Tracheal agenesis: Esophageal airway support with a 3-dimensional–printed bioresorbable splint

To interrupt positive-pressure ventilation of the stomach, the lower esophagus was separated from the upper esophageal airway. Via laparotomy, the esophageal hiatus was opened, a paraesophageal dissection performed, the esophagus divided below the fistula with an endoscopic stapler, and the distal esophageal remnant was brought into the abdomen. The stomach was decompressed with gastrostomy tube placement. Duodenal obstruction was appreciated but treatment deferred.

Computed tomography (CT) imaging later confirmed (DOL 6) Floyd type I tracheal agenesis with a 2- to 4-mm diameter fistula connecting the mid-esophagus to a short tracheal segment. Duodenal obstruction and malrotation were addressed (DOL 8) with duodenojejunostomy and a Ladd procedure. Mucus plugging of the fistula occasionally necessitated bedside bronchoscopic clearance of secretions.

To provide a secure airway and divert oral secretions, the patient underwent neck dissection and division of the esophagus. The upper esophagus was diverted to a right cervical esophagostomy (“spit fistula”), and the middle esophagus externalized as a stoma above the sternal notch (airway esophagostomy, or “pseudo-tracheostomy”). The stoma was intubated with a 4.5-Fr, 44-mm extended tracheostomy tube.

The patient remained sedated and ventilated with positive pressure to minimize airway collapse. Gastrostomy feeds were initiated. Bedside bronchoscopy was intermittently performed for maintenance clearance of mucus.

A multidisciplinary team discussed the options for airway reconstruction. After evaluation of the reported experience and existing technologies, the decision was made to externally support the esophageal airway with a 3D-printed splint composed of slowly bioresorbable polycaprolactone (PCL). Similar splints had been successfully used to manage tracheobronchomalacia.

Permission for use of the device was obtained from the US Food and Drug Administration under Expanded Access, Penn State University institutional review board (Protocol No. 2018-002SU, March 28, 2018), and the Pennsylvania Department of Health. Using CT data imported into computer-aided design software, a virtual anatomic model was created and used for custom design of the airway device. Splints were 3D-printed using from 96% polycaprolactone and 4% hydroxyapatite and sterilized with ethylene oxide according to established processes (Nelson Laboratories, Salt Lake City, Utah). Additional benchtop fit-testing was performed with life-size 3D-printed splints and airway models.

After we obtained informed consent from the parents of the patient including the use of the 3D-printed splint under expanded access and manuscript publication related to the case, definitive airway reconstruction was performed through a median sternotomy with cardiopulmonary bypass support. Repair of the complete atrioventricular canal defect was accomplished first using a single-patch technique with cleft closure; epicardial echocardiography showed no residual shunts and trace-to-mild atrioventricular valve regurgitation. Airway reconstruction followed (Figure 1, Video 1). The esophagus, fistula, and mainstem bronchi were mobilized with preservation of the vagus nerves and vascular supply. A 15-mm inner-diameter, 41-mm long PCL splint was selected and trimmed. The tracheoesophageal fistula was sharply resected (Figure 2, A). Incisions were carried from the lateral trachea onto each bronchus. The esophagus was divided and the distal remnant removed. An esophagotracheostomy anastomosis was fashioned using interrupted sutures (6-0 polydioxanone). Subsequently, partial-thickness mattress sutures (6-0 polypropylene) were placed in 5 longitudinal rows along the anterior and lateral aspects of the esophagus. The sutures were directed through the graft interstices, the splint parachuted down onto the airway, and the sutures tied. Bronchoscopy showed a large internal lumen without collapse during provocative suctioning. Cardiopulmonary bypass was concluded, the sternum closed, and the infant recovered to the intensive care unit.

Bronchoscopy for pulmonary toilet was performed daily during the first week and then less frequently; even in the absence of positive pressure, airway collapse was not observed (Figure E2). CT of the chest at 8.6 months showed a well-expanded esophageal airway without stenosis (Figure 2, B). At 11 months, the airway esophagostomy stoma was revised to address minor breakdown and the cervical esophagostomy dilated. The patient progressed and was discharged home at 13 months on a home ventilator program. CT of the chest at 23 months continued to demonstrate favorable airway anatomy (Figure 2, C); subsequent axial imaging has not been indicated. The patient eventually liberated from continuous mechanical ventilation at 2.4 years, spontaneously breathing on her own in the daytime with a heat-moisture exchanger and tracheostomy tube (Bivona 5-mm tracheostomy tube, 60-mm length; Smiths Medical, Minneapolis, Minn), and resting overnight on ventilator support for safety.

Gastrointestinal continuity was re-established using a colon interposition graft to connect the cervical esophagus to the stomach. Working through the neck, right chest, and abdomen, the cervical esophagus and right colon were extensively mobilized and an intrathoracic anastomosis performed with interrupted sutures (4-0 polydioxanone). A pyloroplasty was added to augment gastric emptying. An upper gastrointestinal contrast study 6 weeks later showed an intact colon interposition graft without leak or obstruction (Figure E3).

At latest follow-up (age 3.4 years), the patient is alive and well and remains off ventilator support during the day. With speech therapy, she is progressing toward oral consumption and is taking soft pureed foods by mouth. Aphonic, she uses basic sign language to communicate. Although developmentally delayed, she is crawling and cruising.