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Gilbert G. Florentino, MD
Makati Medical Center
1. Present a case of
arteriovenous malformation
2. Discuss the definition,
pathophysiology of
arteriovenous malformation
3. Discuss treatment options for
AV malformation
OBJECTIVES
A.F.
38/Male
Filipino
Easy fatigability
Shortness of breath for 7 months
Consulted
GENERAL DATA
13 years old
Edema , left arm
Developed varicosities
Dx: Hemangioma
No treatment done
No cardiac symptoms
History of present illness
26 years old progressive development of tortuous varicosities on Left upper arm
-graft prosthesis Left
subclavian artery
-noted improvement
post surgery
25 years old –gradual enlargement,
Left arm
multiple tortuous varicosities
Dx: AV Malformation left upper arm
- repair of AV malformation and ligation of feeding arteries
History of Present Illness
32 years old new varicosities on medial left arm with progressive dilatation, with extension to the left upper chest and neck
7 months PTC
still with varicosities on L arm
- (+) easy fatigability
- (+) shortness of breath
Patient was advised surgery
Patient consulted
History of Present Illness
REVIEW OF SYSTEMSGENERAL: (-) anorexia,(-) fever,(-) weight loss,
generalized body weakness, numbness; (-) loss of consciousness (-) headache; (-) loss of appetite
SKIN: (-) rash, hematoma, jaundice, abnormal pigmentation, itching
HEENT: (-) tinnitus, hearing loss, dryness of the mouth, dysphagia, eye pain; (-) blurring of vision
CARDIOVASCULAR: (-) chest pain; (-) orthopnea and(-)palpitations; (-) easy fatigability
REVIEW OF SYSTEMSGASTROINTESTINAL: (-) epigastric pain; (-) melena,
diarrhea, nausea, vomiting, urinary and bowel incontinence
GENITOURINARY: (-) dysuria, oliguria, hematuria, nocturia
ENDOCRINE: (-) polyuria, polydipsia, polyphagia, heat/cold intolerance
MUSCULOSKELETAL: (-) joint pains, tremors, weakness of the extremities, stiffening of extremities, back pain,(-)
PAST MEDICAL HISTORYNon hypertensive, non diabetic, non asthmatic
(+) diagnosed with Congenital AV malformation, left upper arm (1987)
S/P Ligation of Feeders left arm (1999)
S/P Graft prosthesis on Left Subclavianartery (2000)
S/P Inguinal herniorrhaphy, left (2004)
FAMILY HISTORY(+) Gastric Cancer – father
(+) Leukemia –mother
UNREMARKABLE
SOCIAL HISTORY
PHYSICAL EXAMINATION• Conscious, coherent, ambulatory,
not in cardiorespiratory distress
• BP 130/80 HR82 regular RR20 T 36.7
• Anicteric, pink conjunctivae, moist buccal mucosa, no tonsillopharyngeal congestion, no nasoaural
discharge,no CLAD; (+) multiple tortuous varicosities on the left neck extending to the left upper chest wall and left upper arm, (+) warm to touch, non-erythematous, non tender (+) thrill, (+)bruit
PHYSICAL EXAMINATION• Equal chest expansion, clear lungs
• Adynamic precordium, AB at the 6th LICS MCL, regular rhythm, no S3 or S4, (+) grade 3/6 systolic murmur at the LPSB, 4th ICS; (+)thrill on tortuous veins on the anterior chest wall, neck and left arm
• flat, soft, normoactive bowel sounds, non tender, no masses, no tenderness
• full equal pulses, no cyanosis, no edema
• Peripheral Arterial Disease
• Congenital Arteriovenous Malformation, Left upper arm, Left Anterior Chest and Neck
• Functional Class II-A
• S/P Ligation of Feeders left arm (1999)
• S/P Graft prosthesis on Left Subclavian artery (2000
• S/P Inguinal herniorrhaphy, left (2004)
IMPRESSION
CBC 3/9/12
Hgb 12.9
Hct 39.2
WBC 3.75
Seg 40
Lym 48
Mono 8
Eo 4
Plt 197,000
CBC (3/9/12)
3/9/12
Na 141
K 4.2
Creatinine 0.76
SERUM ELECTROLYTES (3/9/12)
PTT
Patient 29.7
Control 25.8
PT
Patient 12.3
Activity 84.6
Control 11.5
INR 1.07
COAGULATION PARAMETERS
SMALL OVOID DENSITY IN THE LEFT UPPER LOBE (could be due to a calcific granuloma or merely a blood vessel viewed en face).
