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Most patients with carotid artery fibrodysplasia are likely to be asymptomatic, although the number reported without symptoms is small because most reported series are surgical experiences encompassing more advanced disease. Complications of this disease include embolization, dissections, and rupture with formation of an arteriovenous fistula. The most catastrophic clinical complication is stroke, and prophylactic interventions in asymptomatic patients are directed at reducing or eliminating this sequela of the disease. The risk of stroke or other related neurologic events varies widely and is unpredictable. Once clinical manifestations of this entity have arisen, operative intervention appears justified.
Open surgical therapy for extracranial internal carotid artery fibrodysplasia includes resection of the diseased vessel with interposition grafting, angioplasty with patch grafts for focal lesions, and graduated intraluminal dilation. Operative exposure of the carotid vessel in these cases usually requires dissection of the entire internal carotid artery to within a few centimeters of the base of the skull ( Figure 1 ). Care must be used not to cause injury to cranial nerves IX, X, XI, and XII.
Extended exposure of the internal carotid artery at the upper cervical levels may be facilitated by subluxation of the mandible. In the author’s experience, intermittent traction on the mandible, with an external clamp inserted into the angle of the mandible, facilitates the subluxation. Others have advocated fixed traction with placement of arch bars, as well as transection of the mandibular ramus.
Patients undergoing open procedures are placed on preoperative antiplatelet agents. They are systemically anticoagulated intraoperatively with intravenous heparin before the carotid vessels are occluded, and unless incessant bleeding is present after restoring antegrade carotid flow, the heparin effect is not reversed.
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