Congenital Absence of Tracheal or Bronchial Rings

Otolaryngol Head Neck Surg. 2021 Feb;164(2):422-426. doi: 10.1177/0194599820950725. Epub 2020 Aug 25.


Objective: Congenital airway stenosis secondary to absent tracheal or bronchial rings is a rare congenital anomaly that is difficult to manage both clinically and surgically. This typically manifests as severe segmental tracheomalacia, and only isolated cases with short-term follow-up have been previously described. We aim to describe a series of children with absent tracheal or bronchial rings who underwent surgical management and had long-term follow-up.

Study design: Case series with chart review.

Setting: Tertiary care pediatric hospital.

Methods: Patients with absent tracheal or bronchial rings from 2002 to 2016. Electronic and paper medical records were queried to obtain demographics, age at diagnosis and surgery, pre- and postoperative symptoms, location of absent rings, procedure performed, length of follow-up, and adjunctive procedures performed.

Results: Nine subjects were identified who underwent slide tracheoplasty for correction of congenital absent tracheal or bronchial rings. Age at diagnosis ranged from 10 days to 5 years of age (median, 4 weeks). Age at surgery ranged from 3 weeks to 5 years of age (median, 5 weeks). Six out of 9 subjects were extubated on postoperative day 1. Only 1 subject required additional intervention, which included balloon dilation, tracheobronchial stenting, and aortopexy to alleviate the obstruction. Mean follow-up time was 5.89 years.

Conclusions: This is the largest series of children with absent tracheal rings who underwent slide tracheoplasty with long-term follow-up presented to date. Slide tracheoplasty is an effective surgical intervention for the treatment of absent tracheal or bronchial rings in infants and young children.

Keywords: absent/deficient rings; bronchial stenosis; slide tracheoplasty; tracheal stenosis.

MeSH terms

  • Bronchi / abnormalities*
  • Bronchi / diagnostic imaging
  • Bronchi / surgery
  • Bronchial Diseases / congenital*
  • Bronchial Diseases / diagnosis
  • Bronchial Diseases / surgery
  • Bronchoscopy
  • Constriction, Pathologic / diagnosis
  • Constriction, Pathologic / surgery*
  • Female
  • Follow-Up Studies
  • Humans
  • Infant
  • Infant, Newborn
  • Male
  • Plastic Surgery Procedures / methods*
  • Retrospective Studies
  • Trachea / abnormalities*
  • Trachea / surgery

Supplementary concepts

  • Tracheal agenesis