Porcelain Aorta and Quadruple Extracranial Vessel Occlusion: A Case of Minimal Neurological Deficits Despite Severe Vascular Blockages

Introduction

Cerebrovascular occlusion is a critical health concern that poses a significant threat to an individual’s life. It occurs when a specific area of the brain is deprived of adequate blood supply. Strokes, a leading cause of worldwide mortality, largely result from this insufficient blood flow, with approximately 85% being ischemic, indicating that they are a result of cerebrovascular occlusions [1,2]. Large vessel occlusion constitutes nearly 38% of acute ischemic stroke cases and is associated with serious and harmful outcomes for patients [3].

However, most stroke patients usually have a single vessel blockage, while about 10.7% of cases show as multiple large vessel occlusions [4]. As an illustration, the incidence of bilateral vertebral artery occlusion is infrequent, even when considering the existence of atherosclerotic lesions [5]. Certainly, while there are a few documented cases in the literature in which patients experienced occlusion in 2 extracranial arteries [6], occurrences of occlusions involving 4 extracranial vessels are exceptionally rare, as indicated by the scarcity of reports [7–9].

When there is occlusion in all 4 extracranial vessels, patients may not show any noticeable symptoms. This absence of overt signs or manifestations could pose a challenge in early detection or diagnosis [8]. In this case report, we present a patient with total occlusion of all large arteries supplying the brain, but he experienced only a few mild symptoms.

Case Report

The patient, a 65-year-old man, had no prior history of hypertension, diabetes mellitus, dyslipidemia, or known coronary artery disease. However, he had a significant smoking history, with a 30-pack-year habit. Until a week before his visit, he had been relatively well, but he began experiencing recurrent episodes of seizures characterized by generalized tonic seizures, occurring at a frequency of 4 to 5 episodes per day, each lasting approximately 10 to 15 s. Despite these seizures, the patient’s neurological examinations were normal, with no reported focal neurological deficits. Upon referral to a neurologist and subsequent workup, neck sonography indicated potential obstruction in the carotid and vertebral arteries.

The patient underwent coronary and cerebral conventional angiography. The coronary angiography revealed mild plaques in the left anterior descending and diagonal ostia. In contrast, the cerebral angiography indicated calcification and complete occlusion of all cranial branches originating from the aorta. Additionally, the angiography findings supported the presence of a porcelain aorta (Figure 1).

The patient underwent neck computed tomography (CT) angiography, revealing calcifications of the aortic arch and its branches, including the brachiocephalic artery, left common carotid artery, and left subclavian artery. These findings strongly suggested complete occlusion of the supra-aortic branches of the aorta at the proximal level (Figure 2, Video 1). Additionally, CT angiography demonstrated the absence of the right internal carotid artery, as well as proximal cutoffs of both the right and left vertebral arteries. Furthermore, there was evidence of proximal occlusion of the left internal carotid artery. Notably, the collateral pathway originating from the aorta played a crucial role in supplying the brain arteries (Figure 3).

Due to the presence of a porcelain aorta and multiple occlusions of cerebral arteries, the patient underwent medical management without any intervention. Remarkably, the patient did not present with any focal neurological deficits and was discharged with a medication regimen consisting of clopidogrel 75 mg daily, aspirin 80 mg daily, rosuvastatin 40 mg nightly, and cilostazol 50 mg daily. At a 4-month follow-up period, the patient reported significant improvement in symptoms and expressed no further concerns. A follow-up CT scan was performed to assess changes in brain blood flow circulation. Notably, the findings from the follow-up CT scan indicated minimal changes in blood perfusion, compared with earlier imaging assessments (Figure 4).

Discussion

In this case report, we presented a case of porcelain aorta with occlusion of all branches of ascending aorta. While the presence of occlusion in a solitary vertebral or internal carotid artery is a common observation in cerebral angiography, the incidence of multiple occlusions in extracranial vessels is relatively infrequent [10,11]. Despite the occlusion of all 4 extracranial arteries, the patient showed mild neurologic symptoms. The manifestation of clinical events does not consistently correspond with the severity of occlusive lesions in major cerebral arteries. In a case report of a patient with 4 vessel occlusions, including bilateral occlusions of internal carotid arteries and vertebral arteries, the only sign of ischemia was hyperthermia [12].

