Arteriovenous Fistulas and Malformations Clinical Presentation

Updated: Nov 15, 2021
  • Author: Sateesh C Babu, MD; Chief Editor: Vincent Lopez Rowe, MD, FACS  more...
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Cutaneous malformations can present with a mass, pink stain, dilated veins, unequal limb length and girth, or skin ulceration. (See the image below.)

Buttock port-wine stain. Buttock port-wine stain.

Patients may experience limb heaviness that is aggravated with dependency and relieved with elevation. One half of patients experience pain. [13] The pain may be caused by tissue ischemia or by mass effect on local nerves. Some lesions, such as glomuvenous malformations, can be tender to palpation. [14]

The increased blood flow to the limb in congenital arteriovenous malformation (AVM) or arteriovenous fistula (AVF) may result in increased growth of the limb (ie, one leg may be larger and longer than the other). [13] In acquired AVFs, a history of trauma (eg, gunshot wound, stab wound, or even blunt trauma and fractures) can exist. AVF can also occur after medical diagnostic or interventional procedures (eg, angiography) or even after operative procedures that have caused inadvertent trauma to the artery and vein.


Physical Examination

Small AVFs and AVMs may be totally asymptomatic and may be discovered incidentally. Large AVFs may present with increased size of the limb, mild discoloration, swelling, or prominent veins with audible murmur or palpable thrill. (See the images below.)

Lower extremity venous malformation. Lower extremity venous malformation.
Upper extremity arteriovenous malformation (AVM). Upper extremity arteriovenous malformation (AVM).

The lesion may be pulsatile. The Branham sign may be present (slowing of the heart rate upon compression proximal to the AVM). Patients may develop hyperhidrosis, hypertrichosis, hyperthermia, or a palpable thrill or bruit over the lesion. They may have functional impairment of limbs or joints from mass effect or gangrene from prolonged tissue ischemia. Visceral AVMs can present with hematuria, hematemesis, hemoptysis, or melena.

Rarely, patients present initially with signs of congestive heart failure (eg, dyspnea, leg edema). This is particularly common when the communication is between a very large artery and a vein.



Except for very small AVFs, all acquired AVFs must be treated to prevent complications of distal limb ischemia, continued large flow of blood with eventual heart failure, and rarely infection (eg, endocarditis). Recurrence is a complication of inadequate or incomplete treatment.