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Tracheomalacia (TM) is a disorder of the airway consisting of weakness of the anterior tracheal cartilaginous rings and laxity of the posterior membranous trachea, resulting in dynamic airway obstruction. The clinical symptoms range from chronic cough and wheezing to life-threatening apnea with cyanosis and profound airway obstruction (acute life-threatening events). TM in infancy occurs either in isolation or in association with a variety of anatomic conditions, including a wide range of congenital heart lesions, as well as primary tracheal and esophageal disorders. TM is also associated with chromosomal abnormalities, such as 22q11.2 deletion syndrome, and trisomy 21.
Severe TM is a life-threatening condition with reported mortality rates as high as 80%. Prolonged intubation and chronic positive pressure ventilation is particularly common in affected patients following reparative congenital heart surgery; in the most severe cases, tracheostomy is required. Although often life-saving in these patients, tracheostomy results in a high risk of medical, social, and developmental complications. Patients requiring tracheostomy are technology-bound until their anterior tracheal wall matures and they effectively outgrow their malacia. Most concerning, patients with tracheostomy and persistent severe TM remain at risk of sudden death, recurrent infections, and equipment failure.
Aortopexy has been accepted as the standard surgical treatment to alleviate TM associated with congenital heart disease and carries a success rate of 85 to 90%. In our experience, innominate artery-pexy coupled with aortopexy has been particularly effective in relieving TM secondary to isolated innominate artery compression. However, it has also been our experience that aortopexy alone has failed to relieve symptoms in a number of cases of more complex TM and tracheobronchial malacia.
Since March 2008, we have used anterior tracheal suspension for the treatment of severe refractory TM and tracheobronchial malacia in infants and children as an alternative to tracheostomy. The majority of children operated on with this technique had undergone previous surgery to repair congenital heart defects, including division of vascular rings and pulmonary artery slings, repair of atrioventricular canal, tetralogy of Fallot, and ventricular septal defects. Several had undergone slide tracheoplasty for long segment tracheal stenosis, with resultant distal tracheobronchial malacia, or had severe TM following repair of tracheoesophageal fistula. The objective of this operation is to provide direct anterior tracheal support, permitting weaning from positive pressure, and allowing patients to outgrow their malacia without the need for tracheostomy. Additional objectives include safe discharge from the hospital with long-term avoidance of acute life-threatening events.
We undertake anterior tracheal suspension as a last attempt to avoid the need for tracheostomy. The majority of patients in our series required a prolonged period of intubation following repair of another anatomic defect and had failed several attempts at extubation before consideration of anterior tracheal suspension. Others have described anterior tracheal suspension via a lower neck incision, or posterior tracheopexy via thoracotomy. We have found the anterior approach via a median sternotomy to provide superior exposure to the anterior tracheal wall, permitting more accurate suture positioning. Although the procedure is conceptually simple, several concerning challenges exist. These challenges include accurate suture positioning, precise or correct suture tension to avoid distortion of the airway, and avoidance of long-term risk to the great vessels by encroachment of suspension sutures into the wall of the great vessels. We have found that directly supporting the anterior tracheal wall with precisely placed anterior tracheal suspension sutures provides relief of airway symptoms in the most severe and refractory cases, allowing both reduction in ventilatory time and avoidance of tracheostomy.
Preoperative evaluation includes bronchoscopy, chest computed tomographic scan with airway series, and intraoperative bronchography. All patients are approached in a collaborative manner by our multidisciplinary tracheal team, which includes ENT specialists, pulmonologists, pediatric surgeons, pediatric cardiothoracic surgeons, anesthesiologists, and pediatric intensivists. All patients are followed longitudinally with clinical protocols that are still in evolution.
Between March 2008 and January 2011, we performed 16 anterior tracheal suspension procedures. All patients have been successfully weaned from positive pressure ventilatory support and discharged. Our tracheal team follows all patients by a protocol, including follow-up bronchoscopies. One patient with a preexisting tracheostomy was able to be decannulated following the procedure. A second patient with severe long segment TM following tracheoesophageal fistula repair was weaned from ventilatory support following anterior tracheal suspension and discharged, but developed recurrence of tracheoesophageal fistula and was ultimately managed with repair of recurrent tracheoesophageal fistula and tracheostomy. That patient has been successfully decannulated. The traditional and accepted treatment for TM due to vascular compression is aortopexy. We have found a subset of patients who, despite division of vascular ring, and aortopexy, have persistent severe TM and require continued positive pressure ventilatory support. This group of patients has consistently responded to anterior tracheal suspension.
We have found innominate artery-pexy coupled with distal ascending aortopexy to be an extremely reliable procedure in cases of isolated innominate artery compression, because in these cases aortopexy directly addresses the anatomic problem. However, in general, aortopexy is an indirect approach to TM, as it relies on broad distribution of tension based on the broad fascial attachments between the aorta and trachea, while anterior tracheal suspension provides direct, pinpoint support of the affected region. We have found that direct support of the anterior tracheal wall with anterior tracheal suspension produces an impressive direct effect on the anterior cartilage. We believe this is critical in successfully managing cases of more complex and refractory tracheobronchial malacia. Caution must be taken to avoid distortion of the airway because of excessive tension on the sutures. Careful assessment must be made to ensure safe pathways for the suspension sutures. Currently, we give consideration to this anterior tracheal suspension in all cases of patients with severe TM who are being considered for tracheostomy or chronic ventilatory support, as well as those patients who already have a tracheostomy and suffer from persistent malacia, limiting the ability to wean from ventilatory support.
I would like to thank the entire Children's Hospital of Wisconsin Tracheal Team for the development of this operation at our institution as well as for the outstanding preoperative, intraoperative, and postoperative care of these challenging patients. Team members include the following: Julie M. Baughn, MD, Richard Berens, MD, David J. Beste, MD, Nicole Braun, RN, Robert H. Chun, MD, Tim Fehrenbacher, PA, Mario G. Gasparri, MD, Nancy Ghanayem, MD, Mary Jean Hubert, RN, Kristina L. Keppel, RN, Joseph E. Kerschner, MD, Tim Martin, MD, Ndidiamaka Musa, MD, Kathleen A. Mussatto, PhD, Daiva Parakininkas, MD, Nisreen Rumman, MD, Aparna Rao, MD, Thomas Sato, MD, William B. Tisol, MD, James S. Tweddell, MD, and Michael R. Uhing, MD.
Dr. Mitchell reports equity ownership and receiving consulting fees from Aria Diagnostics.