional bloodblood
––
Maximizes endothelial exposureMaximizes endothelial exposure
http://vascular.surgery.duke.edu
••
Foam Foam sclerotherapysclerotherapy–
Mechanism of vascular obliteration
• Disruption of endothelial cell membrane• Vasospasm due to exposure of subendothelial
collagen to sclerosant• Platelet aggregation, inflammation and subsequent
endofibrosis• Ideally a sclerotic, not thrombotic
occlusion
http://vascular.surgery.duke.edu
http://vascular.surgery.duke.edu
••
Foam Foam sclerotherapysclerotherapy
–
Effectively used for varicose veins, telangiectasias
and
recently VMs–
Expce
primarily European
using polidocanol–
Duke experience the first reported use of STS for VMs
••
Foam Foam sclerotherapysclerotherapy–
Treatment in the office settingoffice setting
under local local
anesthesiaanesthesia–
General anesthesia for pediatric patients
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••
Foam Foam sclerotherapysclerotherapy: ultrasound guidance: ultrasound guidance––
When accessing deeper/nonWhen accessing deeper/non--visualized vesselsvisualized vessels
––
To control foam delivery preventing entry into To control foam delivery preventing entry into major venous structures through communicating major venous structures through communicating vesselsvessels
http://vascular.surgery.duke.edu
••
Foam Foam sclerotherapysclerotherapy: : Visual guidanceVisual guidance–
for visible subdermal
malformations w/o deep
communications
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http://vascular.surgery.duke.edu
••
Clinical case 1Clinical case 1–
53 year old male with long history of left face and left ear vascular malformation
–
Diagnosis:
Low flow (venous) vascular malformation
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••
Clinical case 1Clinical case 1–
Initial treatments:
surgical resections over 20 years
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••
Clinical case 1Clinical case 1–
Subsequent treatment:
STS Foam
Sclerotherapy –
Outpatient; visual guidance
–
Goals set primarily for:
•
cosmesis•
pain relief
http://vascular.surgery.duke.edu
••
Clinical case 1Clinical case 1–
Outcome:
symptoms improved after the treatment
–
No complications
Before STS FS After 3
STS FSAfter 7
STS FS
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••
Clinical case 2Clinical case 2––
13 year old female c/o 13 year old female c/o right leg swelling, right leg swelling, discoloration, discoloration, varicosities and varicosities and severe discomfort severe discomfort with exertion. with exertion.
––
Lesion present from Lesion present from birth but progressivebirth but progressive
––
Referred by Referred by dermatologistdermatologist
http://vascular.surgery.duke.edu
••
Clinical case 2Clinical case 2–
Diagnosis:
Klippel-
Trenaunay
Syndrome–
Venous malformation on the right anteromedial
thigh
–
Initial treatment: Ethanol Sclerotherapy
–
Complicated by popliteal
DVT
http://vascular.surgery.duke.edu
••
Clinical case 2Clinical case 2––
Subsequent therapy: STS foam Subsequent therapy: STS foam sclerotherapysclerotherapy
––
Goals preset as:Goals preset as:••
Decreased painDecreased pain••
Decreased swellingDecreased swelling••
Improved Improved cosmesiscosmesis••
Increased mobilityIncreased mobility–
General anesthesia due to age
••
US GuidanceUS Guidance••
TessariTessari
method of mixingmethod of mixing•
Percutaneous injection under US surveillance
•
Displaces intralesional
blood better
•
Leg elevation•
Compression wrappings
http://vascular.surgery.duke.edu
Tessari
L, Cavezzi
A, Frullini
A. Preliminary experience with a new sclerosing
foam in the treatment of varicose veins. Dermatol
Surg
2001;27:58-60.
••
Clinical case 2Clinical case 2
http://vascular.surgery.duke.edu
••
Clinical case 2 Clinical case 2 ––
OutcomeOutcome
Before STS FS After 4 STS FS
http://vascular.surgery.duke.edu
••
Clinical case 2 Clinical case 2 ––
OutcomeOutcome
––
100% goal achieved after 7 treatments100% goal achieved after 7 treatments––
No complicationsNo complications
––
No side effectsNo side effects
http://vascular.surgery.duke.edu
••
Clinical case 3Clinical case 3–
7 yo
male with right
leg pain, varicosities, discoloration, decreased mobility, bleeding since birth
–
Diagnosis: Klippel- Trenaunay
Syndrome
http://vascular.surgery.duke.edu
••
Clinical case 3Clinical case 3–
Initial treatment: Ethanol sclerotherapy
(outside
facility)–
Subsequent treatment: STS foam sclerotherapy
–
Goals set at:•
increased mobility•
decreased bleeding risk @ hockey
http://vascular.surgery.duke.edu
••
Clinical case 3Clinical case 3Combination of therapies as with
prior case
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••
Clinical case 3 Clinical case 3 outcome: outcome:
••
Symptoms resolvedSymptoms resolved••
Able to play hockeyAble to play hockey
••
Socially comfortableSocially comfortable••
No complicationsNo complications 5 Treatments
SUMMARYSUMMARY
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VASCULAR MALFORMATIONS ARE VASCULAR MALFORMATIONS ARE NOT HEMANGIOMASNOT HEMANGIOMAS
http://vascular.surgery.duke.edu
DIFFERENTIATING BETWEEN HIGH DIFFERENTIATING BETWEEN HIGH FLOW AND LOW FLOW LESIONS IS VITAL FLOW AND LOW FLOW LESIONS IS VITAL
TO DIAGNOSIS AND THERAPY OF TO DIAGNOSIS AND THERAPY OF VMsVMs
MOST MOST VMsVMs
ARE PALLIATED, NOT CURED ARE PALLIATED, NOT CURED AND REQUIRE MULTIPLE TREATMENTSAND REQUIRE MULTIPLE TREATMENTS
THE MULTITHE MULTI--DISCIPLINARY APPROACH DISCIPLINARY APPROACH IMPROVES CARE AND PATIENT IMPROVES CARE AND PATIENT
EXPERIENCEEXPERIENCE
Thanks to Jovan Markovic
MD, who helped prepare these slides
Thank YouThank You
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Questions?Questions?
Duke Multidisciplinary Vascular Malformation Team 2009Duke Multidisciplinary Vascular Malformation Team 2009