Close
New

Medscape is available in 5 Language Editions – Choose your Edition here.

 

Pediatric Tracheomalacia

  • Author: Emily Concepcion, DO; Chief Editor: Michael R Bye, MD  more...
 
Updated: Oct 25, 2015
 

Background

Tracheomalacia is an abnormal collapse of the tracheal walls. It may occur in an isolated lesion or can be found in combination with other lesions that cause compression or damage of the airway. Tracheomalacia is usually benign, with symptoms due to airway obstruction. As such, this condition is often mistaken for chronic asthma or prolonged bronchiolitis.

This shows the trachea during inspiration and expi This shows the trachea during inspiration and expiration. Tracheal collapse of more than 50% during expiration is diagnostic of tracheomalacia.
Next

Pathophysiology

Tracheomalacia may occur as a primary lesion, in which case the cartilage of the trachea develops abnormally. This results in tracheal walls that are soft and collapse during respiration. The collapse causes airflow obstruction and wheezing, stridor, or both. If the lesion is extrathoracic, the collapse and airway sounds occur primarily during inspiration. If the lesion is intrathoracic, the collapse and airway sounds occur primarily during exhalation. Because most of the trachea is intrathoracic, exhalatory collapse accounts for most cases of tracheomalacia.

Tracheomalacia may also be found in conjunction with lesions that compress the airway, such as mediastinal masses, vascular slings, and vascular rings. It also occurs with increased frequency in children with chronic inflammation of the proximal airways. Less common in asthma, this etiology of tracheomalacia is more often seen in children with chronic lung disease of infancy, gastroesophageal reflux, or other forms of chronic aspiration.

Primary tracheomalacia is sometimes referred to as type 1, tracheomalacia associated with extrinsic compression is sometimes referred to as type 2, and tracheomalacia associated with intra-airway irritation/inflammation is sometimes referred to as type 3.

Tracheomalacia is frequently found after repair of a tracheoesophageal fistula and may cause significant symptoms for several years after the repair.

Previous
Next

Epidemiology

Frequency

United States

The frequency of tracheomalacia is unclear. The condition appears to primarily derive from a developmental defect in the cartilage of the tracheal wall. Therefore, the lesion usually occurs in infants and young children. It is frequently found in children who have undergone repair of a tracheoesophageal fistula, chronic lung disease of infancy, vascular compression of the airway, or mediastinal masses of sufficient firmness to compress the airway. Children with gastroesophageal reflux, or aspiration from above, have an increased incidence of tracheomalacia. The problem in this last situation is trying to decide which condition is the cause and which is the effect.

International

Data from the Sophia Children's Hospital in Rotterdam (southwest Netherlands), the only facility in that country performing bronchoscopy in children, suggest an incidence rate of 1 case per 2100 newborns.[1]

Mortality/Morbidity

Morbidity and mortality are extremely rare. On occasion, tracheomalacia causes enough obstruction to necessitate intervention. This obstruction generally takes the form of episodic severe airway obstruction causing cyanosis. When infants with chronic lung disease of infancy become irritated, they may have what has been called a "BPD fit." This episode usually involves a cry, with either a breath hold or with a sufficient increase in intrathoracic pressure to partially occlude the airway. If the child has tracheomalacia, the frequency and severity of these episodes is often increased.

Race

No racial predilections are known.

Sex

No gender predilections are known.

Age

Because most cases of tracheomalacia appear to be related to a developmental defect in the cartilage of the tracheal wall, the lesion typically occurs in infants and young children. In most children, the tracheal cartilage normalizes, the airway enlarges, and symptoms resolve by 3 years of age (in many before age 1 y).

Because tracheoesophageal fistula is usually repaired early in life, the associated tracheomalacia also appears in early infancy, usually shortly after surgery.

If the tracheomalacia is a result of compression, the patient's age at presentation depends on the cause of compression. Vascular rings, present from birth, cause tracheomalacia early in life. Other causes of compression, especially tumors, occur later in life.

Previous
 
 
Contributor Information and Disclosures
Author

Emily Concepcion, DO Fellow, Department of Pediatric Pulmonology, State University of New York Downstate Medical Center

Disclosure: Nothing to disclose.

Coauthor(s)

Michael R Bye, MD Professor of Clinical Pediatrics, State University of New York at Buffalo School of Medicine; Attending Physician, Pediatric Pulmonary Division, Women's and Children's Hospital of Buffalo

Michael R Bye, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Chest Physicians, American Thoracic Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Chief Editor

Michael R Bye, MD Professor of Clinical Pediatrics, State University of New York at Buffalo School of Medicine; Attending Physician, Pediatric Pulmonary Division, Women's and Children's Hospital of Buffalo

Michael R Bye, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Chest Physicians, American Thoracic Society

Disclosure: Nothing to disclose.

Additional Contributors

Susanna A McColley, MD Professor of Pediatrics, Northwestern University, The Feinberg School of Medicine; Director of Cystic Fibrosis Center, Head, Division of Pulmonary Medicine, Children's Memorial Medical Center of Chicago

Susanna A McColley, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Chest Physicians, American Sleep Disorders Association, American Thoracic Society

Disclosure: Received honoraria from Genentech for speaking and teaching; Received honoraria from Genentech for consulting; Partner received consulting fee from Boston Scientific for consulting; Received honoraria from Gilead for speaking and teaching; Received consulting fee from Caremark for consulting; Received honoraria from Vertex Pharmaceuticals for speaking and teaching.

