Cervical aortic arch

Cervical aortic arches are a rare aortic arch anomaly characterized by an elongated, high-lying aortic arch extending at or above the level of the medial ends of the clavicles.

Clinical presentation

Patients with cervical aortic arch are usually asymptomatic. Symptomatic patients may present with ^1-13:

• palpable supraclavicular mass
• dysphagia
• wheezing
• coughing
• hoarseness
• stridor
• choking
• apneic spells
• chest pain
• recurrent pulmonary infection

Pathology

A cervical aortic arch develops most likely from the persistence of the embryonic third aortic arch instead of the fourth ^1-13.

Classification

According to Haughton and colleagues, there are five distinct forms of cervical aortic arch, which are classified according to the configuration of the aorta, sequence of brachiocephalic branching, and embryogenesis ¹:

• type A: contralateral descending aorta and absence of one common carotid artery
  • has separate external and internal carotid artery branches from the aortic arch
  • results from persistence of the entire third primitive aortic arch on one side and involution of the contralateral ductus caroticus and of the fourth aortic arches bilaterally
• type B: contralateral descending aorta and presence of both common carotid arteries
  • dual common carotid arteries
  • development is similar to type A but with formation of common carotid arteries rather than separate internal and external carotid arteries proximally
• type C: contralateral descending aorta and bicarotid trunk
  • usually left cervical arch with a right-sided descending aorta
  • results when the distal aorta is connected with a right-sided ductus arteriosus and the transverse aorta has migrated abnormally
  • cases have a common trunk from which right and left common carotids originate (bicarotid trunk)
• type D: ipsilateral descending aorta with normal sequence of brachiocephalic branching
  • most common
  • forms when the process of involution is conventional but the aortic segment between the left common carotid and left subclavian arteries is excessively long
  • usually left cervical arch with a normal branching pattern, redundant transverse aorta and left-sided often hypoplastic descending aorta
  • redundancy of the aorta, high position of the transverse aorta, and a predilection for hypoplasia of the descending aorta are features common to both the type D cervical aortic arch and pseudocoarctation of the aorta
• type E: right aortic arch and right descending aorta 
  • formed by development of a right arch and failure of migration of the apex of the arch to the thoracic region
  • with an aberrant left subclavian artery

Associations

Reported associations include:

• aneurysms
  • in approximately 20% of cases ^5,7
  • aneurysm formation predominantly occurs in women ¹³
  • pathophysiology is not clear but possible explanations include:
    • abnormal embryologic development
    • abnormal connective tissue
    • altered hemodynamics
    • aortic wall stress
    • atherosclerotic disease
    • trauma
• coarctation of the aorta
• aortic pseudocoarctation
• congenital cardiac anomalies
• Di George syndrome ¹¹
• Turner syndrome ¹¹

Radiographic features

Plain radiograph

• the high-riding arch may be seen as a mass-like paratracheal soft tissue density
• normal aortic knob may be absent
• widened mediastinum
• compression and displacement of the trachea and esophagus may be demonstrated

Echocardiography

Echocardiography may demonstrate color flow jets in opposite directions indicative of tortuous aneurysmal dilatation of the aortic arch at the cervical position ¹⁰. 

Angiography: CTA/MRA/DSA

Will allow direct visualization of arch anatomy and associated anomalies.

Treatment and prognosis

Surgical treatment is commonly indicated for the relief of symptoms resulting from compression of the trachea and esophagus, correction of associated intracardiac defects, repair of associated aneurysms and aortic obstruction ^2,3. 

History and etymology

DG Reid first reported the first case of cervical aortic arch in 1914 ^12,13.

Differential diagnosis

Possible differential considerations for certain variants include

• aortic pseudocoarctations
• chronic Takayasu arteritis
• thoracic aortic aneurysms