The Case Files

Fig. 1.: A CTA of the neck demonstrating a short segment of dissection at the distal aspect of the right ICA proximal to the base of the skull.FigureFigureFigureFigureA 50-year-old woman presented with 20 hours of dysphagia and a muffled voice. She reported that she had had difficulty swallowing while eating dinner the previous evening. She had then felt persistent dysphagia with a changed voice upon waking that morning. She was evaluated at an urgent care center where it was noted that she had a blood pressure of 230/120 mm Hg, and she was sent to our ED out of concern for retropharyngeal abscess. She underwent an emergent stroke evaluation in the ED because of her hypertensive emergency with concurrent acute dysphagia. The patient's systolic blood pressure was initially between 205 and 232 mm Hg, and it was resistant to initial treatment with labetalol 10 mg IV and then enalaprilat 2.5 mg IV. A nicardipine infusion eventually brought her blood pressure down to 135/81 mm Hg. A so-called hot potato voice was noted, but her physical exam was otherwise unremarkable with a score of 0 on the National Institute of Health Stroke Scale (NIHSS). A computed tomography angiography of her head and a CTA brain perfusion were also unremarkable. A CTA of the patient's neck demonstrated bilateral distal internal carotid arteries (ICAs) consistent with fibromuscular dysplasia (FMD), a diagnosis of which she was unaware. A short segment of dissection was noted at the distal aspect of the right ICA proximal to the base of the patient's skull. (Fig. 1.) A linear structure at the distal aspect of the left ICA proximal to the skull base possibly representing a short segment of dissection was also noted. (Fig. 2.) The patient was given aspirin and then reinterviewed. She said she had not experienced any chiropractic changes, trauma, or headache and had not ingested a foreign body. She again had an unremarkable neurological exam aside from dysphagia, and her NIHSS score remained at 0. A Previous Strangulation Our neurologist and neurointerventionalist said she had no acute indication for anticoagulation, and she was admitted to the intensive care unit. She was noted on chart review to have had a recent renal ultrasound that demonstrated an atrophic left kidney and mildly elevated peak systolic velocity in her right renal artery. A CTA was ordered to rule out FMD of the renal arteries. The patient said during her ICU course that her ex-husband had tried to strangle her about eight weeks earlier; he had used enough force to make her lose her breath for a few seconds. The patient's blood pressure was closely monitored in the ICU, and she was started on dual antiplatelet therapy (aspirin 81 mg and clopidogrel 75 mg). Her nicardipine infusion was discontinued after her systolic blood pressure stabilized to between 130 and 140 mm Hg, and she was resumed on her medications of losartan 100 mg and amlodipine 10 mg with labetalol as needed if her systolic blood pressure was greater than 150 mm Hg. The patient was cleared for discharge, advised to follow up with her primary care physician, a vascular surgeon, and a neurologist within one week, and continue dual antiplatelet therapy.Fig. 2.: A CTA of the neck demonstrating a linear structure at the distal aspect of the left ICA proximal to the skull base, possibly representing a short segment of dissection.Traumatic CAD Carotid artery dissection (CAD) occurs when an artery's intimal wall is damaged. Blood then fills the layers of the arterial wall, and eventually thrombi can form and lead to ischemic disease. CAD has low incidence, but emergency physicians must still be vigilant about this differential diagnosis because timely diagnosis is paramount given the poor neurological outcomes that result without early intervention. (Emerg Med Pract. 2012;14[4]:1.) The common clinical manifestations of CAD generally include headache, neck pain, visual symptoms, dizziness, and focal neurological deficits. (J Neurol. 1995;242[4]:231.) Acute onset of dysphagia with voice changes is an uncommon presentation. Risk factors for CAD include smoking, hypertension, hypercholesterolemia, migraine headaches, contraceptive use, and trauma. (J Neurol. 1995;242[4]:231.) FMD, an arterial disease characterized by an abnormal configuration of the vessel walls, causing them to stenose or bulge, is a less common risk factor that requires more investigation. (Stroke. 2021;52[3]:821; https://bit.ly/3w0a9m5.) Traumatic CAD has been reported in cases of blunt head and neck trauma in addition to those resulting from the shearing force on the vessels during neck extension in a motor vehicle collision. (Br J Anaesth. 2000;85[3]:476; https://bit.ly/3bNkOtF; Turk J Pediatr. 2020;62[6]:1077.) Patients often present with the neurological sequelae of the dissection, including paresis, dysphasia, Horner syndrome, and obtundation. (J Neurosurg. 1989;71[6]:854.) Acute dysphagia with a change in voice is a fairly unusual presentation of CAD and may raise suspicion of an abscess in the deep spaces of the neck. (J Laryngol Otol. 1995;109[3]:252.) We were concerned, however, that she had experienced a cerebral ischemic event because of her symptoms and uncontrolled hypertension, so we obtained a CTA of her cervical arteries that demonstrated dissection of her bilateral ICAs and previously undiagnosed FMD. A Rare Condition Few studies have examined the relationship between CAD and FMD. A review of the current literature, however, suggested an association between these events. A case series of 44 consecutive patients with internal CAD sought to determine the risk factors and early signs associated with CAD. Six of the 44 patients were found to have FMD. (J Neurol. 