Hypoplasia Of Internal Carotid Artery Research Articles

Right-sided aortic arch with isolation of the left innominate artery and hypoplasia of the left internal carotid artery.

Here, we report a case of the right-sided aortic arch with isolation of the left innominate artery and hypoplasia of the left internal carotid artery. A 42-year-old male patient underwent a whole-body computed tomography angiography (CTA) examination upon the clinical suspicion of vasculitis. CTA revealed a right-sided aortic arch with the isolation of the left innominate artery and hypoplasia of the left internal carotid artery. The right-sided aortic arch, with the isolation of the left innominate artery, is a scarce vascular variation that may occur with other cardiovascular anomalies such as ventricular septal defect. It can be asymptomatic or can present with symptoms of subclavian steal syndrome. Although its association with the agenesis of the left internal carotid artery has been reported, its association with the hypoplasia of the left internal carotid artery has not been reported previously to the best of our knowledge.

Non-arteritic anterior ischemic optic neuropathy associated with ipsilateral hypoplastic internal carotid artery.

Vascular insufficiency of the optic nerve head is the cause of non-arteritic ischemic optic neuropathy (NAION). Carotid artery abnormalities are now considered as a predisposing factor for developing NAION. In this report, we present a case of NAION associated with ipsilateral internal carotid artery hypoplasia. A 69-year-old female was referred for evaluating the cause of vision loss in her left eye. The patient was diagnosed with left NAION, however, the magnetic resonance imaging (MRI) showed a considerably smaller left internal carotid artery (ICA) than the right side. The magnetic resonance angiography (MRA) confirmed the disparity as well as perfusion abnormalities of intracranial carotid arteries. The diagnosis of left ICA hypoplasia was made and verified by depicting a left side small carotid canal on the computed tomography (CT) scan. This report suggests that ICA hypoplasia could be associated with NAION.

Abstract Number ‐ 220: Internal Carotid Artery Hypoplasia of Unique Anatomical Variance

Introduction Hypoplasia of the internal carotid artery (ICA) is incomplete development of the ICA with the presence of empty carotid canal. Despite anecdotal data reporting less than a 0.01% of developmental anomalies of ICA, the true incidence remains unknown. Many cases are asymptomatic and thus escape diagnostic detection or appropriate intervention. In 1968, Lie and Hage described collateral circulation in ICA developmental anomalies through the Circle of Willis, persistent embryonic vessels, or skull base arteries/anastomosis or rete mirabile. While collateral systems exist, subsequent cerebrovascular insufficiency and vascular malformations may occur as a result. To our knowledge, there is an incomplete body of literature surrounding the implications of ICA hypoplasia of varying anatomy. Methods We present a case report of left ICA hypoplasia and a comprehensive literature review. Results A 70‐year‐old woman with hypertension, nicotine dependence, and chronic obstructive pulmonary disease (COPD) presented with COPD exacerbation and disorientation. Computed tomography (CT) head angiography showing left cervical/petrous ICA aplasia with presence of a small carotid canal. The left external carotid and vertebral artery originate at the aortic arch. Majority of the blood supply to the left anterior cerebral artery (ACA) and middle cerebral artery (MCA) is suppliedby the right anterior communicating artery (AComA), posterior communicating artery (PComA). There is also a small left supraclinoid and carotid terminus that reconstitute predominantly by right A1 segment and PComA (Figure 1). Conclusions Although ICA hypoplasia may be an incidental finding, accurate and promptrecognition is essential for the consideration of endovascular implications, screening for vascular malformations, understanding cerebral vascular flow, managing cerebrovascular risk, and avoiding misinterpretation of clinical and imaging patterns.

