Cervical Internal Carotid Artery Aneurysm: Spontaneous Dissection of the Cervical Internal Carotid Artery Resulting in Elongation and Pseudoaneurysm Formation Causing Hypoglossal Nerve Palsy; Endovascular Vessel Reconstruction with Stenting, Followed by Telescoping Flow Diversion, Achieving Straightening of the Artery, Aneurysm Occlusion, Hypoglossal Nerve Recovery, and Normalization of the Tongue
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A 48-year-old male patient, with a history of intermittent atrial fibrillation, endogenous depression, and Meulengracht syndrome, presented 2 days after the acute onset of a paralysis of the right-hand side of his tongue, causing dysarthria and difficulty swallowing. When he stuck out his tongue, it deviated to the right. Otherwise, his neurological condition was within normal limits, and the diagnosis of an acute hypoglossal nerve palsy was made. Magnetic resonance imaging and angiography (MRI, MRA), followed by diagnostic digital subtraction angiography (DSA), showed a 360° loop and a dissecting aneurysm in his right cervical internal carotid artery (ICA) just proximal to the petrous segment. The maximum neck and fundus diameters were 6 mm and 11 mm, respectively. Endovascular treatment was carried out, starting with balloon straightening of the ICA followed by deploying a self-expanding Carotid Wallstent (Boston Scientific) into the affected ICA segment. Follow-up examinations after 2 months showed a straightened ICA with persistent perfusion of the false ICA lumen and the dissecting aneurysm. A p64 flow diverter (phenox) was implanted inside the Carotid Wallstent. DSA 3 months later confirmed the occlusion of the pseudoaneurysm and the false lumen. The hypoglossal nerve palsy disappeared within 2 months after the first treatment in which the ICA had been straightened. Hypoglossal nerve palsy due to ICA dissection with pseudoaneurysm formation, including management of this condition, is the main topic of this chapter.
KeywordsCervical internal carotid artery Hypoglossal nerve palsy Dissecting pseudoaneurysm Carotid Wallstent Flow diversion p64
A 48-year-old male patient presented 2 days after the acute onset of a paralysis of the right-hand side of his tongue, causing dysarthria and difficult swallowing. His medical history was, apart from intermittent atrial fibrillation, endogenous depression, and Meulengracht syndrome, otherwise inconspicuous. Neurological examination showed that his tongue lolled to the right when stuck out, in line with an acute hypoglossal nerve palsy.
The symptoms displayed by the patient were due to the hypoglossal nerve being compressed by the aneurysm and the elongated ICA. The primary treatment goal was to recover the function of the hypoglossal nerve. Dissecting pseudoaneurysms of the ICA are a potential source of emboli to the dependent vasculature. The secondary treatment goal was to exclude the dissecting pseudoaneurysm from blood circulation.
Procedure #1, 25.01.2013: endovascular treatment of a dissected right-hand cervical ICA by means of balloon angioplasty and stent implantation
Anesthesia: general anesthesia; 1× 500 mg ASA (Aspirin i.v. 500 mg, Bayer Vital) IV, 1× 5,000 IU unfractionated heparin (1× Heparin-Natrium, B. Braun) IV, 7× 1 mg glyceryl trinitrate (Nitrolingual, Pohl Boskamp) IA
Premedication: 1× 500 mg ASA PO and 1× 600 mg clopidogrel (Plavix, Sanofi-Aventis) PO 1 day before the procedure; 1× 100 mg ASA PO and 1× 75 mg clopidogrel PO on the morning of the treatment day, 4 h before the procedure; response testing immediately before the procedure: Multiplate (Roche Diagnostic), area under curve (ARU): ADP 24, ASPI 9, TRAP 108, indicating that the Aspirin and clopidogrel were, as desired, significantly inhibiting dual platelet function
Access: right femoral 8F, puncture site later occluded with Angio-Seal (Terumo); diagnostic catheter: Tempo4 vertebral (Cordis); guide catheter: 8F Brite Tip 90 cm (Cordis); microcatheters: 1× Marathon, 2× Echelon14 45°, 1× Rebar27 (all then Covidien, now Medtronic); microguidewires: 1× Mirage 0.008″ (then Covidien, now Medtronic), 1× Traxcess 14 (MicroVention), 1× Traxcess 14 EX (MicroVention), 1× X-celerator 10 (Covidien), X-celerator14 (Covidien); 3x Hi-Torque All Star (Abbott Vascular)
