Congenital Aplasia of the Common Carotid Artery: A Comprehensive Review.

In an attempt to describe the morphofunctional consequences of uni- and bilateral aplasia of the common carotid artery (CCA), which is usually a vascular source of the external carotid (ECA) and internal carotid (ICA) arteries, we investigated online databases of anatomical and clinical papers published from the 18th century to the present day. We found 87 recorded cases of uni- and bilateral CCA aplasia in subjects from the first hours to the eighth decade of life, which had been discovered in 14 (known) countries. Four crucial parameters were described: the embryology of the carotid arteries, morphophysiology of the carotid arteries, CCA aplasia, and unilateral versus bilateral CCA aplasia, including history, general data, diagnosing, vascular sources, caliber, course of the separated ECA and ICA, associated vascular variants, and pathological disorders. To complete the knowledge of the morphofunctional consequences of the absence of some artery of the carotid system, and risking the possibility of repeating some words, as "carotid artery", or "carotid aplasia" and the headings from our previous article about bilateral ICA absence, this review is the first in the literature that recorded all cases of the CCA aplasia published and/or cited for the past 233 years. Main characteristic of the CCA absence is its association with 21 different diseases, among which the aneurysms were in 13.69% of cases, and 17.80% of cases were without pathology.

Déficit neurológico transitorio secundario a síndrome estilocarotídeo izquierdo / Transient neurological deficit secondary to left-side stylocarotid syndrome

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Intrathoracic bifurcation of the right common carotid artery.

Intrathoracic carotid artery bifurcation is a very rare condition and only six cases have been reported to the best of our knowledge. We present a case of a 52-year-old man who was found to have an atretic intrathoracic internal carotid originating at the T3 level. The recognition of an intrathoracic carotid bifurcation is essential in the planning of thoracic and vascular procedures to avoid unintentional iatrogenic injuries.

Remote results of reconstructive operations in pathological deformity of the internal carotid artery.

OBJECTIVE: to study remote results of surgical treatment of patients presenting with a pathological deformity of the internal carotid artery. METHODS: the study was based on retrospectively analysing the findings of examination and the outcomes of surgical treatment of 142 patients presenting with an isolated pathological deformity of the internal carotid artery (ICA), who underwent a total of 166 reconstructive operations. RESULTS: 7% of patients were operated on at the asymptomatic stage, more than half (56%) of the patients were found to have transient (21%) or persistent neurological deficit (35%), 37% of patients had stage III cerebrovascular insufficiency. Twenty-five (15%) operations were performed for C- and S-shape tortuosity, ninety-seven (58%) procedures for kinking of the internal carotid artery, and forty-four (27%) interventions for coiling. The operation of choice was resection with redressation of the internal carotid artery and reimplantation into the old ostium (74%). Analysing the results of surgical treatment showed its high neurological efficiency in prevention of cerebral circulation impairments (93% of patients at terms up to 25 years had no such complications), as well as clinical efficacy (relief of cerebrovascular insufficiency symptoms in 91.1% of patients). The effect obtained appeared to preserve during 2 and 5 years in 96% and 92% of patients, respectively, and at terms up to 25 years in 83%. The 3-, 5- and up- to-25-year survival amounted to 95%, 92% and 75%, respectively. Restenosis of the internal carotid artery was significantly more often observed after prosthetic repair of the artery (p <0.05) as compared with other methods of reconstruction employed. Thromboses of the internal carotid artery were encountered with similar frequency (8%) only after prosthetic repair and resection of the internal carotid artery with an «end-to-end¼ anastomosis, which was significantly more often than in resection with redressation (p <0.05). Stroke incidence in the remote postoperative period amounted to 0.8% and the «stroke + stroke-related mortality¼ parameter equalled 1.6%. CONCLUSION: the remote results of surgical treatment for pathological deformity of the internal carotid artery are strongly suggestive of high efficacy of this method of prevention of impairments of cerebral circulation and treatment of cerebrovascular insufficiency in patients with the pathology concerned. The operation of choice in pathological deformity of the internal carotid artery is resection with redressation of the internal carotid artery and reimplantation into the original ostium.

