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Congenital Absence of Tracheal or Bronchial Rings
Matthew M Smith1,2, Yann-Fuu Kou1, Claudia Schweiger3
1Division of Pediatric Otolaryngology, Head and Neck Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA.
Insights
Slide tracheoplasty effectively treats congenital absent tracheal or bronchial rings in children. This study presents the largest series with long-term follow-up, demonstrating surgical success.
Area of Science:
- Pediatric Surgery
- Congenital Malformations
- Respiratory Medicine
Background:
- Congenital airway stenosis due to absent tracheal or bronchial rings is a rare and challenging condition.
- It often presents as severe segmental tracheomalacia, with limited prior data on long-term outcomes.
- Existing literature lacks comprehensive studies on surgical management and follow-up for this anomaly.
Purpose of the Study:
- To describe the surgical management and long-term outcomes of children with absent tracheal or bronchial rings.
- To evaluate the efficacy of slide tracheoplasty in this patient population.
- To contribute the largest case series with extended follow-up to the existing literature.
Main Methods:
- A retrospective chart review of patients diagnosed with absent tracheal or bronchial rings between 2002 and 2016.
- Inclusion of nine subjects who underwent slide tracheoplasty.
- Data collection included demographics, diagnosis and surgery timing, symptoms, ring location, procedures, and follow-up duration.
Main Results:
- Nine children underwent slide tracheoplasty for absent tracheal or bronchial rings.
- Median age at diagnosis and surgery was 4 and 5 weeks, respectively.
- Most patients (6/9) were extubated on postoperative day 1, with a mean follow-up of 5.89 years; one patient required additional interventions.
Conclusions:
- Slide tracheoplasty is an effective surgical treatment for absent tracheal or bronchial rings in infants and young children.
- This study represents the largest series to date detailing slide tracheoplasty outcomes with long-term follow-up.
- The findings support slide tracheoplasty as a viable option for managing this rare congenital anomaly.
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.
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