Rare malformation usually due to presence of complete O-shaped tracheal rings. Sometimes isolated, often associated with other malformations either cardiac (especially of the aortic arch) or in the context of a  VATER or VACTERL syndrome (see this term).

There are 3 types of congenital stenosis of trachea (Cantrell's and Guild's classification):

a)        generalized hypoplasia: 30 %

b)        funnel stenosis: 20 %

c)        segmental stenosis: 50 %, generally due to the presence of a few complete tracheal rings. This type has been  later subdivided as type 3 (presence of a tracheal bronchus and involvement of the carina), type 4 (no tracheal bronchus and normal carina), type 5 (localised to the carina) and type 6 (presence of a single lung) (Anton Pacheco)

A 3rd classification, the Great Ormond classification, has been proposed in 2012: it considers the type of division of the trachea (normal, tracheal bronchus, tracheal trifurcation, agenesis of the main bronchus) and the possible association with a bronchial stenosis.

Symptoms: depending on the importance of the stenosis, respiratory distress at birth, respiratory failure during an upper airway infection, inspiratory and expiratory stridor, incidental finding during intubation.

Treatment: resection-anastomosis in case of segmental involvement; some teams try endoscopic dilatations of the trachea, but those are poorly successful in the presence of complete tracheal rings. Slide-tracheoplasty for the more extended forms.

Anesthetic implications:

-        keep this pathology in mind in case of difficult intubation despite an easy laryngoscopy in a newborn or infant with a cardiopathy, or suffering of a VATER/VACTERL syndrome; keep a supraglottic airway ready and different very small diameter tubes to bypass the stenosis; emergency ORL examination to establish a diagnosis (  congenital or acquired stenosis, extrinsic compression, significant tracheomalacia)

-        in case of intubation where the endotracheal tube is maintained above the stenosis (segmental form), it is prudent to leave a distance of about 1 cm between the distal end of the tube and the beginning of the stenosis to avoid edema or other local traumatic lesions

-        for repair: cardiopulmonary bypass, ECMO, or high frequency ventilation; in some cases, wait for the tracheal incision before injecting the myorelaxant and controlling ventilation

-        for peripheral surgery: keep a supraglottic airway ready, associated if possible with locoregional anesthesia ;  keep at hand different very small diameter tubes to bypass the stenosis (e.g., suction or ureteral catheter)

-        if the child has been operated for a tracheal stenosis: use, if possible, a supraglottic airway; atraumatic intubation !

References : 

-        Murphy PM, Lloyd-Thomas A. 
The anaesthetic management of congenital tracheal stenosis. 
Anaesthesia 1991; 46: 106-9.

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Unexpected congenital tracheal stenosis in infants with congenital heart disease. 
Anesth Analg 1993; 77: 198-9.

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Epidural anaesthesia for ureteral reimplantation in an infant with congenital tracheal stenosis. 
Can J Anaesth 1997; 44: 666-8 

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Unsuspected concentric tracheal rings in a 14-year-old with scoliosis.
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-        Ali MI, Brunson CD, Mayhew JF. 
Failed intubation secondary to complete tracheal rings: a case report and literature review. 
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-        Taylor K, Levine M. 
Management of congenital tracheal stenosis: using spontaneous ventilation to facilitate cardiopulmonary bypass. 
Pediatr Anessth 2006; 16: 492-5.

-        Sacco O, Toma P, Alberti D, Locatelli G et al.
Inspiratory stridor in a 12 month-old girl.
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-        Speggiorin S, Torre M, Roebuck DJ, McClaren CA, Elliott MJ. 
A new morphologic classification of congenital tracheobronchial stenosis.
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-         De Cloedt L, Papadopoulos J, Corouge P, Khalil P, Van Laer P. 
Hypoplasie trachéale complexe chez un enfant de 2 mois. 
Arch Pédiatr 2013 ; 20 : 1321-4.

-        Obara S, O’Leary JD. 
Congenital tracheal stenosis : unanticipated and anticipated difficult airway management in a neonate. 
Anesth Analg Case Reports 2014; 3: 120-2

-        Mauron S, Longchamp D, Perez M-H, Prętre R, Sandu K, De Bernardo S, Boegli Y, Dolci M.
Anesthetic management for concomitant correction of congenital cardiac defects and long “O” ring tracheal stenosis: a role for heliox? Report of 2 cases.
A&A Practice 2018; 10: 154-6

-        Yokoi A, Hasegawa T, Oshima Y, Higashide S et al.
Clinical outcomes after tracheoplasty in patients with congenital tracheal stenosis in 1997-2014.
J Pediatr Surg 2018; 53: 2140-4.

Updated: July 2023