Correlation of systemic involvement and presence of pathological skin calcification assessed by ex vivo nonlinear microscopy in Pseudoxanthoma elasticum

Pseudoxanthoma elasticum (PXE (OMIM 264800)) is an autosomal recessive connective tissue disorder mainly caused by mutations in the ABCC6 gene. PXE results in ectopic calcification primarily in the skin, eye and blood vessels that can lead to blindness, peripheral arterial disease and stroke. Previous studies found correlation between macroscopic skin involvement and severe ophthalmological and cardiovascular complications. This study aimed to investigate correlation between skin calcification and systemic involvement in PXE. Ex vivo nonlinear microscopy (NLM) imaging was performed on formalin fixed, deparaffinized, unstained skin sections to assess the extent of skin calcification. The area affected by calcification (CA) in the dermis and density of calcification (CD) was calculated. From CA and CD, calcification score (CS) was determined. The number of affected typical and nontypical skin sites were counted. Phenodex + scores were determined. The relationship between the ophthalmological, cerebro- and cardiovascular and other systemic complications and CA, CD and CS, respectively, and skin involvement were analyzed. Regression models were built for adjustment to age and sex. We found significant correlation of CA with the number of affected typical skin sites (r = 0.48), the Phenodex + score (r = 0.435), extent of vessel involvement (V-score) (r = 0.434) and disease duration (r = 0.48). CD correlated significantly with V-score (r = 0.539). CA was significantly higher in patients with more severe eye (p = 0.04) and vascular (p = 0.005) complications. We found significantly higher CD in patients with higher V-score (p = 0.018), and with internal carotid artery hypoplasia (p = 0.045). Significant correlation was found between higher CA and the presence of macula atrophy (β = − 0.44, p = 0.032) and acneiform skin changes (β = 0.40, p = 0.047). Based on our results, the assessment of skin calcification pattern with nonlinear microscopy in PXE may be useful for clinicians to identify PXE patients who develop severe systemic complications.

Differentiation Between Internal Carotid Artery Hypoplasia and Acquired Narrowing by Neurovascular Ultrasound: Case Series and Literature Review.

Uniformly narrowed internal carotid artery (ICA) without proximal steno-occlusion or parietal anomalies is often subject to misdiagnosis due to lack of awareness. We combined our experiences of 4 cases with 29 previously published cases to form a retrospective series including 18 cases of ICA hypoplasia and 15 cases of ICA acquired narrowing. The ultrasonic manifestations of ICA acquired narrowing and ICA hypoplasia are extremely similar, but narrowed ICA without intracranial occlusion or bottle-neck-sign highly indicates ICA hypoplasia, whereas moyamoya vessels favor ICA acquired narrowing, thus promoting the understanding of and discriminability between the two on neurovascular ultrasound.

1679 An Unusual Case of Hypoplastic Internal Carotid Artery with Segmental Aplasia of The Anterior Cerebral Artery: A Case Report and Systematic Literature Review

Abstract Aim Hypoplasia of the internal carotid artery (ICA) is a rare morphological variant with potential implications in disease and clinical decision-making. We describe an unusual case of ICA hypoplasia in a 50-year-old female who presented with an acute episode of vertigo. CT angiogram showed an unusually short common carotid artery (CCA) on the right side, hypoplasia of the right ICA, and agenesis of the A1 segment of the right anterior cerebral artery (ACA). We provide a short review of the available literature. Method The literature review was performed according to PRISMA guidelines. Three databases (Pubmed, Web of Science, and Ovid) were searched using the terms “ICA” and “Hypoplasia”. Case reports published in English in the last 10 years were considered eligible for inclusion. Reports of acquired ICA hypoplasia or ICA agenesis were excluded. Results Our systematic literature search revealed that 19 cases of congenital ICA hypoplasia have been reported in the last 10 years. Of these, 14 were unilateral hypoplasia, including nine cases in which the anomaly was on the left, and five cases in which the anomaly was on the right. Two cases had additional aplasias; one with aplasia of the ACOM and another with aplasia of segment C6 of the ICA. Conclusions We conclude that ICA hypoplasia remains a rare anomaly, despite the increasing incidence due to the availability of imaging technology. Clinicians should be aware of these variations, as they are frequently associated with haemodynamic changes, aneurysms, and fenestrations. Such variations have important implications for planning angiographic and surgical approaches.

