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.