Sternal Tracheostomy for Jarcho Levin Syndrome
The technical aspects of tracheostomy in patients with Jarcho Levin Syndrome are relatively straightforward for the pediatric otolaryngologist with sufficient airway experience. The main hurdle to overcome is the need in many instances to remove a portion of the upper sternum for access to the trachea for tube placement. If unfamiliar with removal of the upper sternal anatomy and access to the mediastinum, the pediatric otolaryngologist should partner with a pediatric general surgeon or thoracic surgeon. In some cases, portions of the clavicular heads may need to be shaved down for the tracheostomy tube to sit flush against the skin.
During surgery, the thymus will need to be either reflected and secured laterally or partially removed to avoid the potential of obstructing the tracheal stoma during subsequent tube changes or in the situation of accidental decannulation (tube inadvertently falling out of the stoma). The innominate artery is usually deeper in the chest and is rarely in the way for tracheostomy tube placement and care. Subsequent tracheo-innominate artery fistula is always of some theoretical concern but not seen more frequently in clinical practice.
The main challenge after tracheostomy tube placement is that the tube angle is more acute and requires a greater bend than a standard stock “off the shelf” tube. Tubes need to be custom fitted for each patient’s skin to trachea angle for proper fit and potential ventilation without tube obstruction against the tracheal wall. The tube will also sit slightly away from the skin in the lower neck and care must be taken not to allow skin breakdown overlying the clavicular heads.