Isolation of right internal carotid artery, persistent proatlantal 1 artery and rete mirabile in a child with 22q11 deletion syndrome

Abstract

We report a case of a 2-year-old girl with 22q11 deletion syndrome who underwent studies for cardiac murmur. Ultrasound, computed tomography angiography and digital subtraction angiography revealed an aberrant right subclavian artery and an isolated right internal carotid artery (ICA) originating from the right pulmonary artery. A right carotid rete mirabile (CRM) and a proatlantal (Pa) type 1 artery were also found. We hypothesize that Pa type 1 persistence and CRM development are secondary to the reversal of flow of the isolated ICA.

Isolated left common carotid artery: multidisciplinary management is the key to success

“…Isolation of the carotid artery is a rare form of aortic arch anomaly, with only 15 cases reported thus far (Table 1). [2][3][4][5][6][7][8][9][10][11][12][13][14][15][16] All except one case had a right aortic arch with an isolated left carotid artery. 14 In three patients, only the internal carotid artery was isolated, and the external carotid artery was arising from the aorta (8,11,14).…”

“…[2][3][4][5][6][7][8][9][10][11][12][13][14][15][16] All except one case had a right aortic arch with an isolated left carotid artery. 14 In three patients, only the internal carotid artery was isolated, and the external carotid artery was arising from the aorta (8,11,14). In all these patients, the ipsilateral subclavian artery was aberrant.…”

“…However, the presence of independent ipsilateral arterial duct or ductal ligament in 9 out of 15 reported cases (60%) as well as in the index case makes the connection between isolated carotid artery with the pulmonary artery unlikely to be via an arterial duct. 3,7,9,10,[12][13][14][15][16] Some authors have proposed an alternative hypothesis of malseptation of the trunco-aortic sac to explain the isolation of the aortic arch artery, especially the carotid artery. 6,9,16,19 Although not reported previously, the coexistence of anomalous origin of right coronary artery from the isolated left common carotid artery in our case also favours the theory of malseptation of the trunco-aortic sac (Fig 9).…”

“…2,3,13,15 Although the lower end of the isolated carotid artery can be closed using an occluder device, surgical ligation is preferred as it would also permit ligation and release of arterial duct or ductal ligament. 14 Two infants underwent concomitant aorta-pulmonary shunt for TOF/ pulmonary atresia. 7,15 Ligation of the isolated carotid artery is an attractive option and would also avoid the risk of cerebral hyperperfusion syndrome but the stump of the carotid artery distal to ligation may have the risk of thrombogenesis.…”

“…The isolated carotid artery is the rarest form of arch vessel isolation, with only 15 cases reported thus far. [2][3][4][5][6][7][8][9][10][11][12][13][14][15][16] Surgical reimplantation of the isolated carotid artery is the preferred option. Nonetheless, the risk of post-operative cerebral hyperperfusion syndrome mandates careful preoperative evaluation and perioperative management.…”