Normally, the trachea is made of 18-22 incomplete ‘C’or horse-shoe shape (cartilaginous + membranous) rings. Rarely, these rings could be ‘O’ or complete, and can cause significant respiratory difficulties. These rings can present as a short or long segment. Sometimes, even with the complete rings, children may be able to cope up with life without any significant respiratory problems. Though, majority of infants will present very early in life with respiratory distress needing an operation to correct the airway narrowing.
The length of the stenotic segment is less important than the severity of narrowing of the airway. During inspiration and expiration (biphasic), children present with stridor that is typically called the "washing machine" breathing, that resembles a harsh noise of pent up secretions in the airway which the child wants to cough out, but cannot. Children present also chest retractions, cyanotic (blue) spells and recurrent episodes of pneumonia.
Around half of children with complete rings have other congenital anomalies – like heart or other vascular abnormalities, and these may need to be corrected at the same time as the airway.
The surgery that is offered as treatment for complete rings is called slide tracheoplasty. The airway reconstruction is a highly specialized surgery and requires a team (ENT and cardiac surgeons) approach. The principal of the surgery is to slide 2 narrow tubes on each other to give a larger diameter tube. The trachea is repaired through a neck incision, combined with a sternotomy (opening of the thorax).
The child is placed on a cardiopulmonary bypass (or ECMO - extracorporeal membrane oxygenation), until the airway and other cardiac or vascular pathologies are repaired during the same anesthesia.