068 Association of systemic involvement with skin morphology assessed by multiphoton microscopy in pseudoxanthoma elasticum

Pseudoxanthoma elasticum (PXE (OMIM #264800)) is an autosomal recessive connective tissue disorder mainly caused by mutations in the ABCC6 gene. PXE leads to ectopic calcification primarily in the skin, eye and blood vessels that may result in blindness and cardiovascular complications. We investigated the association between the skin morphology and systemic involvement in 25 confirmed PXE patients. Ex vivo multiphoton microscopic (MPM) imaging was performed to assess the extent of calcification. The area affected by calcification (CA) in the dermis and density of calcification (CD) was calculated. The number of involved skin sites typically affected by PXE and nontypical skin sites were counted. Extended Phenodex scores were determined. The correlation of the ophthalmological and cardiovascular complications and CA, CD and skin involvement was analysed. Regression models was used for adjustment to age, duration of the disease and sex. The number of typical skin sites showed correlation with the Phenodex score (ρ=0.55, p=0.004), Skin- score (r=0.75, p=<0.001), whereas the number of affected nontypical skin sites correlated with the Eye-score (r=0.4, p=0.047). We found significantly higher CA (p= 0.012) and CD (p=0.032) among patients with redundant skin folds. Also, significantly higher CD was observed in patients with inflammatory skin signs (p=0.039) and with internal carotid artery hypoplasia (p=0.045). There was a significant correlation between CA and the number of affected typical skin sites (r=0.44, p=0.026), aortic calcification (r=-0.44, p=0.027) and peripheral atherosclerosis (r=0.4, p=0.02). CD correlated significantly with the Vascular scores (r=0.45, p=0.022). To conclude, MPM may prove to be useful for the identification of PXE patients with high risk for more severe systemic complications.

Unilateral Internal Carotid Artery Hypoplasia Incidentally Found in Di George Syndrome diagnosed in Adolescence

Unilateral Internal Carotid Artery Hypoplasia Incidentally Found in Di George Syndrome diagnosed in Adolescence

Monocular central retinal artery occlusion caused by bilateral internal carotid artery hypoplasia complicated with patent foramen ovale: a case report and review of literature

BackgroundCentral retinal artery occlusion (CRAO) is an emergent ophthalmic disease which is commonly caused by atherosclerosis, thromboembolism, and arteriospasm. Here, we report a case of CRAO which is caused by extreme rare bilateral internal carotid artery (ICA) hypoplasia complicated with patent foramen ovale (PFO). The cardiogenic emboli blocked central retinal artery through unclosed foramen ovale and specific blood flow pathway.Case presentationThis report describes a case of a 46-year-old woman sudden onset with amaurosis fugax for about 20 min and persistent visual impairment of left eye. Fundus fluorescein angiography shows the arm-retinal circulation time of left eye is 25 s, indicating that the occlusion occurs in the pathway from aortic arch to ophthalmic artery. The MRA and CTA examinations reveal the bilateral ICA hypoplasia and variation of Wills circle. Furthermore, transesophageal echocardiography (TEE) confirms the PFO and cardiogenic embolic event.ConclusionsThis work presents a CRAO case caused by rare congenital hypoplasia of ICA complicated with PFO, reminding us every single cause of vascular disease should be investigated carefully and the TOAST typing of cerebrovascular disease can be of great reference to the ocular vascular disease.

