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  • • Congenital or acquired

    • Abnormal connection between artery and vein; located anywhere in body

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Congenital

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  • • Systemic effect minimal

    • Noted in infancy or childhood

    • Limb involvement leads to hypertrophied, longer extremity

    • Frequently involve brain, viscera, lungs

    Osler-Weber-Rendu syndrome (autosomal dominant): Hemorrhages in form of epistaxis, GI bleeding, polycythemia, cyanosis, clubbing

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Acquired

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  • • Enlarge rapidly

    • Can cause heart failure

    • Generally, result from trauma or disease

    Venous malformations: Rarely cause hemodynamic effects

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Epidemiology

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Acquired

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  • • Most commonly a result of traumatic injury

    • Iatrogenic injury after angiography or angioplasty common

    • Connective tissue disorders (Ehlers-Danlos), erosion of mycotic aneurysm, communication with prosthetic graft, neoplastic invasion can cause false aneurysm and arteriovenous fistula

    • Rare cause is injury to aorta and inferior vena cava after excision of herniated disk

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Symptoms and Signs

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  • • Determine time of onset and presence of associated disease

    • Continuous murmur may be heard

    • Palpable thrill and increased skin temperature

    • Proximal vein dilatation, distal pulse diminished, distal coolness

    Tachycardia: Increased cardiac output

    Branham sign: Compression of fistula results in slowed heart rate

    Venous malformations: Mass; may be tender; no hemodynamic effects

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Imaging Findings

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  • • MRI study of choice for peripheral AV malformations

    • Angiograms give precise delineation of AV fistulas

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  • • Evaluate for signs of systemic or distant intravascular infection

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  • • Physical exam

    • Duplex US or MRI for most peripheral lesions

    • Occasionally, use angiography to delineate further

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  • • Not all require treatment

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Venous Fistulas

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  • • Compression garments when possible

    • Injection of sclerosing agents possible

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AV Fistulas

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  • • Monitor small peripheral fistulas

    • Most managed with radiographic embolization; head and neck, pelvis best

    Surgical: Ligate all feeding vessels; amputate extremity; repair of fistula, oversewing defects (avoid for congenital fistulas)

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Congenital

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  • • En bloc resection of all tissue involved in fistula

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Surgery

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Indications

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  • • Hemorrhage

    • Expanding false aneurysm

    • Severe venous or arterial insufficiency

    • Cosmesis

    • Heart failure

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Contraindications

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  • • Congenital fistula (relative)

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Prognosis

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  • • Vary according to extent, location, and type

    • Traumatic type has better prognosis

    • Congenital has worse prognosis because of high recurrence

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References

Jacobowitz GR et al. Transcatheter embolization of complex pelvic vascular malformations: results and long-term follow-up. J Vasc Surg. 2001;33:51.  [PubMed: 11137923]
White RI Jr et al. Long-term outcome of embolotherapy and surgery for high-flow extremity arteriovenous maformations. J Vasc Interv Radiol. 2000;11:1285.  [PubMed: 11099238]
Mattle HP et ...

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