1981
DOI: 10.1007/bf00975188
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The cervical aortic arch: A case with diffuse arterial dysplasia and neurocutaneous angiomatosis

Abstract: The authors present a case of cervical aortic arch which was discovered in a child of 5 years. The association with a diffuse and major vascular dysplasia, mainly neuroectodermic, is worth reporting. Only 40 cases of this abnormality have been published up to now. The anatomic classification of the different types of cervical aortic arch is presented.

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Cited by 13 publications
(3 citation statements)
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“…Three of these cases were related to surgery for treating CHD without the CAA ( 14 , 27 , 39 ), while only one case focused on surgery for isolated CAA ( 2 ). The three non-operated patients experienced different causes of death: one patient died due to acute cerebral hemorrhage ( 6 ), another patient died due to sepsis with deranged coagulation, renal failure, and cardiopulmonary failure ( 50 ), and the third patient experienced unexpected death 24 h after undergoing cerebral arteriography, with no identifiable cause ( 17 ). After surgery, only four patients (among the symptomatic), all with a vascular ring, did not completely resolve their symptoms, which persisted in a milder form than prior to intervention ( 6 , 19 , 39 , 54 ).…”
Section: Resultsmentioning
confidence: 99%
“…Three of these cases were related to surgery for treating CHD without the CAA ( 14 , 27 , 39 ), while only one case focused on surgery for isolated CAA ( 2 ). The three non-operated patients experienced different causes of death: one patient died due to acute cerebral hemorrhage ( 6 ), another patient died due to sepsis with deranged coagulation, renal failure, and cardiopulmonary failure ( 50 ), and the third patient experienced unexpected death 24 h after undergoing cerebral arteriography, with no identifiable cause ( 17 ). After surgery, only four patients (among the symptomatic), all with a vascular ring, did not completely resolve their symptoms, which persisted in a milder form than prior to intervention ( 6 , 19 , 39 , 54 ).…”
Section: Resultsmentioning
confidence: 99%
“…Three theories are advanced as a cause of CAA: (1) persistence of the more superiorly located 3 rd branchial arch and the ductus caroticus (segment of dorsal aorta between the 3 rd and 4 th aortic arch) with disappearance of the fourth arch on the side of the aortic arch-this theory explains the separate origin of the external and internal carotid arteries noted occasionally, (2) fusion of the 3 rd and 4 th arches, and 3) absence of the caudal migration of the 4 th arch-the branching is normal in this case. 8 The latter two explain the abnormally long distance between the left common carotid and the left subclavian which predisposes the patient to a true or pseudocoarctation. 4 22 q11 deletions have been described in CAA with and without conotruncal anomalies.…”
Section: Discussionmentioning
confidence: 99%
“…Aneurismatic dilation, kinking or stenosis of CAA also suggests the presence of primary developmental disease of arterial wall. Moreover histological analysis documented the association of degenerative changes in the arterial wall of CAA and beyond, such as mediocystic necrosis [38][39][40].…”
Section: Discussionmentioning
confidence: 99%