II. Epidemiology

  1. Presents in infancy

III. Pathophysiology

  1. Weakness and softness of the trachea's cartilage and walls
  2. Abnormally soft cartilage more easily collapses, narrowing the trachea, especially with expiration
  3. May result in difficult clearance of airway secretions

IV. Causes

  1. Congenital (most common)
  2. Other congenital anomalies
    1. Tracheoesophageal fistula
    2. Vascular Ring
  3. Prolonged intubation
  4. Prolonged Pediatric Gastroesophageal Reflux disease

V. Signs

  1. Barking cough
  2. Expiratory Wheeze or expiratory Stridor
  3. Provocative factors (increase Wheezing)
    1. Crying
    2. Feeding
    3. Cough
    4. Nebulized beta Agonists

VI. Diagnosis

  1. Bronchoscopy while child breathes spontaneously

VII. Management

  1. Consult pediatric pulmonology
  2. Supportive care (most cases)
  3. Severe Tracheomalacia
    1. Tracheostomy
    2. CPAP

VIII. Course

  1. Self limited
  2. Improves by age 12 to 24 months

IX. References

  1. Mehta and Eliason (2024) Crit Dec Emerg Med 38(6): 27-35
  2. Benjamin (1984) Ann Otol Rhinol Laryngol 93:438-42 [PubMed]

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Related Studies

Ontology: Tracheobronchomalacia (C0340231)

Definition (MSH) A congenital or acquired condition of underdeveloped or degeneration of CARTILAGE in the TRACHEA and the BRONCHI. This results in a floppy non-rigid airway making patency difficult to maintain.
Definition (MSHCZE) Vrozený nebo získaný stav nedovyvinuté nebo degenerované chrupavky v PRŮDUŠNICI a v PRŮDUŠKÁCH. Je pak obtížné udržovat průchodnost ochablých a nezpevněných vzduchových cest.
Definition (NCI) A rare congenital or acquired disorder characterized by excessive collapsibility of the trachea and major bronchi resulting in breathing problems. It is caused by damage to the tracheobronchial cartilage.
Concepts Disease or Syndrome (T047)
MSH D055089
SnomedCT 233788001
English WILLIAMS-CAMPBELL SYNDROME, Tracheobronchomalacia, Chondromalacia of Trachea and Bronchi, Tracheobronchomalacias, Tracheobronchomalacia [Disease/Finding], Syndrome, Williams-Campbell, Williams-Campbell Syndrome, Williams Campbell Syndrome, Williams-Campbell syndrome, Tracheobronchomalacia (disorder)
Spanish Condromalacia de la Tráquea y Bronquios, Traqueobroncomalacia, síndrome de Williams-Campbell, traqueobronquiomalacia (trastorno), traqueobronquiomalacia
French Trachéobronchomalacie, Syndrome de Williams-Campbell
Italian Tracheobroncomalacia, Condromalacia della trachea e dei bronchi, Condromalacia tracheo-bronchiale
Russian ХОНДРОМАЛЯЦИЯ ТРАХЕЙ И БРОНХОВ, ТРАХЕОБРОНХОМАЛЯЦИЯ, KHONDROMALIATSIIA TRAKHEI I BRONKHOV, TRAKHEOBRONKHOMALIATSIIA
German Chondromalazie der Luftröhre und Bronchien, Tracheobronchomalazie
Portuguese Traqueobroncomalácia, Condromalacia da Traqueia e dos Brônquios
Swedish Trakeobronkomalaci
Czech tracheobronchomalacie, trachea a bronchy - chondromalacie, Williamsův-Campbellův syndrom
Polish Wiotkość tchawicy i oskrzeli, Tracheobronchomalacja
Japanese 気管-気管支軟化症, 気管気管支軟化症, 気管・気管支軟化症
Norwegian Trakeobronkomalasi, Kondromalasi i luftrør og bronkier, Brusksykdom i luftrør og luftveier

Ontology: Tracheomalacia (C0948187)

Definition (MSH) A congenital or acquired condition of underdeveloped or degeneration of CARTILAGE in the TRACHEA. This results in a floppy tracheal wall making patency difficult to maintain. It is characterized by wheezing and difficult breathing.
Definition (MSHCZE) Regresivní změna průdušnice s výrazným změknutím chrupavek. Může vzniknout např. lokálně z dlouhodobého útlaku např. strumou či při zánětech nebo v rámci celkové chondromalacie. Může způsobovat poruchy ventilace. (cit. Velký lékařský slovník online, 2013 http://lekarske.slovniky.cz/ )
Definition (NCI) A congenital or acquired abnormality of the wall of the trachea. In congenital cases, there is lack of rigidity in the cartilage of the tracheal wall. In acquired cases, the cartilage of the tracheal wall is degenerated, secondary to tracheostomy or prolonged presence of a breathing tube, or as a complication of a tracheoesophageal fistula surgical repair. Symptoms include stridor, noisy breathing, and upper respiratory infections.
Concepts Disease or Syndrome (T047)
MSH D055090
SnomedCT 266411000, 155627006, 95434006
Dutch tracheomalacie (excl. congenitaal), tracheomalacie
French Trachéomalacie (excl congénitale), Trachéomalacie, Chondromalacie de la trachée
German Tracheomalazie (ausschl kongenital), Chondromalazie der Luftröhre, Tracheomalazie
Italian Tracheomalacia (congenita esclusa), Condromalacia della trachea, Tracheomalacia, Condromalacia tracheale
Portuguese Traqueomalacia (excl. congénita), Traqueomalácia, Condromalacia da Traqueia
Spanish Traqueomalacia (excl congénita), Condromalacia de la Tráquea, Traqueomalacia, Condromalacia Traqueal, traqueomalacia (trastorno), traqueomalacia, Traqueomalasia
Japanese 気管軟化症(先天性を除く), キカンナンカショウ, キカンナンカショウセンテンセイヲノゾク, 気管軟化症
English Tracheomalacia, Tracheomalacias, Trachea Chondromalacia, Trachea Chondromalacias, Chondromalacia of Trachea, Tracheomalacia [Disease/Finding], tracheomalacia, Tracheomalacia (excl congenital), Tracheomalacia (disorder), Tracheomalacia (excluding congenital)
Czech Tracheomalacie, Tracheomalacie (kromě kongenitální), trachea - chondromalacie, tracheomalacie
Russian KHONDROMALIATSIIA TRAKHEI, ТРАХЕОМАЛЯЦИЯ, ХОНДРОМАЛЯЦИЯ ТРАХЕИ, TRAKHEOMALIATSIIA
Swedish Trakeomalaci
Polish Tracheomalacja, Rozmiękanie tchawicy wrodzone, Wiotkość tchawicy
Hungarian Tracheomalacia, Tracheomalacia (kivéve veleszületett)
Norwegian Brusksykdom i luftrør, Trakeomalasi, Kondromalasi i luftrør