1995;242[4]:231.) A recent retrospective analysis of 1283 patients with spontaneous cervical artery dissection found that 103 (8%) had coexisting FMD. The study concluded that coexisting FMD is an independent predictor of the risk of recurrent cervical artery dissection. (Stroke. 2021;52[3]:821; https://bit.ly/3w0a9m5.) A cross-sectional study examining 921 patients from the U.S. Registry for Fibromuscular Dysplasia found that 151 (16%) had experienced CAD. (J Am Coll Cardiol. 2016;68[2]:176; https://bit.ly/3bO5H2X.) FMD may compromise the affected vessels' structural integrity and predispose patients to vessel dissection following trauma, which was demonstrated by our patient who was found to have bilateral ICA dissection following strangulation. (Br J Anaesth. 2000;85[3]:476; https://bit.ly/3bNkOtF.) One of the rare causes of CAD is strangulation by domestic violence, which happens due to the manual compression of the ICA and can result in a delayed stroke. Most patients require antithrombotic therapy based on the extent of the dissection and their neurological symptoms. Other interventions include angioplasty to relieve stenosis, which improves cerebral perfusion and lowers the risk of an ischemic or embolic stroke. (J Neurosurg. 2000;92[3]:481.) Forced extension of the neck may also result in CAD because of the shear force that happens at the junction of the intrapetrous and extracranial segments of the ICAs. (Br J Anaesth. 2000;85[3]:476; https://bit.ly/3bNkOtF.) Disorders leading to dysphagia are generally caused by neuromuscular or varying types of structural disturbances of the oropharynx or esophagus. CAD is a possibility in patients presenting with dysphagia and an unclear clinical picture. Special care should be taken to note the possible diagnosis of CAD in patients presenting with dysphagia and undifferentiated persistent hypertension despite the lack of a formal FMD diagnosis. Post-ICU Treatment The goal in managing CAD is to prevent future adverse events like stroke. Our patient was treated with dual antiplatelet therapy and therapy to control her blood pressure. Guidelines from the American Heart Association/American Stroke Association state that patients who have experienced stroke or transient ischemic attack associated with CAD should receive antithrombotic treatment with antiplatelet or anticoagulant therapy for at least three to six months. (Stroke. 2021;52[7]:e364; https://bit.ly/3pdZvV1.) The efficacy of antiplatelet therapy compared with anticoagulant therapy is unclear in these patients. (Stroke. 2021;52[7]:e364; https://bit.ly/3pdZvV1.) Those treated with anticoagulation, however, are at risk of increased bleeding events including intracranial hemorrhage, which may offset the benefit of this treatment. The Canadian Stroke Consortium also performed a pilot study of 105 patients with cervical artery dissections; they found no significant difference in preventing TIA, stroke, or death following initial dissection between patients treated with antiplatelet therapy v. those treated with anticoagulation. (Stroke. 2003;34[12]:2856; https://bit.ly/3pgrun8.) Similarly, it was found in a retrospective study of 298 patients with spontaneous CAD that the incidence of ischemic and hemorrhagic events during follow-up did not significantly differ between patients treated with anticoagulation and patients treated with aspirin alone. (Neurology. 2009;72[21]:1810.) Current consensus maintains that blood pressure management, like antithrombotic treatment, is beneficial in cases of cervical artery dissection. The idea correlates to the recommendation by the AHA to lower the blood pressure to 140/90 mm Hg in aortic dissection. (J Thorac Dis. 2017;9[5]:1369; https://bit.ly/3w4I4dp.) The AHA/ASA, however, do not currently offer clear guidelines on blood pressure management in cases of CAD. They do note that the efficacy of pharmacologic therapy to lower blood pressure to normal range (with the idea of reducing arterial wall stress) is not well established in CAD. (Stroke. 2011;42[8]:e464; https://bit.ly/3AfJww7.) The results of one multicenter, prospective, hospital-based case control study suggested that a history of hypertension is significantly associated with an increased risk of spontaneous CAD compared with healthy controls. (J Neurol Neurosurg Psychiatry. 2006;77[1]:95; https://bit.ly/3JOCbH2.) The undeniable relationship between hypertension and CAD makes it necessary to conduct further research on this topic and create guidelines to manage blood pressure to optimize care. Underlying pathologies like FMD can predispose patients to CAD. Physicians need to be aware of the rare causes and presentations of CAD such as trauma and dysphagia. Prompt management is needed to help prevent the development of stroke and other devastating neurological sequelae, and it is important for EPs to keep a broad differential of ENT presentations in general because they may be neurological. Further research is necessary to establish a relationship between FMD and CAD, and clear guidelines on pharmacotherapy choices for antithrombotic and antihypertensive goals for CAD patients are also needed.

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