Carotid rete mirabile and pseudoxanthoma elasticum: an accidental association?

We report the case of a young female patient with a transient amaurosis due to a carotid rete mirabile (CRM), a rare congenital carotid malformation, and pseudoxanthoma elasticum (PXE), an inherited autosomal recessive systemic metabolic disorder characterised by fragmentation and mineralisation of elastic fibres in connective tissues (skin, eyes) and the vascular system. CRM is a rare form of intracranial carotid malformation whose association with PXE (6 cases at present) would appear not to be accidental. This observation suggests a new link between congenital arterial remodelling and the PXE.

[Immediate outcomes of reconstructive operations for pathological deformity of the internal carotid artery].

OBJECTIVE: To investigate the immediate outcomes of surgical treatment of patients presenting with pathological deformity of the internal carotid artery. METHODS: The study was based on retrospective analysis of the outcomes of surgical management of 142 patients with isolated pathological deformity of the internal carotid artery who underwent a total of 166 reconstructive procedures. RESULTS: Resection with redressing of the internal carotid artery and reimplantation into the old bed was a method of choice of repairing the deformed artery (74%). More often it was possible to have performed it in kinking, C- and S-shape tortuosity of the internal carotid artery, whereas prosthetic repair was more often in coiling (P < 0.05). A positive clinical effect of the operation was achieved in 97% of the patients. Neurological efficacy of the performed interventions depended upon the baseline degree of cerebrovascular insufficiency and the duration of clamping of carotid arteries (P < 0.05). The incidence of the development of thrombosis of the reconstructed internal carotid artery (1.2%) did not depend on either the form of pathological deformity or the method of reconstruction thereof. Cerebral circulation impairments in two (1.2%) cases were transitory and in three (1.8%) resulted in ischaemic stroke, one of which having become the cause of the patient's death. The index "stroke + lethality from stroke" amounted to 2.4%. Neurological complications had been caused by rheological impairments, instability of haemodynamics and inadequate redressing of the internal carotid artery. CONCLUSION: Analysing the immediate outcomes confirmed that surgical treatment of pathological deformity of the internal carotid artery is an effective method aimed at preventing cerebral circulation impairments and removal of cerebrovascular insufficiency in patients with the disease concerned.

Persistent primitive trigeminal arteries (PTA) and its variant (PTAV): analysis of 103 cases detected in 16,415 cases of MRA over 3 years.

INTRODUCTION: The primitive persistent trigeminal artery (PTA) is the most common carotid-basilar anastomosis, but because of its extremely low incidence, it is poorly understood. The purpose of this study is to clarify the features of PTA and its variants (PTAV) based on a large magnetic resonance angiography (MRA) series. METHODS: MRA was performed on continuous 16,415 patients (8,610 males and 7,805 females) between October 2005 and September 2008 using two 1.5-T systems and one 1.0-T system. These all MRAs were evaluated by neuroradiologists. RESULTS: The incidence of both PTA and PTAV was 0.68% (48 cases of PTA, 50 cases of PTAV, and five unclassified). Among them, 65 cases originated from the left internal carotid artery and 38 from the right. There were 44 cases of lateral type PTA and four cases of medial type. Hypoplasia of the basilar artery proximal to the union of PTA was recognized as follows: no hypoplasia in 12 cases, moderate hypoplasia in 22 cases, and severe hypoplasia in 13 cases. Distance from SCA and the union was an average of 6.7 mm. Four cases of pre- and postoperative cerebral aneurysms were recognized in 103 cases (3.9%). CONCLUSION: We have identified details of both PTA/PTAV. The incidence of PTA was similar to previous studies, and the co-existence of cerebral aneurysm was also similar when compared to patients in the general population without PTA or PTAV.

Congenital external carotid artery aneurysm.

An 8-month-old child presented with a right pulsatile neck mass. The tumor's rapid increase in size and respiratory problems prompted image evaluation. An external carotid artery aneurysm was found, which was compressing other neck structures. The patient underwent aneurysm resection and ligation at its insertion on the common carotid artery. Recovery was uneventful and no further aneurysms on other arteries were found.

