Abstract
Aortic pseudocoarctation is a rare congenital anomaly characterized by elongation and deformity of the aortic arch and is known to be associated with aneurysmal formation. Several studies unite to say it leads to a surgical sanction as soon as symptomatic or associated with aneurysms of the aortic arch. Our patient is a 12 years old boy, followed since birth for a little tight pseudocoarctation with a cervical aortic arch and transverse aortic arch hypoplasia. Close clinical and paraclinical monitoring including angioscans, showed the gradual enlargement of the superior mediastinum, in relation with the appearance of three aneurysms of the aortic arch. The intervention, performed by sternotomy, has consisted of the resection of the aneurysmal area and the interposition of a Dacron tube to repair the aortic arch and the reimplantation of the left subclavian artery into the left carotid artery. The postoperative course was uneventful. Management of pseudocoarctation associated with cervical aortic arch and aneurysms remains surgical. Close monitoring of patients with pseudocorctation, seems to be essential to avoid fatal complications such as aneurysmal rupture.
Highlights
Pseudocoarctation is a rare congenital anomaly defined as kinking of the aorta with normal blood flow dynamics [1], characterized by elongation and deformation of the aortic arch similar to classic coarctation, but in which no obstruction is demonstrable neither collateral circulation [2]
We report an uncommon case of pseudocoarctation associated with cervical aortic arch and 3 aneurysmal formations of the aorta, and we focus on the interest of close monitoring of these patients to avoid complications such as aneurysmal rupture
During the follow-up, including physical exams, chest radiographs and annual CT scan, we found an enlargement of the mediastinal mass and an obvious protrusion of the aortic knuckle with a cardiothoracic ratio at 45%
Summary
Pseudocoarctation is a rare congenital anomaly defined as kinking of the aorta with normal blood flow dynamics [1], characterized by elongation and deformation of the aortic arch similar to classic coarctation, but in which no obstruction is demonstrable neither collateral circulation [2]. We report an uncommon case of pseudocoarctation associated with cervical aortic arch and 3 aneurysmal formations of the aorta, and we focus on the interest of close monitoring of these patients to avoid complications such as aneurysmal rupture. A chest CT scan was performed in June 2013; at the age of 11years and showed a relative hypoplasia of the proximal segment of the aortic arch at 10 mm, a cervical aortic arch, three transverse aortic aneurysms (Figure 1) between the left carotid artery and a low implanted left subclavian artery, with a kinking of the aortic isthmus (Figure 2). We clamped the descending thoracic aorta and cut the distal aortic arch, we noted that the aneurysm wall was extremely thin nearly to rupture; we made an end-to-end anastomosis between a 14 mm Dacron tube and the descending thoracic aorta. During the operation we noticed the total absence of collateral circulation, the aortic arch was reaching the left clavicle and there was no aortic lumen stenosis
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