298 Case Report Authors R. Kanazawa, S. Ishihara, M. Okawara, H. Ishihara, S. Kohyama, F. Yamane Affiliation Division of Endovascular Neurosurgery, International Medical Center, Saitama Medical University, Saitama, Japan Key words 䉴 carotid arterial stenting 䊉 䉴 persistent primitive 䊉 hypoglossal artery 䉴 cervical internal carotid 䊉 artery stenosis Abstract & A 68-year-old male presented with a syncopal attack subsequent to acute myocardial infarction. His ultrasonographic and radiological examination revealed severe left internal carotid artery (ICA) stenosis and the presence of a persistent primitive hypoglossal artery (PPHA) immediately distal to the stenosis. The bilateral anterior and left middle cerebral arteries, and the vertebrobasilar system were opacified via the stenotic ICA. Carotid arterial stenting was selected as the treatment method because the lesion was high Introduction & Bibliography DOI 10.1055/s-0028-1082299 Minim Invas Neurosurg 2008; 51: 298–302 © Georg Thieme Verlag KG Stuttgart · New York ISSN 0946-7211 Correspondence R. Kanazawa, MD, PhD Division of Endovascular Neurosurgery International Medical Center Saitama Medical University 1397-1 Yamane Hidaka Saitama 350-1298 Japan Tel.: + 81/42/984 41 00 ext 91 40 jpn2412@saitama-med.ac.jp or ryu@nms.ac.jp Persistent primitive hypoglossal artery (PPHA) is one of the rudimentary vessels forming the carotid-basilar anastomosis in a fetus. A PPHA is usually observed incidentally while performing carotid angiography and its estimated incidence is from 0.05 or 0.02–0.026 % [1–8]. We treated a patient with a PPHA who experienced ischemic attacks of the vertebrobasilar arterial system and had an ipsilateral cervical internal carotid artery (ICA) stenosis. Case Report & A 68-year-old male had complained of loss of consciousness subsequent to an acute myocardial infarction (AMI), and was hospitalized at another hospital for treatment. During his hospitalization, an ultrasonographic examination revealed left cervical ICA stenosis and cerebral angiograms showed an ipsilateral PPHA. His past medical and familial history was unremarkable except for diabetes mellitus. After these examinations and treatment of the AMI, he was transferred to our institution. and a shunt placement during carotid endarterectomy was considered to be technically difficult. A self-expanding stent was successfully deployed with flow control, and the patient was discharged six days after surgery without any neurological deficit. There are sixteen reported cases including ours of PPHA associated with ICA stenosis presenting with ischemic attacks of the vertebrobasilar system. To the best of our knowledge, the current case is the first report of a cervical ICA stenosis with ipsilateral carotidbasilar anastomosis treated with carotid arterial stenting. Neurological and physical examination on admission showed no abnormalities. Cerebral angiograms showed severe left ICA stenosis and divergence of the PPHA immediately distal to the stenosis (䊉䉴 Figs. 1, 2). The right-sided horizontal portion of the anterior cerebral artery (ACA) and the bilateral vertebral arteries exhibited hypoplasia; the posterior communicating arteries could not be visualized. Therefore, the bilateral ACA, left middle cerebral artery, and vertebrobasilar system were mainly opacified via the stenotic ICA. There was no sign of a contralateral collateral circulation. Carotid arterial stenting (CAS) was chosen over carotid endarterectomy (CEA) because the lesion was observed at a higher position than usual and shunt placement was considered not to be easy. In this case, double shunts or a shunt between the common carotid artery (CCA) and the portion of the ICA proximal to the bifurcation of the PPHA would be needed, which was considered to be difficult. CAS was performed using modified Parodi’s methods [9] (䊉䉴 Fig. 3). Stenting devices were introduced using the GuardWire plus System (Medtronic, Minneapolis, USA) and were manipulated through a 9-F PATLIVE, a guiding catheter with an occlusive balloon (Clinical Supply, Gifu, Kanazawa R et al. A Successful Treatment with Carotid Stenting … Minim Invas Neurosurg 2008; 51: 298–302 Downloaded by: National University of Singapore. Copyrighted material. A Successful Treatment with Carotid Arterial Stenting for Symptomatic Internal Carotid Artery Severe Stenosis with Ipsilateral Persistent Primitive Hypoglossal Artery: Case Report and Review of the Literature Case Report 299 Fig. 2 Bone image CT showing the artery passing through the hypoglossal canal, which confirmed the diagnosis (arrows). Fig. 3 Set-up of the procedure: Stenting devices introduced using the GuardWire plus System (white arrow) were manipulated through the 9-F PATLIVE (large black arrow), and a HyperForm balloon catheter (arrowhead) was inserted in the ECA through the 5-F Guider (thin arrow). Two guiding catheters were placed in the left CCA with the 5-F Guider set proximal to the balloon of the 9-F PATLIVE. The occlusion of the left CCA and PPHA with these two balloon catheters resulted