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Aortopexy is indicated in young patients with life-threatening tracheal obstruction secondary to severe tracheomalacia. The tracheomalacia may be primary or secondary. The secondary form is often seen in combination with esophageal atresia (EA) and vascular rings. It has also been described in combination with congenital heart disease. Symptoms vary from mild and not requiring therapy, to severe and not allowing extubation, to producing acute life-threatening events (ALTEs). Randomized controlled trials regarding the different treatment modalities do not exist. Continuous positive airway pressure ventilation is occasionally effective but is not a viable long-term therapy. Bronchial stenting has not gained popularity because of its high failure rate and sometimes more severe morbidity and mortality.
Aortopexy is a relatively straightforward operation that results in immediate relief of symptoms in about two-thirds of cases. There is little doubt that this approach is the treatment of choice in severely symptomatic infants. Moreover, the thoracoscopic technique is much less traumatic than other operative approaches, such as left thoracotomy or a low cervical–upper sternotomy approach.
Indications for Workup and Operation
Aortopexy is indicated in cases of life-threatening apnea due to tracheomalacia. In infants with severe symptoms, once this diagnosis is made, not much time should be lost before the procedure is performed.
The diagnosis of tracheomalacia is made on clinical grounds and confirmed by tracheobronchoscopy. A lateral chest radiograph can sometimes show the narrowed trachea in the thoracic aperture. In children with EA and distal fistula, clinical tracheomalacia can be documented in almost all babies. However, only a small percentage require aortopexy. Tracheomalacia can also be seen in babies with aortic arch anomalies.
We prefer to perform rigid tracheobronchoscopy under general anesthesia, but with the patient spontaneously breathing to thoroughly evaluate and control the airway. This should be done prior to determining if the child needs an aortopexy. Moreover, a flexible bronchoscope is generally used during the procedure to determine the effectiveness of the aortopexy.
The young patient is placed supine on the operating table. The midsternal line is marked on the skin ( Fig. 38-1A ). The left chest is elevated about 15 degrees, and the operating table is tilted in reverse Trendelenburg ( Fig. 38-1B ). The surgeon stands to the left of the patient with the camera operator on the surgeon’s left and the scrub nurse at the caudal end of the short operating table ( Fig. 38-2 ). The most important monitor is positioned in front of the surgeon, at the right side of the patient. The equipment tower is placed behind the surgeon or on the right side of the table, adjacent to the monitor.
The procedure is usually performed with standard tracheal intubation, using CO 2 insufflation to collapse the lung and create adequate working space. This also allows for the placement of a flexible bronchoscope through the endotracheal tube during the procedure to evaluate the success of the aortopexy. A right mainstem intubation can be used, but the tube would need to be pulled back at the time of bronchoscopy for evaluation of the aortopexy.
The procedure is started by inserting a Veress needle in the fourth or fifth intercostal space in the anterior axillary line (roughly at the level of the nipple). A low-flow (1 L/min) and low-pressure (6 mm Hg) CO 2 insufflation are used to create a mild tension pneumothorax to collapse the lung. The first port, a 4-mm cannula, is inserted at this site. A piece of red rubber catheter is placed on the cannula and sutured to the skin to prevent slippage ( Fig. 38-3A ). A 4-mm 30-degree lens is then introduced through this port. A 5-mm port and scope can also be used per surgeon preference. If necessary, the insufflation pressure can be increased to 8 mm Hg to achieve adequate lung collapse. Next, the two working ports are inserted, one 3-mm port for the surgeon’s left hand and a 5-mm radially expandable cannula for the right hand. The surgeon’s left-hand port is one interspace lower and more medial, and the right hand port is placed two to three interspaces higher in the axilla ( Fig. 38-3B ). The working angle between the ports should be about 60 to 90 degrees. These cannulas can also be secured to the chest wall to prevent dislodgment.