Anomalies Of Carotid Arteries Research Articles

Patulous Eustachian Tube Accompanied by Internal Carotid Artery Anomalies

Patulous Eustachian Tube Accompanied by Internal Carotid Artery Anomalies

Radiographic and clinical features of internal carotid artery anomalies

Objective To analyze the the radiographic and clinical features of the internal carotid artery (ICA) anomalies. Methods The clinical and radiographic characteristics were retrospectively analyzed in cases of ICA anomalies screened from our intervention database in changhai hospital from september 2009 to september 2014. Results Totally, 6 female patients were included with average age of (45±13) years old. The clinical manifestations included subarachnoid hemorrhage in 1 cases, asymptomatic complaint in 1, cerebral infarction in 1, dizziness in 2 and headache in, Unilateral dysgenesis was observed in 2 cases and bilateral in four with 2 cases of dysgenesis of vertebral artery. Intracranial aneurysm was observed in 4 cases and 1 complicated with dural arterial venous fistulous. Endovascular treatment was performed in 2 patients and coupled with bypass surgery in one. Risks control was performed in 2 patients. No adverse event was observed. Conclusions The diagnosis of ICA anomalies was based on the morphology of carotid canal. Multiple intracranial vascular disorders might be complicated. Thorough analysis of radiographic features was beneficial to decision-making for clinical practice. Key words: Internal carotid artery; Developmental disabilities; Radiographic; Clinical features

Preoperative detection of anomalies of carotid arteries in the neck surgery.

The aim of this study was to examine the course of the carotid artery (CA) and define CA anomalies important for neck surgery using conventional and three-dimensional computer tomography (3D CT). This was designed as an observational study with retrospective CT and 3D CT angiography analysis. 4,000 CAs depicted on 2,000 CT angiographies were analyzed to determine the relationship of common, internal, and external portions of the CA to the neck organs, with special emphasis on the thyroid gland area and organs involved in the neck dissection surgery. A total of 47 out of 4,000 imaged CAs had congenital anomalies (1.18%). These anomalies were found in 45 patients, unilaterally in 33 and bilaterally in two. The level of the common CA bifurcation to internal and external CAs was rarely abnormal. It was at the level of C5 and C6 in two cases only (0.05%). The common CA, either aberrant or tortuous, ran abnormally close to the thyroid gland in 22 cases (0.55%). In planning neck dissection surgery, laryngectomy, or thyroidectomy, the surgeon should bear in mind that CA anomalies are present in approximately 1.2% of cases. Preoperative conventional CT angiography or 3D CT allows precise identification of anomalous CAs, thereby reducing the possible risk of intraoperative injury.

Incidence and Risk Factors of the Retropharyngeal Carotid Artery on Cervical Magnetic Resonance Imaging

Retrospective cohort. Define incidence of anomalous carotid vasculature and associated risk factors as pertains to the anterior approach. The carotid artery system, including the common, internal, and external branches, is lateral to the foramen transversarium. If unrecognized, aberrancies in carotid vessel anatomy can lead to devastating complications. A total of 1000 cervical magnetic resonance imagings were screened to localize the carotid artery respective to medial/lateral location of the vessel at each segment from C2-C3 to C6-C7 bilaterally. Vessel location was classified in 3 zones: lateral to the vertebral foramen (type I) (normal); between the lateral foramen and uncoverterbral joint (type II); and medial to the uncovertebral joint (type III). Type III locations were compared with age-matched controls for assessment of cervical alignment via the Ishihara index, C2-C7 angle, and degree of spondylosis. A total of 123 patients demonstrated carotid artery anomalies (type II and III) (12.3%). Twenty-six patients had type III aberrancy (2.6%). Patients with anomalies were significantly older and more likely to be female (60 vs. 51 yr of age, 74% vs. 57% female, respectively, P < 0.05). The type III group average age was 66.1 years and 88% were female. Aberrancies were more likely right-sided and at C3-C4 or cranial. C2-C7 angle showed significantly greater kyphosis in patients with anomalies compared with controls (6.2 vs. 14.4, P = 0.03). The number of severely spondolytic segments was significantly greater in patients with Type III locations than controls (2.0 vs. 1.1 P < 0.05). Carotid arterial anomalies occurred in 12.3% of cases; severe aberrancy was present in 2.6% of patients. In elderly females with kyphotic alignment, a high index of suspicion must be raised for aberrancy. Preoperative assessment of the vasculature in the anterior neck may avoid catastrophic complications.