Otherwise, normal.
CHEST X-RAY (3/9/12)
ECG (3/9/12)
CHEST CT ANGIOGRAPHY (Nov. 2011)
Normal CT examination of the lungs
Right atrial enlargement due to increased flow from AVM of the Left upper extremity.
Left ventricular enlargement.
No pericardial or pleural effusion.
No evident mediastinal, hilar or axillary lymphadenopathy.
Normal CT angiography of the thoracic aorta and pulmonary vessels.
No evident thoracic aortic aneurysm, wall dissection or pulmonary embolism.
CHEST CT ANGIOGRAPHY(Nov. 2011)
High flow arteriovenous fistula, arterial feeders immediately proximal and distal to the graft prosthesis, arising from the left distal subclavian/left proximal axillary and left distal axillary artery
Large draining venous channels presumably representing ectatic cephalic, brachial and axillary veins.
Non visualization of the left subclavian vein.
Venous extension with arterial blood into the anterior sternal area and left side of the neck.
CT ANGIOGRAPHY OF LEFT UPPER EXTREMITY
Dilated LV with normal wall motion and contractility
Normal LV Ejection Fraction 64% (Teicholz)
Dilated LA, w/o thrombus
RAE, RVE
Normal main pulmonary artery, aortic root and proximal ascending aorta
Normal MV, AV, TV, PV
No pericardial effusion
2DECHO (3/14/12)
Color Flow and Doppler Study:
Mild Mitral Regurgitation
Moderate Tricuspid Regurgitation
Normal pulmonary artery pressure 185msec by PAT
- Belong to Vascular Malformations
- Defects of the circulatory system that arise during embryonic or fetaldevelopment or soon after birth
- Masses of abnormal blood vessels
- Consist of a blood vessel “NIDUS” (NEST)
Through w/c arteries = veins
DEFINITION
- Mass covered by normal
color or angiomatous skin
- Smooth and shiny
- Increased local
temperature
- (+)fremitus and murmur
- tortuous
- Drainage veins
• MAIN LOCATIONS
o Head and neck 40%
o Extremities 40%
o Trunk 20%
CLINICAL CHARACTERISTICS
• Arise during early fetal development due to failure of regression of AV channels in the primitive retiform plexus
• Most occur sporadically
• ULTRASONOGRAPHY
Method of choice for initial evaluation
Demonstrates anechoic tubular structures w/o well-defined soft tissue masses
Doppler:
o Areas of arteriovenous shunt
o Increased peak systolic velocity
o Ectatic veins with arterialized flow
• MRI Areas with signal absence (flow void) on T1 and T2-
weighted sequences No mass adjacent to pathological vessels
• CT Multiple ectatic supplying arteries Early contrast-enhancement of the drainage veins
with no significant interposed mass
• ARTERIOGRAPHY Gold standard for AVM diagnosis Early contrast enhancement of venous structures (+) malformative nidus
• vascular malformations are responsive to stimuli (ex.injury and surgery), improper treatment rapidly stimulates quiescent vascular malformations –worsens the condition.
