Unrepaired Complete Tracheal Rings: Natural History and Management Considerations

Abstract

Objectives To document the natural growth pattern of unrepaired complete tracheal rings (UCTRs) and describe the patient population managed conservatively. Study Design Case series with chart review. Setting Tertiary pediatric academic center. Subjects/Methods Medical records of patients with confirmed complete tracheal rings on bronchoscopy from 1993 to 2017 were reviewed. Patients aged 0 to 18 who had documented tracheal sizing over time and did not require surgical intervention were included. Exclusion criteria included tracheal stenosis not caused by complete tracheal rings. Comorbidities and airway characteristics were documented in addition to endoscopic findings. These were compared with children requiring surgical repair. Results In total, 149 patients with complete tracheal rings were identified. Twenty-five had UCTRs for an overall 16.8% rate of conservative management. Nineteen patients met inclusion criteria and underwent a total of 90 microlaryngoscopy and bronchoscopies (MLBs) with sizing. The growth of the UCTRs over time, based on MLB sizing, was chronicled. The median airway growth noted was 0.38 mm/y. A moderately strong positive correlation was seen between age and airway size ( r = 0.72, P < .0001). Children with UCTRs were less likely to have long-segment involvement than those who required repair (92%, P = .024). Conclusions A select group of children with complete tracheal rings can be managed expectantly without surgical intervention. Conservative management may be less successful in children with long-segment complete tracheal rings. Airway growth does occur in this population and can be monitored over time. Having a standardized method for sizing UCTRs allows for more effective communication between providers and assurance of continued growth of the airway while following these patients.

Growth and Management of Repaired Complete Tracheal Rings after Slide Tracheoplasty

“…In these cases, a modified endotracheal tube was created from 2 uncuffed endotracheal tubes as previously described elsewhere. 4 The airway growth over time was defined by the change in airway diameter throughout postoperative follow-up based on this sizing method. Of note, the airway may not have been sized at every MLB if there was concern for acute exacerbation of symptoms, interfering with healing, or the airway growth was obviously adequate.…”

“…While previous reports have shown approximately 17% of patients with complete tracheal rings can be managed conservatively, most children will require surgical repair. 4…”

An infant with stridor and hypoxemia

“…3 Management is surgical, including pulmonary artery re-implantation and slide tracheoplasty to increase diameter and allow growth of the tracheal rings. [4][5][6] Our patient was diagnosed using radiographs and CT scans. Ultrasound has emerged as an alternative means of airway assessment that is available at the bedside and avoids exposure to ionizing radiation.…”

Hedgehog and WNT Signaling Hubs in Tracheal Morphogenesis

“…The development of the trachea has been explored using genetic mouse models that reveal the importance of multiple paracrine signaling pathways, such as the SHH (sonic hedgehog) and WNT (Wingless-related integration site) pathways (1–4). In humans, tracheal defects are common in the pediatric patient population and include defects such as tracheomalacia and the rarer disease, complete tracheal ring deformity (CTRD) (5, 6). In CTRD, the cartilaginous rings surrounding the trachea are complete and lack a dorsal gap filled in with the trachealis muscle.…”