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Some of our patients come to us as newborns in respiratory distress. In such cases, the ear, nose and throat (ENT)/airway team immediately stabilizes the young patient’s breathing, evaluates the airway, and works with the intensive care unit team to keep him or her safe and breathing well until surgery, which may occur within hours or days. These patients often also have chest CT angiograms and echocardiograms to evaluate their heart and lungs.
Other patients are referred to us because they are known to have cardiac defects frequently associated with a tracheal stenosis, even though those patients may not yet be symptomatic or diagnosed with a stenosis. For example, patients with a pulmonary artery sling, in which the misdirected left pulmonary artery passes between the trachea and the esophagus, frequently also have a tracheal stenosis. These patients should undergo a comprehensive airway evaluation prior to cardiac surgery. If patients are not adequately evaluated beforehand, the tracheal stenosis may be discovered only when heart surgery is already underway or even after it is complete. This may make airway reconstruction far more dangerous and difficult to perform. “Here at Stanford Medicine Children’s Health, we do whatever we possibly can to identify all the issues up front and to do the heart or vascular surgery and the airway surgery at the same time,” says Douglas Sidell, MD, the lead otolaryngologist on the team.
Although there are rare acquired forms of tracheal stenosis, the most common type of congenital stenosis among children is caused by a condition called complete tracheal rings. Normally, the horseshoe shape of the cartilage in the trachea keeps the airway from collapsing, and the soft back wall (in the open part of the horseshoe) of the airway allows it to expand during breathing. Patients with complete tracheal rings do not have the ability to expand the airway but instead have narrow, circular pieces of cartilage that completely surround and constrict the airway. This can cause severe and dangerous respiratory distress.
Some complete tracheal ring patients are asymptomatic when we first see them. Surgery for many of these patients may be safely delayed or avoided. But even in asymptomatic cases where there is no critical cardiac component, the patient should still have periodic bronchoscopies and clinic visits to ensure that the airway remains open and viable. These visits vary in frequency depending on the patient’s age, the severity of the stenosis, and other risk factors.
Unless a patient requires immediate surgery, his or her case is reviewed in a weekly cardiothoracic/cardiology conference where each case is examined and discussed by our multidisciplinary team. Working closely together, the group (which typically includes cardiologists, cardiac intensivists, radiologists specializing in cardiothoracic pathology, cardiothoracic (CT) surgeons, otolaryngologists who specialize in airway reconstruction, neonatal intensivists, and cardiac anesthesiologists) considers all the options, maps a treatment strategy, and, if necessary, composes a surgical plan.