Balloon catheters: 2× Ryujin Plus (Terumo) 4/40 mm; manometer (Medflator II, Smiths Medical)
Stents: 1× Carotid Wallstent (Boston Scientific) 5/30 mm (implanted); Coroflex Please (B. Braun) 4/28 mm (not insertable)
Duration: 1st–28th DSA run: 274 min; fluoroscopy time: 154 min
Postmedication: 1× 100 mg ASA PO daily for life, 1× 75 mg clopidogrel PO daily for 1 year; prolonged prescription of dual antiplatelet medication was chosen on the assumption that the “false lumen” created by the balloon angioplasty and stent implantation would need several months for complete endothelialization
The first treatment session had been partially successful. While the vessel had now been straightened, the false lumen and the pseudoaneurysm, both remnants of a previous dissection, were still perfused and considered a potential source of future emboli to the distal vasculature. The hypoglossal nerve palsy had resolved within 2 months after the first treatment. A much denser coverage of the false lumen was considered crucial. An offer to implant dedicated flow-diverting stents inside the Carotid Wallstent was accepted by the patient.
Procedure #2, 20.03.2013: endovascular treatment of a false lumen of the right-hand cervical ICA (a remnant of an earlier dissection) by implanting flow diverters inside a previously deployed Carotid Wallstent
Anesthesia: general anesthesia; 1× 500 mg ASA (Aspirin i.v. 500 mg, Bayer Vital) IV, 1× 5,000 IU unfractionated heparin (1× Heparin-Natrium, B. Braun) IV, 1× 1 mg glyceryl trinitrate (Nitrolingual, Pohl Boskamp) IA
Premedication: 1× 100 mg ASA PO daily and 1× 75 mg clopidogrel PO daily for the last 2 months; Multiplate (Roche Diagnostic), area under curve (ARU): ADP 7, ASPI 10, TRAP 93, indicating significant dual platelet function inhibition by the ASA and clopidogrel
Access: right femoral 6F, puncture site later occluded with Angio-Seal (Terumo); guide catheter: 6F Brite Tip 90 cm (Cordis); microcatheter: 1× Excelsior XT27 (Stryker); microguidewire: 1× Traxcess14 (MicroVention)
Flow diverter: 1x p64 (phenox) 5/24 (diameter too large, did not open properly and was therefore withdrawn); 1x p64 (phenox) 4.5/24 (implanted)
Duration: 1st–7th DSA run: 37 min; fluoroscopy time: 25 min
Postmedication: 1× 100 mg ASA PO daily for life, 1× 75 mg clopidogrel PO daily for 1 year
The hemiatrophy of the tongue resolved within 2 months after the first treatment. At the last consultation, 6.5 years after the first stent procedure, the patient was neurologically asymptomatic.
Internal carotid artery dissection is a recognized cause of ischemic stroke and cranial nerve palsy among young and middle-aged patients, accounting for up to 25% of all ischemic stroke cases in patients under age 45 (Bogousslavsky and Pierre 1992; Schievink 2001). Most ICA dissections occur spontaneously or are related to a minor trauma such as straining, sneezing, coughing, hiccups, or chiropractic manipulation of the neck. Less frequently, major trauma, such as a motor vehicle accident, is the cause. Other etiologies include arterial hypertension, connective tissue disorders like fibromuscular dysplasia, Marfan syndrome, type IV Ehlers-Danlos syndrome, alpha-1 antitrypsin deficiency, autosomal dominant polycystic kidney disease, osteogenesis imperfecta, internal carotid artery redundancy, estrogen-progesterone treatment, and infectious diseases (Campos-Herrera et al. 2008; Fusco and Harrigan 2011). In most cases, the extradural ICA is affected; however, rarely a dissection of the ICA may extend further distally and reach the intradural segment (Fusco and Harrigan 2011). There are two types of arterial wall dissections. The first is the subintimal type, which is more common and typically leads to arterial lumen narrowing causing the risk of a cerebrovascular ischemic event. The second type is subadventitial dissection, where the lumen and thus the flow rate as examined by ultrasound may remain normal.