in a flow arrest of the left ICA for preventing distal embolism. Fig. 4 Postoperative left carotid angiography: Left: Live image. Right: Subtraction image. Stenosis was restored. The stent was placed between the ICA origin and the divergence (white area showing the extent). Japan). A HyperForm balloon catheter (Medtronic) was inserted in the external carotid artery (ECA) through a 5-F Guider (Boston & Scientific, MA, USA) (䊉䉴 Fig. 3). To achieve flow reversal in one direction in the left ICA, clumping of the ECA, CCA, and PPHA was initiated with HyperForm, PATLIVE, and GuardWire. The GuardWire was used to avoid embolic complications in the PPHA in case of a possible latent anastomosis, which would result in an incomplete flow arrest. The balloons and stent were selected with respect to the diameters of the CCA and normal ICA. Each step was performed with the HyperForm inflation being maintained. Two guiding catheters were placed in the left CCA with the 5-F Guider was set proximal to the balloon of the 9-F PATLIVE (䊉䉴 Fig. 3). While these three arteries were being occluded with these devices, which resulted in a feeble reverse flow in the left ICA, pre-dilatation using a 4 mm × 2 cm balloon catheter, Amiia (Cordis, FL, USA) and the deployment of a self-expanding stent, Precise, (10 mm × 3 cm; Cordis, FL, USA) were performed within about six minutes under GuardWire inflation in the PPHA (䊉䉴 Fig. 4). After a while, post-dilatation (Amiia, Cordis) was initiated under the reverse flow condition within two minutes. The accumulated blood was suctioned and dumped through the 9-F PATLIVE in each step. Although the stump pressure of the ICA measured during cross-clamping of the CCA and PPHA was 30 mmHg, CAS was performed without any significant changes in regional cerebral oxygen saturation. The patient’s postoperative clinical course was good. Postoperative magnetic resonance imaging showed no ischemic complications Kanazawa R et al. A Successful Treatment with Carotid Stenting … Minim Invas Neurosurg 2008; 51: 298–302 Downloaded by: National University of Singapore. Copyrighted material. Fig. 1 Preoperative left carotid angiography. Left: subtraction image. Cerebral angiography showed severe left ICA stenosis (arrow) and divergence of the PPHA immediately distal to the stenosis (arrowhead). The white arrow shows the distal ICA after the divergence. Middle: Live image. The lesion extended from the middle of the C-2 body to the lower border of the C-3 (black arrow showing the extent). Right: 3D image. The lesion was about 2.5 cm long. Both the CCA and ICA (before the divergence), had a diameter of 9.8 mm. The profile of the balloon and stent was selected based on this information. 300 Case Report Fig. 5 Postoperative MRI: Postoperative MRI (diffusion-weighted image) showing no ischemic complications. Table 1 Summary of previously reported cases of other primitive arteries with ipsilateral ICA stenosis. Sixteen prior patients including ours with PPHA associated with ICA stenosis presented with ischemic attacks of the vertebrobasilar system. CEA was performed in 13 cases. Eight PTA cases and 6 PIA cases have been reported. Age Sex Side Symptoms Primitive Treatment Vertebral artery pcom. not described R: absent L: hypoplasia R: absent, L: hypoplasia absent L: not visualized, R: not described L: absent, R: hypoplasia R: absent, L: PICA end R: PICA end, L: absent not described not described not visualized PHA CEA PIA (type1) CEA hypoplasia hypoplasia not visualized not visualized PTA CEA R: absent, L: normal absent hypoplasia R: adult, L: fetal not described suppling only PCA not visualized not visualized not visualized fetal type not described not described not described not described absent not described Bertoletti et al. 2000 Bour et al. 1991 72 58 F M R R asymptomatic asymptomatic PHA PIA CEA PTA Cartier 1995 74 F R asymptomatic PHA CEA Conforto et al. 2007 Fantini et al. 1994 66 67 M M R L PHA PHA medication CEA 62 F L PHA CEA Grego et al. 2004 78 F L visual disturbance dizziness, vertigo, R.hemiparesis R. hemiparesis, dysarthria, diplopia syncope, vertigo PIA CEA Hatayama et al. 1999 71 F L vertigo PHA CEA Heeney et al. 1980 65 F L PTA CEA Katoh et al. 1999 Kolbinger et al. 1993 42 66 F M L R Lewis et al. 1983 73 F L McCartney et al. 1989 Momma et al. 1992 76 47 F M R L temporary blind, dizziness syncope, vertigo R. cerebellar, thalamus, parietal weakness of the R. hand R. blind R. hemiparesis PHA PTA CEA medication Mori et al. 1998 Ouriel et al. 1988 65 not shown not shown not shown not shown not shown not shown 61 41 56 M not shown not shown not shown not shown not shown not shown M F M R not shown not shown not shown not shown not shown not shown L L R syncope VBI retinal ischemia TIA TIA TIA TIA R. numbness, diplopia R. hemiparesis/TIA L. hemiparesis PIA PHA PIA PTA PTA PTA PTA PHA PHA – PTA CEA CEA CEA CEA CEA CEA CEA CEA medication Stern et al. 1978 57 F R PHA CEA Sunada et al. 1991 Sutherland et al. 1972 68 62 50 F M M R R R vertigo, nausea, headache vertigo, visual blurring