The rare incidence of occlusion in the extracranial segments of 2 major cerebral arteries without resulting in any ischemic neurological deficits has been infrequently observed, with limited cases documented [13]. Similar cases involving multiple occlusions in the primary cerebral arteries often display few or minimal symptoms and signs [8,14,15]. In another case report, a patient with hypothyroidism presented with multiple occlusion of large vessels; however, no serious neurological loss of function was seen [16]. Moreover, there are case reports of asymptomatic patients with occlusion of the 4 vessels of the brain. In our case report, the patient’s recurrent seizures cannot be solely attributed to multiple cerebral artery occlusion; other potential contributing factors should be considered. Cerebral artery occlusion can certainly increase the risk of seizures, due to reduced blood flow and oxygen supply to the brain.

The minimal symptoms observed can be attributed to the absence of intracranial diseases and the notable development of collateral vessels in cases of chronic occlusion [8]. Collateral pathways are crucial for maintaining optimal cerebral blood flow, particularly in situations that can lead to brain ischemia. The primary cerebral collateral network is formed by the circle of Willis [17]. In chronic total occlusion cases, a well-functioning compensatory collateral circulation ensures an adequate supply of arterial blood to meet the metabolic demands of the brain [18]. Nevertheless, in the event of occlusion in all 4 major arteries, even robustly established connections can experience significant strain [19].

Corresponding to our presented case, a distinct case report delineates an infrequent manifestation of atherosclerotic occlusive disease involving all supra-aortic vessels, with the exception of the left common carotid artery. This occurrence was identified incidentally in an asymptomatic patient during a routine pacemaker extraction [20]. In another documented case, a 46-year-old male patient presented to the Emergency Department with acute hemiparesis and facial droop, showcasing occlusion of all major vessels in the aortic arch. The subsequent medical intervention involved multiple angioplasty procedures, followed by aortic arch reconstruction within a 2-month time-frame [21]. Our patient had no other risk factors besides a history of smoking and no pre-existing health conditions, which suggests that smoking may have been the primary or significant contributor to the extensive calcification and complete occlusion of the superior aortic branch.

The incidental finding of a porcelain aorta is commonly associated with widespread atherosclerotic cardiovascular disease [22]. Patients with a porcelain aorta have an increased risk of experiencing an embolic stroke as a result of manipulating aortic atheroma during surgery [23]. A porcelain aorta can pose challenges during cardiac surgeries involving the aortic region [24]. There are various surgical options, such as direct, indirect, or combined bypass techniques, that can be utilized for revascularization in the treatment of intracranial large-vessel occlusion [25,26]. There is ongoing debate regarding the necessity of revascularization in asymptomatic patients [27]. For chronic total occlusions with stable hemodynamics and complete compensation, the recommended approach is the best available medical treatment [28].

In this particular case report, the patient received medical treatment without any surgical intervention. Antiplatelet agents, such as aspirin or clopidogrel, reduce the risk of recurrent stroke in patients with atherosclerotic disease, including those with multiple large vessel occlusions. These agents inhibit platelet aggregation and thrombus formation, thereby reducing the risk of thromboembolic events [29]. Moreover, statins can decrease the likelihood of cardiovascular incidents, such as stroke, among individuals with atherosclerotic conditions. Beyond simply lowering lipid levels, statins offer additional benefits, such as stabilizing atherosclerotic plaques and possessing anti-inflammatory properties [30]. A significant portion of case reports and small series concerning chronic occlusions of multiple extracranial vessels frequently highlight either minimal symptoms upon presentation or positive outcomes with either medical management or surgical intervention [21,31].

Conclusions

In conclusion, this case study highlights the brain’s remarkable ability to withstand severe vascular challenges. Despite complete blockage of the main arteries supplying the brain, the patient exhibited only mild symptoms, indicating the brain’s adaptive capacity. This rarity is characterized by the presence of occlusions in multiple extracranial vessels. The presence of a porcelain aorta further complicated the case. This case study underscores the importance of utilizing advanced imaging techniques, such as brain and neck CT angiography, in the evaluation of complex vascular conditions. Opting for medication over surgery emphasizes the importance of personalized treatment approaches. This case underscores the necessity for ongoing research and careful monitoring to advance our understanding and management of complex vascular conditions.