Caso clínico radiológico: carótida interna aberrante / Radiologic clinic case: deviant internal carotid

La carótida interna aberrante es una rara malformación congénita. Se presenta el caso de una paciente pediátrica con esta alteración como hallazgo durante la evaluación por imágenes, complementaria de una patología otorrinolaringológica frecuente.

The deviant internal carotid is a unusual congenital malformation. The case of a pediatric patient with this alteration is presented. The finding was made during a rutine image assesment of an ENT disease.

Agenesis of internal carotid artery in a child with ipsilateral Horner's syndrome.

Horner's syndrome is characterized by a classic triad of ipsilateral pupillary miosis, partial eyelid ptosis, and facial anhydrosis. This case study reports a 7-year-old boy with right miosis, mild blepharoptosis, and iris hypopigmentation detected in a routine pediatric follow-up without ipsilateral facial anhydrosis, flushing, or pain. There was no history of birth trauma and test with cocaine provoked no response of the right pupil, suggesting right Horner's syndrome. Mediastinal tumor was ruled out and brain magnetic resonance imaging incidentally showed absence of flow in the right internal carotid artery. Subsequent magnetic resonance angiography demonstrated agenesis of the right internal carotid artery without other vascular-associated malformations. The final diagnosis was right, congenital Horner's syndrome due to ipsilateral internal carotid agenesis. We describe in detail the radiological findings and pathophysiological mechanisms of this unusual association.

A clinical classification system for aberrant internal carotid arteries.

OBJECTIVES/HYPOTHESIS: Pronounced anatomic variations of the extracranial internal carotid artery (ICA) can be found in 5% to 6% of the general population. An aberrant ICA is at risk of injury during routine pharyngeal procedures if the vessel is placed into close relationship with the pharyngeal wall. The objective was to highlight the impact of parapharyngeal ICA aberrations and to analyze the clinical usefulness of the current anatomic classification system of these anomalies. STUDY DESIGN: Prospective quality assessment study in an academic tertiary care medical center. METHODS: A systematic clinicoradiological follow-up study was performed over a period of 18 months. The ICA's course was classified according to Weibel and Fields in 21 consecutive patients with 35 pronounced parapharyngeal ICA aberrations. The vessels' minimum distance to the pharyngeal wall and the corresponding pharyngeal level was analyzed. RESULTS: Tortuosity was found in 13, kinking in 18, and coiling in 4 aberrant ICAs. The vessel's minimum distance to the pharyngeal wall ranged from 0.8 to 17.9 mm (mean, 7.0 mm), but the currently used anatomic classification was inadequate to determine the risk potential for ICA injury in the given cases. We propose a clinicoradiological classification system, which considers the vessel's relation to the pharyngeal wall. CONCLUSIONS: Parapharyngeal ICA aberrations are common and probably under-appreciated anatomic variations. The awareness of these anomalies is essential for clinicians. Since the current anatomic classification is insufficient to outline patients with an increased risk of ICA injury, a clinicoradiological graduation of cervical ICA aberrations may be of practical benefit.

Bilateral internal carotid artery agenesis with artery compression of the brain parenchyma.

Bilateral internal carotid artery (ICA) agenesis is an extremely rare congenital anomaly. The most common type of collateral circulation is developed through the circle of Willis via the basilar and posterior communicating arteries. Both anterior circulations are usually supplied by enlarged posterior communicating arteries (PCOMs). We present an unusual case of bilateral ICA agenesis, which is associated with dolichoectatic left PCOM and left posterior cerebral artery (PCA) compression of left basal ganglia and thalamus. These complex cerebral and vascular anomalies can be noninvasively revealed and evaluated by CT and MR.

Tortuous internal carotid artery narrowing pyriform sinus: two cases.

An elongation of the internal carotid artery should be classified as a tortuosity or a kink. Tortuosity of the carotid artery is rarely seen in otolaryngologic practice. In videolaryngoscopic examination, bilateral pyriform sinus narrowing was found in one patient and unilateral pyriform sinus narrowing in the other patient. Computed tomographic scans showed tortuous internal carotid artery in both patients. Awareness of the possible internal carotid artery tortuosity is very important to prevent serious complications during laryngeal and pharyngeal surgical procedures.