Cervical vascular and upper airway asymmetry in velo-cardio-facial syndrome: Correlation of nasopharyngoscopy with MRA

Purpose Velo-cardio-facial syndrome (VCFS), the most common genetic syndrome causing cleft palate, is associated with internal carotid and vertebral artery anomalies, as well as upper airway asymmetry. Medially displaced internal carotid arteries, often immediately submucosal, present a risk of vascular injury during pharyngeal flap surgery for velopharyngeal insufficiency (VPI). We evaluate the frequency and spectrum of cervical vascular anomalies in a large cohort of VCFS patients correlating MRA with nasopharyngolaryngoscopy in detecting at risk carotid arteries. Furthermore, we assess the relationship with respect to laterality between cervical vascular patterns and the asymmetric abnormalities of these subjects’ upper airways. Methods Cervical MRAs of 86 subjects with VCFS and 50 control subjects were independently reviewed by three neuroradiologists. The course of the internal carotid and vertebral arteries was identified within the pharyngeal soft tissues. Medial deviation, level of bifurcation, dominance, anomalous origin, and vessel tortuosity were recorded. Nasopharyngoscopy examinations were available for retrospective review in 43 patients and were assessed for palatal and posterior pharyngeal wall symmetry, true vocal cord motion and size, and for the presence or absence of carotid pulsations. The endoscopic findings were compared with MRA results. Results Of the 86 subjects, 80 (93%) had one or more vascular anomalies. 42 subjects (49%) were found to have medial deviation of at least one internal carotid artery. In 24 subjects (28%) the anomalous internal carotid artery was directly submucosal; four of these were bilateral (5% of the total sample, 17% of those with a submucosal internal carotid). Other carotid anomalies included low carotid bifurcation (44 subjects or 51%), anomalous origin of the right common carotid (32 cases, or 37%), and two cases of internal carotid agenesis/hypoplasia. Vertebral artery anomalies included vessel tortuosity (34 cases, or 40%), hypoplasia (10 cases, or 12%), looping (4 cases, or 5%), and one case of a double left vertebral artery. Though patients in our study showed an asymmetric distribution of vascular anomalies, no association was found between the laterality of palatal motion, pharyngeal fullness, or laryngeal movement and structure with ipsilateral vertebral or carotid artery anomalies. Of the 33 pulsatile carotid arteries visualized at nasopharyngoscopy, only nine were found to be submucosal on MRA. In contrast, 11 submucosal carotid arteries confirmed at MRA demonstrated no visible pulsations. Positive and negative predictive values of pulsative arteries seen endoscopically for MRA confirmation of a submucosal carotid course was 27% and 79% respectively. Conclusions Carotid and vertebral artery anomalies are common in VCFS including marked medial deviation of the internal carotid artery in close proximity to the donor site for pharyngeal flap surgery. Lack of correlation between laterality of vascular anomalies and upper airway structural asymmetry in VCFS does not support the hypothesis that palatal, pharyngeal, and laryngeal anomalies are due to secondary developmental sequences caused by in utero vascular insufficiency. The presence or absence of carotid pulsations seen by nasopharyngoscopy does not correlate with the carotid arterial depth identified on MRA. Furthermore, identification of the relative medial–lateral retropharyngeal position of a submucosal carotid affords the opportunity to modify the surgical approach. These findings further support the routine use of pre-operative neck MRA in VCFS patients in surgical planning.

