Case Report

A 2-year-old boy with Down syndrome first presented to another institution with a history of stridor, fever, and coryzal symptoms. His symptoms were unresponsive to nebulised budesonide and adrenaline. He developed severe respiratory distress and carbon dioxide retention and required intubation and mechanical ventilation. His condition continued to deteriorate, and on day 2 of admission he was transferred to our unit and placed on venoarterial extracorporeal membrane oxygenation, ECMO. Direct laryngoscopy, bronchoscopy, and computed tomography showed a circumferential long segment tracheal stenosis in the mid trachea measuring 3 centimetres in length, with 1 ${\scale50%{\vskip-6pt1}{\hskip-2.1pt\vskip-4.1pt\rot162\scale70%/}{\scale50%{\hskip-4.1pt\vskip0pt2}$ centimetres of normal proximal trachea and 2 ${\scale50%{\vskip-6pt1}{\hskip-2.1pt\vskip-4.1pt\rot162\scale70%/}{\scale50%{\hskip-4.1pt\vskip0pt2}$ centimetres of normal distal trachea (Figs 1 and 2). The trachea was critically stenotic measuring 3 millimetres in diameter.

Figure 1

Computed tomogram image in coronal view demonstrating long segment critical tracheal stenosis.

Figure 2

Computed tomogram image in axial views demonstrating severe degree of tracheal stenosis with ECMO cannula in the superior vena cava and carotid artery. Note the size of the trachea relative to the nasogastric tube.

A median sternotomy and pericardiotomy was performed and the child was placed on cardiopulmonary bypass. Heparin was administered. The right atrium, inferior vena cava, and aorta were cannulated. He was decannulated from extracorporeal membrane oxygenation, and the right internal jugular vein and right carotid artery were repaired. A right atriotomy was performed and the left atrium was vented. The zone of apposition of the left atrioventricular valve was closed with 5/0 surgipro, and the primum atrial septal defect was closed with fresh autologous pericardium. The secundum atrial septal defect was closed primarily. The right atrium was closed in two layers; the heart was deaired and reperfused.

The trachea was dissected from the hyoid to the carina. A hyoid release procedure was performed by dividing the strap muscles superiorly, and the inferior pulmonary ligaments were divided to allow a tension-free anastomosis in the trachea. Tracheal rings were excised two centimetres and a slide tracheoplasty was performed with extended end-to-end anastomosis, with 6/0 Polydioxanone suture continuously posteriorly and interrupted anteriorly. The endotracheal tube was advanced beyond the anastomosis and Liga clips were placed as markers on either side of the anastomosis. He weaned easily off cardiopulmonary bypass; haemostasis was achieved and the surgical wounds were closed. A decision was made to excise 2 centimetres of trachea as well as to perform a slide tracheoplasty, as it was felt that this would optimise the final tracheal diameter. A standard technique of a posterior–superior incision and an inferior–anterior incision in the trachea was used to complete the slide tracheoplasty.

The post-operative course was complicated by an anterolateral myocardial infarction, which became apparent 24 hours after surgery with elevated cardiac enzymes, acute decrease in blood pressure, and ST segment changes on electrocardiogram. Transthoracic echocardiography demonstrated a right atrial thrombus. At 36 hours post-surgery, it became apparent that he had sustained a diffuse left-sided cerebral infarction causing right-sided hemiplegia. The myocardial and cerebral infarctions were presumed to be secondary to embolic events related to the right atrial thrombus, which developed during extracorporeal membrane oxygenation preoperatively, despite adequate anticoagulation. He was extubated 7 days post-operatively. He was transferred from the intensive care unit 2 weeks after surgery. He subsequently developed a clinically silent aspiration syndrome and required a percutaneous endoscopic gastrostomy tube insertion. His post-operative medications included diuretics and warfarin therapy. He was discharged home 2 months later. His right-sided hemiplegia improved with physiotherapy; his gastrostomy tube was removed at 3 ${\scale50%{\vskip-6pt1}{\hskip-2.1pt\vskip-4.1pt\rot162\scale70%/}{\scale50%{\hskip-4.1pt\vskip0pt2}$ years of age and he is now doing well. He has no residual respiratory symptoms, and follow-up bronchoscopy showed no evidence of recurrent tracheal obstruction 6 months after surgery.