The pathophysiology of the ICA dissection is thought to be due to a sequence of insults which lead to a compromise of the structural integrity of the arterial wall due to a tear in the tunica intima and secondary intramural hematoma formation and creation of a false lumen, often resulting in stenosis, occlusion, and intramural thrombus formation and distal emboli that may lead to ischemic stroke. The dissection may separate the tunica media from the tunica adventitia, resulting in aneurysmal dilatation of the affected vessel (Schievink 2001). Such a dilatation may result in compression of adjacent structures, including the hypoglossal nerve.
Spontaneous ICA dissections usually occur unilaterally; however, occasionally they may also occur synchronously on both sides (Woll et al. 2001) and even in combination with vertebral artery dissections.
Patients with ICA dissection usually present with headaches, ipsilateral facial pain, neck pain, ipsilateral Horner’s syndrome (when sympathetic fibers are affected), cerebral ischemia, TIA, amaurosis fugax, or even hemiparesis or hemiplegia. Less common presentations include bruit, which may be audible for the patient as pulsatile tinnitus and cranial nerve palsies. These symptoms are caused by different mechanisms following the dissection. They include ischemic events secondary to arterial stenosis, embolic events, antegrade propagation of the thrombus, or mass effect and pressure on the cranial nerves or the sympathetic chain.
Cranial nerve palsies as a clinical presentation of ICA dissection occur in 10–20% of cases (Baumgartner et al. 2001; Desfontaines and Despland 1995; Gobert et al. 1996; Mokri et al. 1996). The lower cranial nerves are those most often affected because of their vicinity to the ICA. ICA dissection was reported in 1.2% of the patients with hypoglossal nerve palsy (Stino et al. 2016).
A large series involving 190 patients found that cranial nerve palsy was present in 12% of the patients of extracranial ICA dissection (Mokri et al. 1996). The lower four cranial nerves were affected in 5% of the patients with hypoglossal nerve involvement. The hypoglossal nerve was the only cranial nerve involved in just three patients in this study 1.5% (Mokri et al. 1996; Olzowy et al. 2006).
Hypoglossal nerve palsy may cause mild dysphagia, difficulty in chewing, dysarthria, difficulty or inability to move the tongue, and tongue numbness and swelling. The clinical findings include fasciculation, atrophy, diminished mobility, and deviation toward the affected side when the tongue is protruded. On fiber-optic and mirror examination as well as on imaging, the tongue base may be prominent (Keane 1996).
While an isolated hypoglossal nerve palsy is a rare manifestation of a cervical ICA dissection, it may be the only manifestation of this disorder, like in our case.
The imaging appearance of a hypoglossal nerve palsy depends on the clinical phase. In the acute and early subacute phases, the side of the tongue affected may be edematous and swollen with “geographical” T1 hypointensity and T2 hyperintensity on MRI. There is often contrast enhancement, while in the late subacute and early chronic phase, the base of the tongue protrudes into the oropharyngeal lumen, potentially mimicking a tumor. In the late chronic phase, there is volume loss and fatty infiltration of the affected tongue side.
On imaging, an extracranial ICA dissection is characterized by mural hematoma with associated luminal narrowing. When the tunica media and tunica adventitia become separated, a pseudoaneurysm may develop at the site of the intimal tear. The characteristic appearance on MRI is seen on T1-weighted images with fat suppression as a crescentic T1 high signal within the vessel wall, representing the mural hematoma, with luminal narrowing.
Cranial CT scan is frequently the first-line imaging modality requested in patients with a suspected stroke. However, it is unlikely to detect an ICA dissection with CT. The diagnosis of an ICA dissection largely depends on the imaging techniques. A carotid Doppler test is frequently also requested for patients with a suspected stroke, and this may detect abnormal flow patterns in patients with ICA dissection (Schievink 2001). In patients with suspected ICA dissection, CTA/MRA should be considered (Olzowy et al. 2006). Digital subtraction angiography (DSA) has long been considered the gold standard for diagnosing ICA dissections. On DSA, the dissected artery frequently exhibits a beaded and threadlike appearance, irregular fan-shaped stenosis, and indirect signs such as pseudoaneurysm and venous phase contrast agent retention and direct signs like dual lumen appearance of two-way blood flow (Freilinger et al. 2010). In cases of suspected ICA dissection, CTA or MRA should be considered as these tests are noninvasive modalities and the combination of non-contrast non fat-suppression T1-weighted and fat-suppressed T1-weighted and T2-weighted MRI is the imaging modality of choice for identifying an intramural hematoma (Schievink 2001).