Thyroid gland and carotid artery anomalies in 22q11.2 deletion syndromes

Chromosome 22q11.2 deletion syndromes (22q11DS), such as velocardiofacial syndrome and DiGeorge syndrome demonstrate developmental anomalies affecting the branchial arch structures. Mouse models have shown abnormalities of both the thyroid gland and the common carotid arteries and have suggested a developmental link between the two structures. Here we examine radiological abnormalities of the carotid artery bifurcation and thyroid gland. Retrospective case control study. We reviewed multiplanar contrast-enhanced computed tomography scans with 3-D reformats of 66 children falling into three groups: 1) children with velopharyngeal insufficiency testing positive for 22q11DS (n = 28), 2) children with velopharyngeal insufficiency testing negative for 22q11DS (n = 10), and 3) age and gender matched controls (n = 28). The common carotid bifurcations were measured according to cervical spinal (C-spine) level. The thyroid gland morphology was also assessed. The 22q11DS group demonstrated lower overall common carotid bifurcations. The median C-spine level of bifurcation was significantly different in the 22q11DS group compared to controls on the right (5 vs. 3.; P < .01) and left (6.5 vs. 3.25; P < .001). These patients demonstrated asymmetry when comparing the bifurcations between sides. Fourteen of 28 patients (50%) with 22q11 deletions demonstrated at least one of four different thyroid anomalies. Anomalies included absence of a lobe, absence of the isthmus, retrocarotid, or retroesophageal extension. The 22q11DS group had significantly higher proportions of thyroid abnormalities compared to the control groups (P < .01). Patients with 22q11DS demonstrate statistically significantly lower carotid artery bifurcations and a higher proportion of radiological thyroid abnormalities.

Letters to the Editor

4 June 2008 Dear Editor, PHACE SYNDROME: A CONSTELLATION OF CEREBRAL AND CARDIOVASCULAR ANOMALIES AND SEGMENTAL HAEMANGIOMAS PHACE syndrome is an uncommon neurocutaneous syndrome that manifests with multi-system involvement. It refers to the association of large, plaque-like, ‘segmental’ haemangiomas of the face, posterior fossa brain malformations, arterial cerebrovascular anomalies, cardiovascular anomalies, eye anomalies and ventral developmental defects.1 This letter describes a male baby who was born to a 41-year-old mother by elective section at term gestation and who weighed 2.88 kg. A prenatal diagnosis of posterior fossa malformation had been made at a 19-week anomaly scan. The infant was delivered breech by LSCS with forceps lift-out and was noted to have extensive bruising on left side of the face with an ipsilateral facial palsy. Over the next week the bruising was replaced by fine telangiectasia, which became darker and evolved into a confluent segmental facial haemangioma in the distribution of trigeminal cranial nerve extending into the oral cavity (Fig. 1). The important differential diagnosis at this stage was Sturge-Weber syndrome. Left sided segmental haemangioma with oral mucosal extension. MRI brain was consistent with Dandy Walker malformation. In addition, a soft tissue mass encircling cranial nerves VI and VII (possible acoustic neuroma, Fig. 2) along with hypoplasia of left vertebral artery was noted. The facial haemangioma was seen to extend internally to the left cavernous sinus, the extraconal spaces of the left eye and the superior mediastinum. While carotid Doppler showed no evidence of stenosis, the echocardiogram showed features consistent with coarctation of aorta. The ophthalmology review was unremarkable, showing no evidence of raised intraocular pressure or retinal detachment/optic neuropathy. He was started on laser therapy for the surface hemangioma after which it showed signs of gradual resolution. He was readmitted at 8 weeks of age with stridor and bronchoscopy revealed a large sub-glottic haemangioma obstructing most of the airway, which was managed by placement of a tracheostomy tube. MRI brain showing soft tissue mass encircling VI and VII nerves. While the acronym PHACE (OMIM 606519) was suggested by Frieden in 1995,2 the exact incidence of PHACE syndrome is unknown. Pathogenesis too is unknown, while the constellation of anomalies generally represents a ‘developmental field defect’, whereby an insult in embryogenesis at a critical time gives rise to similar developmental outcomes.3 The timing of these errors in morphogenesis occurs early, probably between 3 and 12 weeks of gestation, prior to or during early vasculogenesis. The syndrome represents a spectrum of disease, with few infants manifesting the entire constellation of anomalies, some components appearing later. The marked female preponderance (>80%) suggests a possibility of X-linked inheritance with lethality in males.1 Both sporadic and X-linked or autosomal inheritance patterns are reported, the X-linked form being typically bilateral and diffuse, and often associated with a mutation of filament A gene (FLNA) on Xq28.4 The hallmark of PHACE syndrome, a large, segmental facial haemangioma, may involve one to several facial dermatomes, and an early presentation may be mistaken for the port wine stain associated with the Sturge-Weber syndrome. Structural and cerebrovascular brain malformations occur in at least half. Posterior fossa malformations, including the Dandy-Walker complex, are the most common, and others include agenesis of corpus callosum and polymicrogyria. Developmental delay is a common association. Cerebrovascular defects present primarily as carotid artery anomalies, but also include aberrant/anomalous origins and aneurysm of cerebral vessels. The cerebrovascular anomalies, especially carotid artery anomalies, are of particular concern because of predisposition to progressive arterial occlusion and stroke.5, 6 Cardiac anomalies include coarctation of the aorta and septal defects, while ophthalmologic anomalies include retinal vascular abnormalities, optic nerve atrophy, and colobomas, among others. Infants with PHACE syndrome could represent up to 20% of infants with segmental facial hemangiomas, and both progressive and regressive vascular phenomena may occur in this syndrome.1 The most common therapeutic option directed at regression of haemangioma is prednisolone/ methylprednisolone, while others include topical steroids, pulsed dye laser, interferon alpha, vincristine, excision surgery and wound care.1 To summarise, a segmental hemangioma may be an initial presentation of PHACE syndrome, and a high index of suspicion and thorough investigation would optimise management.