Bilateral internal carotid absence: a case report of a rare congenital anomaly.

BACKGROUND: Bilateral internal carotid artery agenesis (ICAA) is a rare developmental anomaly of unknown etiology that is often associated with disruption of adequate perfusion to the central nervous system. Nevertheless, some patients remain asymptomatic due to collateral circulation involving the communicating arteries of the Circle of Willis. Secondary to the hemodynamical stress through the collateral circulation, affected patients are at an increased risk of developing subarachnoid hemorrhage and intracranial aneurysms. METHODS AND RESULTS: We report an unusual case of a 62-year-old man with bilateral ICAA who expired following two asystolic events during minor surgery. CONCLUSION: This case emphasizes the plasticity of the cerebral collateral circulation during development, to the point of normal cerebral perfusion throughout life with no ischemic complications.

Collateral blood flow patterns in patients with unilateral ICA agenesis and cerebral aneurysm.

BACKGROUND: Agenesis of one or both internal carotid arteries (ICA) is usually a benign congenital anomaly. Many patients are diagnosed incidentally and remain asymptomatic, however associated cerebral aneurysms can be life-threatening and result in high morbidity and mortality rates in this population. MATERIAL & METHODS: Based on the timing of ICA agenesis, during the fetal life, we classified the collateral blood flow pattern into three major types; type I: Collateral blood flow via primitive vessels, type II: Collateral blood flow via ICA branches, and type III: collateral blood flow via branches of the external carotid artery (ECA). The type of collateral blood flow pattern in the reported patients with ICA agenesis and cerebral aneurysm was reviewed in order to determine the relation between the type of collateral blood flow and the development of CAs. RESULTS: Twenty nine patients with ICA agenesis and CA were reported, of these 27 patients (93%) were found to have type II collateral blood flow, 2 patients (7%) had type I collateral blood flow pattern and none had type III collateral flow pattern. The majority of patients (79%) with CA secondary to ICA agenesis presented with subarachinoid hemorrhage (SAH). CONCLUSION: Although ICA agenesis is a symptomatic in the majority of cases, it must be recognized because it promotes the development of CA and SAH. Our new classification system for collateral blood flow patterns is easy to use and can predict those at high risk to develop cerebral aneurysms and SAH, and therefore, need annual screening MRA.

Association between internal carotid artery morphometry and posterior communicating artery aneurysm.

PURPOSE: The goal of this study was to directly measure the association between the internal carotid artery (ICA) morphometry and the presence of ICA-posterior communicating artery (PCOM) aneurysm. MATERIALS AND METHODS: The authors intraoperatively measured the length of the supraclinoid ICA because it is impossible to radiologically determine the exact location of the anterior clinoid process. We used an image analyzer with a CT angiogram to measure the angle between the skull midline and the terminal segment of the ICA (ICA angle), as well as the diameter of the ICA. The lengths and diameters of the supraclinoid ICA and the ICA angle were compared among PCOM aneurysms, anterior communicating artery (ACOM) aneurysms, and middle cerebral artery (MCA) bifurcation aneurysms (n=27 each). Additionally, the lengths and the diameters of M1 and A1 were compared for each aneurysm. RESULTS: The lengths of the supraclinoid ICA were 11.9+/-2.3 mm. The lengths of the supraclinoid ICA in patients with ICA-PCOM aneurysms (9.7+/-2.8 mm) were shorter than those of patients with ACOM aneurysms (13.8+/-2.2 mm, Student's t-test, p<0.001) and with MCA bifurcation aneurysms (12.2+/-1.9 mm, Student's t-test, p<0.001). The diameters of the supraclinoid ICA and A1 in patients with ACOM aneurysms were larger than those in patients with MCA bifurcation aneurysms (Student's t-test, p<0.05). There were no significant differences in the lengths of M1 and A1, ICA angle, or diameter of M1 for each aneurysm. CONCLUSION: These results suggest that the relatively shorter length of the supraclinoid ICA may be a novel risk factor for the development of ICA-PCOM aneurysm with higher hemodynamic stress.