• Feoktistov et al
Hypoxia modulates the expression of adenosine receptors in human endothelial and smooth muscle cells toward an angiogenic phenotype
PRINCIPLES OF TREATMENT
• Only potential cure: COMPLETE DESTRUCTION OF THE NIDUS OF A VASCULAR MALFORMATION
• Ameliorating clinical symptoms is another goal for problematic vascular malformations
PRINCIPLES OF TREATMENT
• Surgical resection
• Laser therapy
• Direct puncture sclerotherapy
• Arterial embolization
PERIPHERAL VASCULAR MALFORMATION: TREATMENT
• Absolute indications for treatment:
• Progressive pain or discomfort
• Functional alterations impairing daily activities or quality life
• Severe deformity
• Vascular bone syndrome
• Hemorrhage
• High output heart failure
• Complications secondary to venous hypertension
• Life threatening lesions
PERIPHERAL VASCULAR MALFORMATION: TREATMENT
Percutaneous puncture of the lesion with a fineneedle attached to a small extension tube
Promotes:
• blood coagulation
• protein denaturation
• severe inflammatory process obliterationof the malformed cavities
Performed under fluoroscopic guidance
SCLEROTHERAPY
ABSOLUTE ETHANOL
Used for the treatment of surgicallyinaccesible lesions
ETHANOLAMINE OLEATE
Less effect on the deep vascular layer
Not associated with neurologic side effects
POLIDOCANOL
Used for sclerotherapy of esophageal and lower limb varices
SCLEROTHERAPY
BLEOMYCIN OK – 432 (Picibanil)
Prepared with Streptococcus pyogenes cells with benzylpenicillin
Utilized for the treatment of lymphatic malformations (macrocystic)
Direct puncture
Low complication rates
Used more frequently for lymphatic malformations
Diluted at a concentration of 1 mg/ml
Most common complications:• Cellulitus• Ulcerations• Hair loss• Cold – like symptoms
SCLEROTHERAPY
Transarterial or transvenous
Preferred treatment of high flow marformation
Solid embolizing agent: platinum metal coils with controlled detachment
Accessed by means of remote arterial puncture with application of liquid and solid particles
EMBOLIZATION
N – BUTYL- CYANOACRYLATE (NBCA) Ethylene vinyl alcohol copolymer (Onyx)
-Acts by precipitation occluding the vessel
-Radiopaque
-Non adhesive
-Long procedure time
-Treat high and low-flow malformations
-Lower rates of complications
-Utilized for treatment of high – flow vascular malformation ( Arterial Malformation)
-Acts by polymerizing when in contact with blood
EMBOLIZATION
Monitoring of the distal blood flow and nervesensation
CT scan, MRI and Color Doppler UTZ: done after 3days of sclerotherapy to evaluate the efficacy oftherapy
At least 2 years of follow up is recommended
FOLLOW UP
• Angiography and Embolization considered
• Purpose of Embolization:
Control growth of AVMs and bleeding
Use sufficient liquid embolizing agents to eradicate the nidus
Successful embolization:
Active bleeding stopped
Localized pulsation disappeared
Lesion lighter
Expanded veins in the neck return to normal
• Surgery indicated: Embolization fails or endovascular access of nidus is not possible
• Surgery difficult:
Vascularity
Lack of distinct margins
Involvement of major structures
• Commonly recur post-surgery
• GOAL OF SURGERY: resect entire nidus
• Lasers less effective with AVMs, rarely used
• Only cure: RADICAL RESECTION, possible only after a PREOPERATIVE ANGIOEMBOLIZATION
• If unresectable: ANGIOGENESIS-INHIBITING AGENTS
• Flow pattern of lesion
• Location (deep vs superficial)
• Operator experience
• Presence of adjunct surgery plan
• Cost
• availability
Main Factors that influence agent selection:
Superficial Low-flow PVM:
Ethanol sclerotherapy
NBCA embolization
Deep High-flow PVM:
NBCA and/or Onyx
Patients with planned adjunct surgery:
NBCA embolization
VEGF LEVELS IN CONGENITAL AVM?
UNKNOWN
ANGIOGENESIS-INHIBITING AGENTS
• Shunting and hypoxemia
• Aneurysm/rupture/bleeding/ hemorrhage
• Pulmonary Embolism
• Skin ulceration
• Cellulitis
• Gangrene
• Hyperpigmentation
• High cardiac output
• Lack of tissue perfusion
• Disfigurement
CLINICAL CONSEQUENCES OF AVM
AVM - SummaryAVMs are complicated vascular malformations which can grow in the brain, head and neck area, limbs, or even in organs.
An understanding of the functional vascular anatomy is critical to successful treatment.
Successful treatment requires a multi-disciplinary team that has constant interaction.
The combination of pre-surgery embolization followed by surgical resection is currently yielding the most successful results for head, neck and body lesions.