Congenital Anomalies of the Internal Carotid Artery
Summary
Congenital anomalies of the internal carotid artery encompass a spectrum of rare developmental defects—most notably agenesis, hypoplasia and aplasia—that arise from aberrant embryological formation of the third aortic arch or dorsal aortic root. Occurring in fewer than 0.01 % of individuals, these anomalies may remain clinically silent due to compensatory collateral circulation through the circle of Willis or external carotid branches. However, altered haemodynamics in these collateral pathways can predispose to ischaemic stroke, subarachnoid haemorrhage and aneurysm formation. High‐resolution non-invasive imaging modalities such as computed tomography angiography and magnetic resonance angiography, together with digital subtraction angiography when intervention is contemplated, are essential for accurate diagnosis. Early recognition informs risk stratification, guides endovascular or surgical management and mitigates long-term cerebrovascular complications, underscoring the global significance of these vascular variants and their impact on patient outcomes.
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Congenital Anomalies of the Internal Carotid Artery publication trend
The graph below shows the total number of articles in congenital anomalies of the internal carotid artery across all publications each year (not limited to Nature Index journals).
Technical terms
Agenesis: complete absence of the internal carotid artery due to failure of embryonic development.
Hypoplasia: underdevelopment or reduced calibre of the internal carotid artery relative to normal anatomy.
Collateral circulation: alternative vascular pathways that maintain cerebral perfusion when primary arteries are absent or narrowed.
Computed tomography angiography: cross-sectional imaging technique using X-ray contrast to visualise arterial structures.
Digital subtraction angiography: invasive imaging method that captures blood vessels by subtracting pre-contrast images from post-contrast images.
References
- Aplastic Internal Carotid Artery: A Potentially Catastrophic Vascular Anomaly. Diagnostics (2023).
- Cerebrovascular disorders associated with agenesis of the internal carotid artery: Findings on digital subtraction angiography. Frontiers in Surgery (2022).
- Bilateral Internal Carotid Artery Agenesis in a Patient With a Family History of Intracranial Pathology. Ochsner Journal (2022).
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