Risk factors of ischemic stroke/transient ischemic attack in young women: A case report

This case report aims to demonstrate the clinical significance of the left internal carotid artery (ICA) hypoplasia in a young woman who received a combined oral contraceptive (COC) and its association with arterial occlusion and the development of a lacunar stroke/lacunar cerebral infarct (LACI). Brain magnetic resonance imaging (MRI) of the left frontal lobe revealed multiple subcortical and periventricular amplification/ischemia foci (49.0×17.0 mm), and lacunar infarct loci (2.0×4.0 mm). Brain magnetic resonance angiography revealed a markedly narrowed lumen of the left ICA (2.0–3.0 mm). MRI diffusion-weighted imaging-fluid attenuated inversion recovery revealed a moderate diffuse wall thickening and reinforcement of the left ICA. The patient was diagnosed with LACI and congenital hypoplasia of the left ICA and underwent antiplatelet drug therapy (aspirin - 100 mg) and a complete withdrawal of COC. A full amelioration of neurological symptoms was observed after one month of treatment.

Ischemic stroke in young women associated with anomalies of the Willis circle and oral contraceptives

Background. Taking into account the tendency to rejuvenate cerebrovascular pathology, it is important to study the etiopathogenesis of ischemic stroke (IS), especially at a young age, including women of the reproductive period. Objective. Analysis of etiopategenetic prerequisites and risk factors for the development of acute cerebrovascular accident (ACVA) in young people and presentation of a clinical case of patient with ACVA, pathogenic type - lacunar infarction in a young woman who took combined oral contraceptives (COCs) as a contraceptive during last 1 year. Methods. The authors analyzed the literature data and presented clinical laboratory data and the results of magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) of cerebral vessels in a patient with a stroke. Results. The authors note that the clinic of ischemic stroke was confirmed by the data of MRI and MRA studies, which revealed in the left frontal lobe among old areas of encephalomalacia the presence of a new focus of lacunar infarction accompanied by disembryonic development of the circle of Willis, which was presented as hypoplasia of the left internal carotid artery (ICA) and left middle cerebral artery (MCA). Laboratory studies have shown a tendency towards thrombophilia. Conclusion. The authors discuss the role of vascular hypoplasia of the internal carotid artery system in the pathogenesis of acute cerebrovascular accidents in young women taking combined oral contraceptives for a long time. The presented case from practice showed that the hypercoagulable syndrome accompanied by COC intake and the small diameter of the ICA and MCA created favorable conditions for occlusion in the small perforating arteries of the deep structures of the brain and, thus, contributed to the development of a brain infarction on the side of the hypoplasia.

Congenital Horner's Syndrome and Internal Carotid Artery Hypoplasia.

Congenital Horner's Syndrome and Internal Carotid Artery Hypoplasia.

Imaging Classification of Internal Carotid Artery Hypoplasia Based on Distal Ophthalmic Segment Occlusion

Background: Internal carotid artery (ICA) hypoplasia (ICAH) is rare. The classification of ICAH is largely unclear. The aim of the study is to propose a new imaging classification for ICAH based on the occlusion of the distal ophthalmic segment and discuss the clinical and radiological differences between the different types. Materials and Methods: This was a retrospective study of patients with congenital ICAH diagnosed at the Department of Neurology of the China-Japan Friendship Hospital between June 2011 and June 2016. The patients underwent temporal bone computed tomography (CT), brain CT, cranial magnetic resonance imaging, transcranial Doppler, and head and neck CT angiography. Results: A total of 20 ICAH patients were divided into the distal occlusion (12 cases; 60%) and nondistal occlusion (8 cases; 40%) types based on whether the distal ophthalmic segment was occluded. The frequencies of collateral circulation from the circle of Willis (P = .01) and dilated cerebrovascular lesions (P = .001) in the distal occlusion type was higher than in the nondistal occlusion type. Five (25%) patients developed adverse cerebrovascular events during followup: 3 ischemic cases were of the nondistal occlusion type, and 2 cases with subarachnoid hemorrhage were of the distal occlusion type. Conclusions: A novel classification of ICAH was revealed based on the occlusion of the distal ophthalmic segment. The 2 types may show differences in collateral circulation patterns, coexisting cerebrovascular abnormalities, and potential clinical outcomes.

