Discussion
This case illustrates how 3D printing can improve patient education and
amplify understanding by clearly displaying all relevant anatomy in one
modality. It was difficult for this patient and her family to fully
understand the three dimensional anatomy of a circumflex aorta with
verbal descriptions alone.
Originally discussed in a case report by Paul in 1948, Circumflex aorta
was further described by the physician Raymond Heim de Balsac in 1960
with correspondence added by the pathologist Jessie Edwards (2,3).
Diagnoses depended at that time on combining multiple indirect
modalities such as an esophagrams.
Circumflex aorta can occur in the setting of either a left or right
aortic arch. The diagnosis requires the aortic transition in sidedness
occur above the level of the carina. An operation to correct circumflex
aorta was first described by Planche and LaCour-Gayet in 1984 (4).
Subsequently a descriptive account using circulatory arrest was
described by Russell et al (1). A clear understanding of the multiple
structures manipulated to achieve symptomatic success is necessary. Key
elements of a successful operation are avoidance of recurrent laryngeal
nerve injury, lymphatic disruption leading to chylothorax, and vascular
injury and hemorrhage during the division and translocation of the
aorta.
Although rare, presentation is usually that of a child who has
persistent symptoms after initial treatment of division of ligamentum
arteriosum with or without an atretic distal arch. We would recommend a
staged treatment pathway, as most patients will have effective relief of
pathology by division of the ligamentum and atretic distal arch, if
present. The aortic uncrossing procedure is reserved for those with
persistent symptoms and evidence of ongoing airway impingement after
initial repair. Postoperative follow up and reinvestigation are
necessary to rule out continued anatomical causes of tracheomalacia and
symptomatic compression in the setting of continued symptoms after
division of vascular rings. The ubiquity of CT scanners makes
re-investigation relatively simple.
3D printing has proved to be a useful adjunct to anatomical imaging.
Although it may be considered redundant to 2D depictions of computer
generated 3D constructs for surgical planning, its use in patient
education can be quite impactful. In our case 3D modeling helped gain
the trust and understanding of a family that had already gone through
one operation, and was faced with a much larger operation with a higher
risk of complications. Appropriately, there were doubts on the validity
of the data presented through traditional methods such as 2D images and
description of physiologic tests. Alleviating these doubts was aided
with the use of a 3D model.