Vascular Surgery for Arteriovenous Malformations Clinical Presentation

Updated: Sep 09, 2016
  • Author: Allison Leigh Speer, MD; Chief Editor: Vincent Lopez Rowe, MD  more...
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Presentation

History

Arteriovenous malformations (AVMs) are commonly misdiagnosed in infancy and childhood as an involuting hemangioma or capillary malformation because the lesion is not yet fast-flow, warm, or pulsatile. [9] They may become more clinically evident during the second or third decade of life. [15, 12] Enjolras et al observed a progression during childhood to grade II in 84% of patients. [14] Puberty (32%), pregnancy (25% adult women), or trauma (20%) can trigger expansion. [5, 14, 1, 9] These lesions grow proportionately with the child and never regress.

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Physical Examination

The head and neck area is the most common location (70%) for AVMs, with a higher incidence of intracranial lesions than of extracranial lesions. Next in frequency are AVMs of the extremity, trunk, and viscera. [5, 1, 9]

When fully developed, AVMs deepen in color with increased erythema, and local warmth, a palpable thrill, and a bruit. Patients with facial AVMs of the skin or facial bones may present with facial asymmetry, gingival hypertrophy, unstable teeth, periodontal bleeding, or skin or mucosal ulcers with secondary infection. Nasal AVMs may cause epistaxis. Bony AVMs create osteolysis (3/200 in the series by Enjolras et al). [14]

Lower-limb skin changes resembling curious dry, brown-violaceous plaques may appear and are known histologically as pseudo-Kaposi sarcomas. [1, 9] Distal-extremity AVMs may lead to ischemia of the tips of fingers or toes associated with arterial steal and venous hypertension. (See the image below.)

Ischemic contractures secondary to a right hand ar Ischemic contractures secondary to a right hand arteriovenous malformation (AVM). Panel A: ventral. Panel B: dorsal. Panel C: excellent outcome after surgical resection/amputation.

Later consequences of expanding AVMs with arteriovenous shunting include ischemic changes, indolent ulceration, intractable pain, and sudden life-threatening hemorrhage or recurrent intermittent bleeding. [9] Increased cardiac output with subsequent congestive heart failure occurs in fewer than 2% of cases (5/200 in the series by Enjolras et al) [5] and usually in newborns with a massive AVM or in young adults with a large rapidly worsening AVMs in the limb or trunk. [1, 9]

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Complications

Complications that may be apparent before embolization/sclerotherapy or surgical excision include the following:

  • Dystrophic skin changes
  • Ulceration
  • Tissue necrosis
  • Hemorrhage
  • Intractable pain
  • Congestive heart failure
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