A Case of Unruptured Cerebral Aneurysm Treated with Coil Embolization Associated with Internal Carotid Artery Hypoplasia and an Aberrant Right Subclavian Artery

内頚動脈低形成と異所性右鎖骨下動脈に合併した未破裂内頚動脈瘤に対し，コイル塞栓術を施行した症例を経験したので報告する．症例は56歳女性．めまいの精査目的に他院で施行したmagnetic resonance (MR) imagingで左内頚動脈瘤を指摘されたため当院へ紹介となった．初診時，神経学的な異常は認めなかった．脳血管撮影とCT angiographyで右内頚動脈低形成と異所性右鎖骨下動脈を認めた．左内頚動脈瘤の大きさは7.5×5.8 mm，neckは3.6 mmであり，ブレブを伴っていた．破裂予防目的に左内頚動脈瘤に対してバルーン併用下コイル塞栓術を施行した．術中，左内頚動脈の血流遮断を数回要した．左内頚動脈瘤は完全に塞栓され，周術期に有害事象は認めなかった．内頚動脈低形成と異所性右鎖骨下動脈の合併は稀であり，これらの破格を有する症例では血管内手術を行う際，アクセスルートの制限や脳虚血耐性に注意が必要である．

Bilateral Internal Carotid Artery Hypoplasia in Asymptomatic Patient. Case Report

Bilateral Internal Carotid Artery Hypoplasia in Asymptomatic Patient. Case Report

Sturge-Weber syndrome with Klippel-Trenaunay syndrome and hypoplasia of internal carotid artery

Sturge-Weber syndrome with Klippel-Trenaunay syndrome and hypoplasia of internal carotid artery

Випадок комбінованого хірургічного лікування нерозірваної гігантської мішкоподібної аневризми супракліноїдного відділу правої внутрішньої сонної артерії

We tried to determine the optimal tactics of endovascular occlusion of a giant saccular aneurysm supraclinoid part of the right internal carotid artery, which moves right middle cerebral artery on the background of hypoplasia and stenosis of the right internal carotid artery (ICA) in petrous segment in conjunction with stenosis in the M1-segment of right middle cerebral artery (MCA) and deconstruction clinoid part of the right ICA using coils with maintaining adequate perfusion in right MCA. We analyzed the results of surgical treatment endovascular embolization of giant intracranial unruptured aneurysms, on the background of concomitant stenosis of the ICA (2 similar cases in my own practice). In all cases, regardless of the degree of ICA stenosis performed a stand-alone coiling of aneurysms occlusion. Stenting of the stenotic part of the ICA was not performed. The results of the combined 3-stage of surgical treatment (bypass, endovascular coiling-aneurysm occlusion of the ICA, deconstruction ICA) patient with a giant saccular aneurysm supraclinoid part of right ICA, from which departs right MCA, against the background of hypoplasia and stenosis of right ICA in the petrous segment in conjunction with stenosis in the M1-segment of right MCA. After 3 months the patient's condition by somatic satisfactory. Neurologically: remained moderate paresis of the abducens nerve on the right. The patient is recommended dynamic observation with conducting magnetic resonance imaging of the brain and cerebral angiography at 6 and 12 months. The analysis of the result of combined surgical treatment in the combined pathology of the cerebral vessels (saccular aneurysm, stenosis of right ICA, stenosis of right MCA). Showed that surgical tactics was justified and can be used as a method of treating this pathology.

Symmetry, asymmetry and hypoplasia of the intracranial internal carotid artery on magnetic resonance angiography.