Acknowledgements

Heidi Connolly, MD Associate Professor of Pediatrics and Psychiatry, University of Rochester School of Medicine and Dentistry; Director, Pediatric Sleep Medicine Services, Strong Sleep Disorders Center

Heidi Connolly, MD is a member of the following medical societies: American Academy of Pediatrics, American Thoracic Society, and Society of Critical Care Medicine

Disclosure: Nothing to disclose.

References
  1. Boogaard R, Huijsmans SH, Pijnenburg MW, et al. Tracheomalacia and bronchomalacia in children: incidence and patient characteristics. Chest. 2005 Nov. 128(5):3391-7. [Medline].

  2. Jamal N, Bent JP, Vicencio AG. A neurologic etiology for tracheomalacia?. Int J Pediatr Otorhinolaryngol. 2009 Jun. 73(6):885-7. [Medline].

  3. Boiselle PM, Ernst A. Tracheal morphology in patients with tracheomalacia: prevalence of inspiratory lunate and expiratory "frown" shapes. J Thorac Imaging. 2006 Aug. 21(3):190-6. [Medline].

  4. Vinograd I, Filler RM, Bahoric A. Long-term functional results of prosthetic airway splinting in tracheomalacia and bronchomalacia. J Pediatr Surg. 1987 Jan. 22(1):38-41. [Medline].

  5. Yalcin E, Dogru D, Ozcelik U, et al. Tracheomalacia and bronchomalacia in 34 children: clinical and radiologic profiles and associations with other diseases. Clin Pediatr (Phila). 2005 Nov-Dec. 44(9):777-81. [Medline].

  6. Vinograd I, Keidar S, Weinberg M, Silbiger A. Treatment of airway obstruction by metallic stents in infants and children. J Thorac Cardiovasc Surg. 2005 Jul. 130(1):146-50. [Medline].

  7. Valerie EP, Durrant AC, Forte V, et al. A decade of using intraluminal tracheal/bronchial stents in the management of tracheomalacia and/or bronchomalacia: is it better than aortopexy?. J Pediatr Surg. 2005 Jun. 40(6):904-7; discussion 907. [Medline].

  8. Austin J, Ali T. Tracheomalacia and bronchomalacia in children: pathophysiology, assessment, treatment and anaesthesia management. Paediatr Anaesth. 2003 Jan. 13(1):3-11. [Medline].

  9. Casiano RR, Numa WA, Nurko YJ. Efficacy of transoral intraluminal Wallstents for tracheal stenosis or tracheomalacia. Laryngoscope. 2000 Oct. 110(10 Pt 1):1607-12. [Medline].

  10. Dave S, Currie BG. The role of aortopexy in severe tracheomalacia. J Pediatr Surg. 2006 Mar. 41(3):533-7. [Medline].

  11. [Guideline] Fayon M, Donato, L. Tracheomalacia (TM) or bronchomalacia (BM) in children: conservative or invasive therapy. (French). Archives de Pediatrie. January 2010. 17:97-104.

  12. Furman RH, Backer CL, Dunham ME, et al. The use of balloon-expandable metallic stents in the treatment of pediatric tracheomalacia and bronchomalacia. Arch Otolaryngol Head Neck Surg. 1999 Feb. 125(2):203-7. [Medline].

  13. Inoue K, Yanagihara J, Ono S, et al. Utility of helical CT for diagnosis and operative planning in tracheomalacia after repair of esophageal atresia. Eur J Pediatr Surg. 1998 Dec. 8(6):355-7. [Medline].

  14. Masters IB, Chang AB. Interventions for primary (intrinsic) tracheomalacia in children. Cochrane Database Syst Rev. 2005. CD005304. [Medline].

  15. Panitch HB, Keklikian EN, Motley RA, et al. Effect of altering smooth muscle tone on maximal expiratory flows in patients with tracheomalacia. Pediatr Pulmonol. 1990. 9(3):170-6. [Medline].

  16. Sirithangkul S, Ranganathan S, Robinson PJ, Robertson CF. Positive expiratory pressure to enhance cough effectiveness in tracheomalacia. Journal of the Medical Association of Thailand. November 2010. 93 Suppl 6:S112-8.

  17. van der Zee DC, Straver M. Thoracoscopic aortopexy for tracheomalacia. World J Surg. 2015 Jan. 39 (1):158-64. [Medline].

  18. Montgomery J, Sau C, Clement W, Danton M, Davis C, Haddock G, et al. Treatment of tracheomalacia with aortopexy in children in Glasgow. Eur J Pediatr Surg. 2014 Oct. 24 (5):389-93. [Medline].

  19. Arnaud AP, Rex D, Elliott MJ, Curry J, Kiely E, Pierro A, et al. Early experience of thoracoscopic aortopexy for severe tracheomalacia in infants after esophageal atresia and tracheo-esophageal fistula repair. J Laparoendosc Adv Surg Tech A. 2014 Jul. 24 (7):508-12. [Medline].

  20. Jennings RW, Hamilton TE, Smithers CJ, Ngerncham M, Feins N, Foker JE. Surgical approaches to aortopexy for severe tracheomalacia. J Pediatr Surg. 2014 Jan. 49 (1):66-70; discussion 70-1. [Medline].

 
Previous
Next
 
This shows the trachea during inspiration and expiration. Tracheal collapse of more than 50% during expiration is diagnostic of tracheomalacia.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.