Pediatric Tracheomalacia Clinical Presentation

  • Author: Michael R Bye, MD; Chief Editor: Michael R Bye, MD   more...
 
Updated: Jul 22, 2011
 

History

  • The history of a patient with tracheomalacia typically includes a wheeze that usually begins when the individual is aged 4-8 weeks. Although the congenital lesion is present before this time, airflow is apparently insufficient during the first few weeks of life to generate abnormal sounds. The wheeze generally increases with activity and colds and decreases during quiet periods.
  • At first, wheezing may be mistaken for a sign of bronchiolitis, especially during respiratory syncytial virus season. As wheezing continues, it may be mistaken for that of asthma or cystic fibrosis. If bronchodilator therapy has been attempted, the history usually indicates no improvement; however, in some infants, tracheomalacia worsens with bronchodilator therapy.
  • Unlike most infants with asthma or cystic fibrosis, infants with tracheomalacia are described as "happy wheezers" (ie, they maintain normal oxygenation and disposition and grow and develop normally, despite the chronic airway noises).
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Physical

  • The physical findings suggest the diagnosis.
  • The baby looks well and happy despite the chronic wheeze.
  • Vital signs, including the patient's respiratory rate and oxygen saturation, are normal.
  • Lungs have good air entry. The typical abnormality is of a wheeze throughout exhalation that sounds the same in all lung fields. It is a coarse monophonic exhalatory sound, which has been described as exhalatory stridor. No asymmetry to the breath sounds is present. On occasion, the lesion is intrathoracic, and the airway sounds occur in inspiration.
  • The patient's voice and cry are normal, unless gastroesophageal reflux is present.
  • Because some children have concomitant laryngomalacia, abnormal sounds may be heard on inspiration.
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Causes

  • As far as tracheomalacia is understood, most cases are isolated and idiopathic. A recent study proposed a possible neurologic etiology for tracheomalacia. This group was caring for a child with increased intracranial pressure. When the pressure was elevated, the child developed tracheomalacia. When the pressure was relieved, the tracheomalacia remitted. This scenario recurred, although the etiology for increased intracranial pressure causing tracheomalacia is unknow.[2]
  • Transient defects in tracheal cartilage development are assumed to be the cause of this condition. This is sometimes referred to as type 1 tracheomalacia.
  • Autopsy data are lacking, and no animal model is noted.
  • Some children with tracheomalacia have the lesion because of vascular anomalies or other causes of compression of the airway. This is referred to as type 2 tracheomalacia.
  • Tracheomalacia is a common finding after repair of a tracheoesophageal fistula.
  • Tracheomalacia may occur with and complicate other disorders, including gastroesophageal reflux disease, other forms of recurrent aspiration, and bronchopulmonary dysplasia (chronic lung disease of infancy).
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Contributor Information and Disclosures
Author

Michael R Bye, MD  Professor of Clinical Pediatrics, Division of Pulmonary Medicine, Columbia University College of Physicians and Surgeons; Attending Physician, Pediatric Pulmonary Medicine, Morgan Stanley Children's Hospital of New York Presbyterian, Columbia University Medical Center

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

Disclosure: Nothing to disclose.

Specialty Editor Board

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, and American Thoracic Society

Disclosure: Genentech Honoraria Speaking and teaching; Genentech Honoraria Consulting; Boston Scientific Consulting fee Consulting; Gilead Honoraria Speaking and teaching; Caremark Consulting fee Consulting; Vertex Pharmaceuticals Honoraria Speaking and teaching

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.

Heidi Connolly, MD  Associate Professor of Pediatrics and Psychiatry, University of Rochester; 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.

Mary E Cataletto, MD  Director of Children's Sleep Services, Winthrop Sleep Disorders Center, Mineola, NY; Professor of Clinical Pediatrics, State University of New York at Stony Brook, Stony Brook, NY

Mary E Cataletto, MD is a member of the following medical societies: American Academy of Pediatrics and American College of Chest Physicians

Disclosure: Shering Plough Pharmaceuticals Honoraria Consulting

Chief Editor

Michael R Bye, MD  Professor of Clinical Pediatrics, Division of Pulmonary Medicine, Columbia University College of Physicians and Surgeons; Attending Physician, Pediatric Pulmonary Medicine, Morgan Stanley Children's Hospital of New York Presbyterian, Columbia University Medical Center

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

Disclosure: Nothing to disclose.

References

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

  2. Jamal N, Bent JP, Vicencio AG. A neurologic etiology for tracheomalacia?. Int J Pediatr Otorhinolaryngol. Jun 2009;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. Aug 2006;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. Jan 1987;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). Nov-Dec 2005;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. Jul 2005;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. Jun 2005;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. Jan 2003;13(1):3-11. [Medline].

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

  10. Dave S, Currie BG. The role of aortopexy in severe tracheomalacia. J Pediatr Surg. Mar 2006;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. Feb 1999;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. Dec 1998;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.

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