Vertebral and carotid artery anomalies in patients with aberrant right subclavian arteries

There is little published evidence regarding the patterns and prevalence of vertebral artery (VA) and common carotid artery (CCA) anomalies in patients with an aberrant right subclavian artery (ARSCA). To study the patterns and prevalence of VA and CCA anomalies in patients with ARSCA. In a 2-year period we reviewed the children referred with suspected vascular ring who had undergone multidetector-row CT. Patients with ARSCA were reviewed for VA and CCA patterns and their prevalence and relevance were calculated. In total, 102 patients with ARSCA were identified. VA anomalies were present in 16 patients (15.7%), and CCA anomalies (common carotid trunk) in 21 patients (20.6%). In some patients with VA anomalies, the right VA arose from the right CCA and in some the left VA arose from the aortic arch. When the left VA arose from the aortic arch it was situated between the left CCA and the left SCA or between the left SCA and the ARSCA. If neurointerventionalists understand these potential anomalies and their prevalence, time and contrast medium could be saved when catheterizing the VA and CCA in patients with ARSCA.

Vascular anomalies in Alagille syndrome: a significant cause of morbidity and mortality.

Alagille syndrome (AGS) is a dominantly inherited multisystem disorder involving the liver, heart, eyes, face, and skeleton, caused by mutations in Jagged1. Intracranial bleeding is a recognized complication and cause of mortality in AGS. There are multiple case reports of intracranial vessel abnormalities and other vascular anomalies in AGS. The objective of this study was to characterize the nature and spectrum of vascular anomalies in AGS. Retrospective chart review of 268 individuals with AGS was performed. Twenty-five patients (9%) had noncardiac vascular anomalies or events. Sixteen patients had documented structural vascular abnormalities. Two had basilar artery aneurysms, 7 had internal carotid artery anomalies, and another had a middle cerebral artery aneurysm. Moyamoya disease was described in 1 patient. Three of the 16 patients had aortic aneurysms, and 2 had aortic coarctations. One of the patients with a basilar artery aneurysm also had coarctation of the aorta. One of the individuals with an internal carotid artery anomaly also had renal artery stenosis. Nine more patients had intracranial events without documented vessel abnormalities. Vascular accidents accounted for 34% of the mortality in this cohort. The vascular anomalies described in our cohort of AGS individuals identify an underrecognized and potentially devastating complication of this disorder. It is a major cause of morbidity and mortality in this population, accounting for 34% of the mortality. We have also reviewed the body of evidence supporting a role for Jagged1 and the Notch signaling pathway in vascular development.

Pharyngeal flap and the internal carotid in velocardiofacial syndrome.

Internal carotid artery anomalies have been documented as a common clinical feature in velocardiofacial syndrome. There has been some controversy over the need for preoperative imaging procedures, such as magnetic resonance angiography, when planning pharyngeal surgery for correcting velopharyngeal insufficiency. The purpose of this article is to describe 20 patients with velocardiofacial syndrome who received comprehensive evaluation and underwent pharyngeal flap surgery within a 2-year period and to report the technique used for dissecting the flap and the surgical outcomes. Anomalies of the major neck vessels were present in all cases, but 5 of these 20 cases had particularly severe anomalies of the internal carotid arteries that placed the vessels directly deep within the donor site for the pharyngeal flap. Surgery was carried out successfully in all 20 cases using a modified approach after radiographic imaging was performed to locate the arteries. In the 5 cases with severe malpositioning of the internal carotid arteries, it was clear that the vessels could have been injured had their location not been identified and the surgical approach modified to avoid them.