L. hemiparesis L. hemiparesis PTA PHA PHA Tanaka et al. 1987 69 M R Thayer et al. 2005 55 F The current case 68 M Pinkerton et al. 1980 Rodan et al. 1985 PIA R vertigo, diplopia, nausea asymptomatic CEA CEA not described CEA PHA CEA L syncope PHA CAS absent hypoplasia absent stenotic or aplastic stenotic or aplastic stenotic or aplastic normal PICA end absent R: hypoplasia, L: normal not described absent not described not described absent poor not visualized not described not visualized not visualized hypoplasia hypoplasia not visualized not described not visualized R: normal, L: absent R: hypoplasia, L: absent bil. absent fetal type not described not visualized PHA: primitive hypoglossal artery; PIA: proatlantal intersegmental artery; PTA: primitive trigeminal artery; CEA: carotid endarterectomy; PTA: percutaneous transluminal angioplasty; CAS: carotid arterial stenting Kanazawa R et al. A Successful Treatment with Carotid Stenting … Minim Invas Neurosurg 2008; 51: 298–302 Downloaded by: National University of Singapore. Copyrighted material. artery Case Report 301 Discussion & The criteria for diagnosis of PPHA are that 1) the artery arises from the cervical ICA at the levels C-1 to C-3, 2) the artery passes through the hypoglossal canal to the posterior cranial fossa, 3) the basilar artery is filled only distal to the point of junction with the anastomosis, and 4) angiography indicates a deficiency or absence of the posterior communicating artery [1, 2, 4, 7, 10, 11, 12]. The second criterion is important for differentiating a proatlantal intersegmental artery from a PPHA [7]. The current case satisfied these criteria. In addition, bone image computed tomography showed that the artery passed through the hypoglossal canal, which confirmed the diagnosis [2] (䊉䉴 Fig. 2). The incidence of posterior fossa ischemic symptoms associated with persistent carotid-basilar anastomosis may be higher than previously thought [2]. The vertebral arteries in patients with carotid-basilar anastomosis tend to be hypoplastic or aplastic, as in the current case. The posterior circulation is maintained mainly by the persistent carotid-basilar anastomosis. Under such specific circulatory conditions, ICA stenosis may easily cause ischemia in the vertebrobasilar system. In our case, a hemodymamic insufficiency of the vertebro-basilar system and left anterior circulation subsequent to AMI associated with ICA stenosis may have caused the loss of consciousness. We planned the treatment because we thought that aggravation of the stenosis would lead to a catastrophic outcome. Sixteen previously reported cases including the current case with a PPHA associated with ICA stenosis presented with ischemic attacks of the vertebrobasilar system [1–4, 6–8, 10–15]. In 13 cases, CEA was performed to prevent possible future ischemic attacks or to avoid recurrent attacks [2–4, 6–8, 10–15]. No previous cases treated with CAS for ICA stenosis associated with ipsilateral simultaneous primitive artery have been reported. CAS may be a reasonable treatment option for preventing further ischemic attacks in the posterior circulation. It may also have some advantages over CEA as described below: § The location of the occlusion balloons can be seen by X-ray imaging, which is a safer, surer, and easier step. In CEA, a shunt might be necessary, but its placement is not easy. Although shunt placement between the CCA and ICA just proximal to the bifurcation [2, 4, 8, 11, 16] and between the CCA and primitive artery [6, 7], and double shunts from the CCA to ICA and PPHA [17] have been reported, attentive manipulation may be essential and may not always be easy compared with balloon placement in CAS. § Pre- and post-stent angiography to examine the association among the lesion, important arteries, and various devices could be made available and more feasible. § The occlusion time can be shortened to within several minutes. However, the condition that the lesion was restricted between the ICA origin and the divergence of the PPHA was conducive to the success of the current case. If the lesion extends beyond the divergence, procedures such as CEA, percutaneous transluminal angioplasty with a balloon, or bypass surgery may be preferable. The previously reported cases of other primitive arteries with ipsilateral ICA stenosis are summarized in 䊉䉴 Table 1 [1, 2, 4–8, 10–23]. In many patients, surgical therapy was initiated as the treatment, and the rest received conservative therapy. Percutaneous balloon angioplasty was initiated in two proatlantal artery cases [18, 22]. To the best of our knowledge, the current case is the first report of cervical ICA stenosis with ipsilateral carotidbasilar anastomosis treated with CAS. Today, there are two options for the treatment of cervical ICA stenosis: a surgical approach and an interventional approach. Although the operative indication for patients should be decided on a case-by-case basis, this option should be taken into consideration for future patients. 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