The natural history of ICA dissections is not completely understood and difficult to predict. Most cases have a benign course. Two-thirds of occlusions recanalize, and one-third of resulting aneurysms spontaneously decrease in size (Cohen et al. 2003; Houser et al. 1984). The healing process may take 2–3 months following the event.
Anticoagulation or antiaggregation over 3–6 months is the recommended treatment for patients with acute extracranial carotid artery dissections (Lyrer and Engelter 2004; Schievink 2000; Schievink et al. 1993) although no randomized controlled trial exists for the management of ICA dissections and the benefits of this treatment are unknown (Lyrer and Engelter 2004, 2010). Most ICA dissections heal spontaneously, and endovascular or surgical management is usually reserved for patients who have not responded appropriately to medical therapy. Endovascular treatment should be considered for patients with recurrent acute cerebral ischemia or progressive symptoms under adequate medical treatment. Cervical artery stenosis or occlusion caused by an intramural hematoma with poor collaterals, an expanding dissection and contraindications for anticoagulation might be arguments in favor of endovascular treatment (Biggs et al. 2004; Moon et al. 2017; Schievink 2001). Although conservative medical therapy is often used for pseudoaneurysms, occasionally, these pseudoaneurysms do not obliterate spontaneously and may instead cause symptoms due to thromboembolism or dilatation or blood flow compression (Chen et al. 2016). The Royal College of Physicians Stroke Guidelines support the use of anticoagulant or antiplatelet therapy in the treatment of ICA dissection (National Collaborating Centre for Chronic Conditions (UK) 2008), and the final decision is usually made by the attending physician. However, there is no evidence of any superiority of anticoagulant or antiplatelet therapy in preventing strokes after carotid and vertebral artery dissections (CADISS trial investigators et al. 2015). According to the literature, about 15–20% of the patients who receive appropriate medical therapy will suffer from a persisting neurological deficit (Anson and Crowell 1991). Surgery has been recommended for patients after the failure of medical treatment, should neurological symptoms worsen and the pseudoaneurysm progress. The surgical options include vessel ligature, resection with revascularization, or extra-intracranial bypass (Biggs et al. 2004; Gonzales-Portillo et al. 2002).
Endovascular treatment has been recommended during the past few years for the management of extracranial ICA dissections and pseudoaneurysms with promising results. Although there is no general consensus, endovascular therapy includes aneurysm coiling, stent-assisted coiling, and, more recently, flow-diverting stents for complex cases involving large pseudoaneurysms, especially after the failure of conservative medical management or a worsening of symptoms under adequate medical therapy.
An early patient was treated with endovascular stent-mediated repair for an acute ICA dissection by Matsuura et al. (1997). Recently, flow diverters have been used for the treatment of ICA dissections, especially for wide-necked and large pseudoaneurysms where straight coiling or stent-assisted coiling may fail to achieve complete occlusion or to decrease the mass effect of the pseudoaneurysm.
The use of flow diverters for extracranial carotid artery dissections was recently suggested by several authors (Chen et al. 2016; Kurre et al. 2016). The mode of action of these implants in treating pseudoaneurysms of the ICA is the same as in treating intracranial aneurysms. They allow the reconstruction of the damaged artery segment and divert the blood flow from the pseudoaneurysm toward the main blood vessel, leading to aneurysm thrombosis and shrinkage, as the clot becomes organized and reduces, thus resolving the mass effect caused by the aneurysm.
The removal of the 360° loop of the cervical ICA required a more complex maneuver and eventually was only possible by an iatrogenic dissection of the ICA with a coronary balloon catheter. The subsequently implanted Wallstent helped to stabilize and straighten the vessel and to avoid an elastic recoiling after withdrawing the balloon catheter. The effectiveness of this concept was confirmed by the rapid restitution of the hypoglossal nerve function.
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