Congenital external carotid-internal carotid artery anastomosis: a report of three cases and literature review

Purpose: Congenital external carotid-internal carotid artery (EC-ICA) anastomosis is a cervical arterial variation that was initially reported, based on anatomic dissection, from Japan in 2000. The purpose of this report is to show the characteristic radiological findings of this extremely rare cervical arterial variation. Methods: We analyzed nine cases, including six previously reported cases and three cases that we recently experienced. Three of the six previously reported cases were from Japan, and the remaining three cases were from Korea. MR angiography (4), CT angiography (2), catheter angiography (2) and autopsy (1) were used as diagnostic modalities. Results: The study population included eight men and one woman. Anastomosis was seen on the left side in seven cases and the right side in two cases, and it was located at the level of the C1/2-C2/3 intervertebral space. In all cases, ECA was larger than the ICA at the level of their origins. In four cases, the ICA was extremely small in caliber. In one case, the ICA may have been occluded postnatally, and its configuration was similar to that of the nonbifurcating cervical carotid artery (CCA). With the exception of this occluded case, the carotid bifurcation and EC-ICA anastomosis formed a large arterial ring in all cases. Conclusion: EC-ICA anastomosis can be regarded as a variant of the nonbifurcating CCA because if the most proximal segment of the small ICA is occluded, nonbifurcating CCA may form. EC-ICA anastomosis is located between the C1/2-C2/3 intervertebral space and may be formed by the remnants of the proatlantal artery I.