The aim of this study was to determine the prevalence of symmetry, asymmetry and hypoplasia of the intracranial internal carotid artery (ICA), and the possible presence of other variants of the ICA by magnetic resonance angiography (MRA). This prospective-retrospective study included 1000 subjects who underwent consecutively MRA of the cerebral arteries. 3D-time of flight angiograms were performed according to the standard protocol. We measured the diameter of the ICA and the diameter of the A1 segment of the anterior cerebral artery (ACA). The symmetry, asymmetry and hypoplasia of the ICA, as well hypoplasia of the A1 segment of the ACA were analysed using the vascular asymmetry coefficient. The right and left ICA were symmetrical in 93.9% examinees, and asymmetrical in 6%, while ICA hypoplasia was found in only one patient (0.1%). Other variants of the ICA were not found. There was no significant difference in the prevalence of individual variations between male and female patients, and no significant frequency difference was found between anatomical variations in terms of the right and left ICA. In 38.3% patients with asymmetrical ICAs, aplasia or hypoplasia of the A1 segment of the ACA was found on the side of the ICA with a reduced diameter. Congenital hypoplasia of ICAs is a very rare abnormality, while asymmetry of ICAs is more common. After evaluating severe asymmetry of intracranial ICAs by MRA, MRA of the neck is recommended, especially in patients with a complete anterior part of the circle of Willis.

The common carotid artery hypoplasia and aplasia of the left internal carotid artery combined with aneurysm of the anterior communicating artery by CT-angiography

In the present article, we discuss the problem of cerebrovascular diseases as one of the most important in social terms. The mechanisms of occurrence and epidemiological parameters characterizing the problem as a whole are presented. The article summarizes the main beam techniques such as angiography, magnetic resonance imaging, ultrasound duplex scanning and X-ray computed tomography with bolus intravenous contrast infusion to allow a timely recognition of the disease. The strengths and weaknesses of different diagnostic tools are considered. The role of CT angiography as a primary diagnostic method is defined. CT angiography offers the advantage of getting comprehensive information about existing circulatory disorders. The case of rare congenital anomaly - hypoplasia of the common carotid artery and internal carotid artery aplasia in conjunction with an aneurysm of the anterior communicating artery, - is described in 58-year-old woman. The characteristics of this disease are discussed. We also present the results of a survey on such vascular anomalies, which is defined on the basis of the type of compensatory intracranial collateral flow. This observation is of particular interest due to the rare occurrence and the existing difficulties in the choice of treatment.

Internal Carotid Artery Hypoplasia: Role of Color-Coded Carotid Duplex Sonography.

The purpose of this study was to determine the role of color-coded carotid duplex sonography for diagnosis of internal carotid artery hypoplasia. We retrospectively reviewed 25,000 color-coded carotid duplex sonograms in our neurosonographic database to establish more diagnostic criteria for internal carotid artery hypoplasia. A definitive diagnosis of internal carotid artery hypoplasia was made in 9 patients. Diagnostic findings on color-coded carotid duplex imaging include a long segmental small-caliber lumen (52% diameter) with markedly decreased flow (13% flow volume) in the affected internal carotid artery relative to the contralateral side but without intraluminal lesions. Indirect findings included markedly increased total flow volume (an increase of 133%) in both vertebral arteries, antegrade ipsilateral ophthalmic arterial flow, and a reduced vessel diameter with increased flow resistance in the ipsilateral common carotid artery. Ten patients with distal internal carotid artery dissection showed a similar color-coded duplex pattern, but the reductions in the internal and common carotid artery diameters and increase in collateral flow from the vertebral artery were less prominent than those in hypoplasia. The ipsilateral ophthalmic arterial flow was retrograde in 40% of patients with distal internal carotid artery dissection. In addition, thin-section axial and sagittal computed tomograms of the skull base could show the small diameter of the carotid canal in internal carotid artery hypoplasia and help distinguish hypoplasia from distal internal carotid artery dissection. Color-coded carotid duplex sonography provides important clues for establishing a diagnosis of internal carotid artery hypoplasia. A hypoplastic carotid canal can be shown by thin-section axial and sagittal skull base computed tomography to confirm the final diagnosis.