Understanding Your Child's Airway: What is CTS?
At a Glance
Congenital Tracheal Stenosis (CTS) is a rare birth defect where a child's windpipe is formed with rigid, O-shaped cartilage rings instead of flexible C-shaped ones. This severely narrows the airway. The standard treatment is a slide tracheoplasty, a surgery that widens the windpipe.
In a typical, healthy airway, the trachea (windpipe) is made of 16 to 20 “C-shaped” rings of cartilage. The back of the “C” is a soft, flexible membrane. This design is crucial because it allows the windpipe to expand slightly when we breathe deeply or cough.
In Congenital Tracheal Stenosis, some of these rings are not “C-shaped.” Instead, they are “O-shaped” or complete tracheal rings [1]. This means:
- The rings form a solid, rigid circle of cartilage [2].
- The soft membrane at the back is missing, so the windpipe cannot expand [3].
- The airway is fixed at a much narrower size than it should be, making it difficult for air to pass through [1].
Why It Happens
CTS is very rare, and its exact cause is still being studied. Scientists believe it happens very early in pregnancy, around the time the lungs and heart are forming [4]. In some cases, it is linked to a specific gene variant (TBX5), but often it occurs without a known genetic cause [5].
Because the airway and heart develop at the same time, children with CTS are often also born with a pulmonary artery sling (where a blood vessel wraps around the windpipe) or other heart-related conditions [4][2]. Your medical team will likely perform a thorough check of your child’s heart to ensure a complete care plan.
The Modern Solution: Slide Tracheoplasty
Until the mid-1990s, treating long segments of narrow airway was extremely difficult. Everything changed with the refinement of slide tracheoplasty.
During this procedure, the surgeon cuts the narrow part of the trachea in half. They then “slide” the two halves over each other and stitch them together. As mentioned earlier, this is like sliding two narrow straws together to make a wider one. This effectively doubles the width of the airway while shortening the length slightly [6][7].
This technique is preferred because it uses the child’s own living tissue, which grows along with them as they get older [8]. While it is a major surgery that requires a specialized multidisciplinary team (a group of experts from different fields like ENT, heart surgery, and intensive care), it has become the standard of care for restoring a child’s ability to breathe freely [9][10].
Next page: Recognizing the Signs: Symptoms and Warning Signs
Common questions in this guide
What is the difference between normal C-shaped and CTS O-shaped tracheal rings?
What causes Congenital Tracheal Stenosis?
Are there other health issues connected to CTS?
What is a slide tracheoplasty?
Questions to Ask Your Doctor
Curated prompts to bring to your next appointment.
- 1.How long is the narrowed segment of the trachea compared to the total length?
- 2.Are there any specific genetic tests (like testing for TBX5 variants) you recommend for my child?
- 3.Can you explain the exact mechanism of how the complete rings are restricting airflow?
Questions For You
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References
References (10)
- 1
A case of asymptomatic complete tracheal rings in an adult: case report.
Hayasaka T, Kobayashi T, Ako Y, et al.
JA clinical reports 2019; (5(1)):45 doi:10.1186/s40981-019-0265-7.
PMID: 32026139 - 2
[Congenital tracheal stenosis: from clinic to treatment.]
Duchoud L, De Sandre-Mouly C, Sandu K
Revue medicale suisse 2019; (15(665)):1765-1768.
PMID: 31580021 - 3
Incidence of congenital tracheal stenosis in left pulmonary artery sling diagnosed by bronchoscopy.
Tsuji E, Morita K, Matsuhisa H, et al.
Pediatric surgery international 2023; (39(1)):240 doi:10.1007/s00383-023-05527-6.
PMID: 37498341 - 4
Clinical and structural aspects of tracheal stenosis and a novel embryological hypothesis of left pulmonary artery sling.
Fukushima N, Shimojima N, Ishitate M, et al.
Pediatric pulmonology 2020; (55(3)):747-753 doi:10.1002/ppul.24661.
PMID: 31975532 - 5
TBX5 pathogenic variant in a patient with congenital heart defect and tracheal stenosis.
Yamoto K, Kato F, Yamoto M, et al.
Congenital anomalies 2024; (64(1)):23-27 doi:10.1111/cga.12548.
PMID: 38062907 - 6
Unravelling the Complexity of Congenital Tracheal Stenosis with 'O' Rings.
Arsie AE, Lamassiaude M, Nocini R, Sandu K
European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery 2026; (68(1)) doi:10.1093/ejcts/ezaf478.
PMID: 41416901 - 7
Slide Tracheoplasty in Long Segment Tracheobronchial Stenosis.
Beeman A, Ramaswamy M, Thiruchelvam T, et al.
The Annals of thoracic surgery 2025; (120(2)):355-364 doi:10.1016/j.athoracsur.2024.11.038.
PMID: 39725255 - 8
Understanding the impact of slide tracheoplasty in congenital tracheal stenosis.
Yong MS, Konstantinov IE
Translational pediatrics 2019; (8(5)):462-464 doi:10.21037/tp.2019.11.02.
PMID: 31993362 - 9
Congenital tracheal malformations.
Varela P, Torre M, Schweiger C, Nakamura H
Pediatric surgery international 2018; (34(7)):701-713 doi:10.1007/s00383-018-4291-8.
PMID: 29846792 - 10
Closed-PICU perioperative management of congenital tracheal stenosis.
Aoki K, Kurosawa H, Seino Y, et al.
Pediatrics international : official journal of the Japan Pediatric Society 2022; (64(1)):e15085 doi:10.1111/ped.15085.
PMID: 34865290
This page provides educational information about Congenital Tracheal Stenosis and slide tracheoplasty. It does not replace professional medical advice from your child's pediatric ENT or surgical team.
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