Double aortic arch, the most common form of
vascular rings, is a congenital anomaly that occurs,
when the ascending aorta is divided into two parts and passes around the trachea and esophagus and, then,
turns into a single pattern aorta.[
4]
The embryological development of the vascular
ring has been described in detail in the literature.
In the embryonic arch system, there are six parts of
the aortic arch originating from the ventral aortic
root. The first, second, and fifth arches regress
and a DAA is formed. In general, the left fourth
arch forms the aortic arch. If the left fourth arch
regresses and the right arch remains, this results in
a right-sided aortic arch.[4] In DAA, due to errors
in the regression of the right aortic arch, both
aortic arches form a vascular ring that completely
surrounds the trachea and esophagus. Classically, there are three types of DAA: right dominant aortic
arch, left dominant aortic arch, and balancedtype
aortic arch. In 75% of cases, the right arch
is dominant and, in about 20% of cases, the left
arch is dominant. In 5% of the cases, both arch
dominance is equal.[5] In our case, the patient had a
balanced-type aortic arch.
Double aortic arch may cause compression of
these structures by surrounding the trachea and
esophagus. Symptoms such as respiratory distress,
feeding difficulties, recurrent pneumonia, stridor
and dysphagia may occur as a result of tracheal
and/or esophageal compression at birth and in early
infancy.[6] However, partial compression may show an
asymptomatic course, as in our patient.
Chest X-ray may be normal and diagnosis may
be missed. Various imaging methods, such as
magnetic resonance imaging (MRI), CT scanning,
and aortography, can be used to reveal DAA and
determine the most optimal surgical approach for
surgical candidates.[7]
The presence of symptoms for vascular rings
is a surgical indication at all ages. In particular,
adults without symptoms may not need surgery.
Nevertheless, we argue that, cases similar to our
patient may need a close follow-up with regular
laboratory studies and proper physical examination.
Surgical delay may cause serious morbidity and
irreversible complications of the respiratory system,
leading to death even.[8] Medical therapy is a choice
that can contribute to surgical treatment. Surgery for
aortic arch division can be accomplished through a
right or left thoracotomy and it can reach out to relieve
tracheoesophageal structures. Maximum effort is
required to avoid vascular and neighboring structures
for acceptable postoperative results. Despite this,
long-term surveillance after surgery is 90 to 95% of
all cases. Particularly asymptomatic adults may not
need surgery.[8]
In conclusion, although double aortic arch is well
described in childhood it is rare in adult patient
because of its asymptomatic course. Surgery may be
necessary if it causes symptoms due to tracheal and
esophageal compression otherwise patients should be
followed up under regular medical follow-up.
Declaration of conflicting interests
The author declared no conflicts of interest with respect
to the authorship and/or publication of this article.
Funding
The author received no financial support for the research
and/or authorship of this article.
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