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Congenital Stridor Clinical Presentation

  • Author: Timothy D Murphy, MD; Chief Editor: Michael R Bye, MD  more...
 
Updated: Jan 21, 2015
 

History

The most common presentation for congenital stridor is chronic noisy breathing since birth. Most patients come to attention by the first 4-6 weeks of life. The stridor may emerge only after a few weeks, as the baby gains strength and airflow velocity increases during that time.

The history can be helpful in determining the cause of stridor. Characteristics of stridor that should be elicited include the following:

  • Association with position
  • Association with feeding
  • Persistent versus intermittent
  • Abnormal phonation
  • Foreign body exposure
  • Presence of other congenital abnormalities
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Physical

Physical examination should focus on examination of the respiratory tract, the quality and characteristics of the stridor, and other physical findings that may be associated with stridor.

  • Characteristics of the patient's stridor
    • Effect of position
    • Effect of feeding[2]
    • Inspiratory versus expiratory
    • Pitch and loudness
  • Respiratory examination
    • Lower respiratory tract sounds (eg, wheezes, crackles)[3]
    • Retractions
    • Quality of voice or crying
  • Other physical findings
    • Skin lesions (eg, hemangiomas)
    • Neurologic status
    • Klein et al conducted a study to find early clues to the diagnosis of DOK7 congenital myasthenic syndrome. The study concluded that stridor and feeding difficulties at birth or progressive weakness despite normal milestones in infancy point to the diagnosis and should lead to neurophysiological and genetic investigation. Fatigability can be absent or easily missed in the first years of life.[4]
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Causes

Differential diagnosis of stridor can be divided into supralaryngeal, laryngeal, tracheal, and nonanatomic categories.

  • Supralaryngeal causes of stridor: Supralaryngeal causes include choanal atresia, vallecular cysts, thyroglossal cysts, and tongue dermoid or teratoma.
  • Laryngeal causes of stridor
    • Laryngomalacia is the most common cause of congenital stridor.[5] Patients typically have high-pitched inspiratory stridor that increases with crying and when supine. It rarely interferes with feeding. Symptoms are usually present at birth or within the first 4-6 weeks of life. Laryngomalacia usually resolves of its own accord within 18-24 months.
    • Vocal cord paralysis may cause a patient to have a weak cry. Patients may also be worse with feeding. Ruling out underlying conditions that may result in vocal cord paralysis, in particular neurologic conditions such as Arnold-Chiari malformation, is important.[6] Bilateral paralysis is more commonly associated with neurologic problems and is a life-threatening emergency.
    • Subglottic stenosis can be congenital or acquired. Stridor of subglottic stenosis is typically biphasic. Acquired forms usually arise from endotracheal (ET) intubation.
    • Other laryngeal abnormalities leading to stridor include webs, cysts, hemangiomas, papillomata, and laryngotracheoesophageal clefts.[7]
  • Tracheal causes of stridor
    • Extrinsic compression of the trachea by vascular abnormalities is a common cause of stridor. Anomalies that may lead to stridor include double aortic arch, innominate artery compression, aberrant right subclavian vein, and pulmonary artery sling.[8]
    • Bronchogenic cysts can also cause extrinsic compression of the trachea.
    • Tracheomalacia may be observed in association with laryngomalacia or as an isolated lesion.
    • Tracheal stenosis can be a complication of long-term ET intubation or a congenital lesion.
    • Complete tracheal rings are a subset of tracheal stenosis with severe biphasic airway sounds.
  • Nonanatomic causes of stridor
    • Cardio-vocal syndrome occurs when cardiac abnormalities lead to compression of the recurrent laryngeal nerve.
    • Foreign body may be present.
    • Gastroesophageal reflux (GER) has been associated with stridor, although no controlled studies have been performed to demonstrate that GER is actually responsible for the stridor.
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Contributor Information and Disclosures
Author

Timothy D Murphy, MD Consulting and Attending Staff, Pediatric Pulmonary and Sleep Medicine, Mary Bridge Children's Hospital

Timothy D Murphy, MD is a member of the following medical societies: American Thoracic Society, American Academy of Sleep Medicine

Disclosure: Nothing to disclose.

Coauthor(s)

Clement L Ren, MD Chief, Division of Pediatric Pulmonology, Department of Pediatrics, Associate Professor, Golisano Children's Hospital at Strong, University of Rochester, New York

Clement L Ren, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, 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.

Charles Callahan, DO Professor, Chief, Department of Pediatrics and Pediatric Pulmonology, Tripler Army Medical Center

Charles Callahan, DO is a member of the following medical societies: American Academy of Pediatrics, American College of Chest Physicians, American College of Osteopathic Pediatricians, American Thoracic Society, Association of Military Surgeons of the US, Christian Medical and Dental Associations

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

Thomas Scanlin, MD Chief, Division of Pulmonary Medicine and Cystic Fibrosis Center, Department of Pediatrics, Rutgers Robert Wood Johnson Medical School

Thomas Scanlin, MD is a member of the following medical societies: American Association for the Advancement of Science, Society for Pediatric Research, American Society for Biochemistry and Molecular Biology, American Thoracic Society, Society for Pediatric Research

Disclosure: Nothing to disclose.

References
  1. Belmont JR, Grundfast K. Congenital laryngeal stridor (laryngomalacia): etiologic factors and associated disorders. Ann Otol Rhinol Laryngol. 1984 Sep-Oct. 93(5 Pt 1):430-7. [Medline].

  2. Jephson CG, Mills NA, Pitt MC, Beeson D, Aloysius A, Muntoni F, et al. Congenital stridor with feeding difficulty as a presenting symptom of Dok7 congenital myasthenic syndrome. Int J Pediatr Otorhinolaryngol. 2010 Sep. 74(9):991-4. [Medline].

  3. Lee ML, Chen M, Tsao LY, Chiu HY, Chiu IS, Yang AD, et al. Congenital stridor and wheezing as harbingers of the del22q11.2 syndrome presenting cardiovascular malformations of right aortic arch, aberrant left subclavian artery, Kommerell's diverticulum, and left ligamentum arteriosum. Cardiovasc Pathol. 2011 Mar-Apr. 20(2):124-9. [Medline].

  4. Klein A, Pitt MC, McHugh JC, Niks EH, Sewry CA, Phadke R, et al. DOK7 congenital myasthenic syndrome in childhood: early diagnostic clues in 23 children. Neuromuscul Disord. 2013 Nov. 23(11):883-91. [Medline].

  5. Wright CT, Goudy SL. Congenital laryngomalacia: symptom duration and need for surgical intervention. Ann Otol Rhinol Laryngol. 2012 Jan. 121(1):57-60. [Medline].

  6. Stritzke AI, Dunham CP, Smyth JA, Steinbok P. Congenital stridor in the context of Chiari malformation type II: the etiological role of vernix caseosa granulomatous meningitis. J Neurosurg Pediatr. 2011 Oct. 8(4):372-6. [Medline].

  7. Aksoy EA, Elsürer C, Serin GM, Unal OF. Evaluation of pediatric subglottic cysts. Int J Pediatr Otorhinolaryngol. 2012 Feb. 76(2):240-3. [Medline].

  8. Berdon WE, Baker DH. Vascular anomalies and the infant lung: rings, slings, and other things. Semin Roentgenol. 1972 Jan. 7(1):39-64. [Medline].

  9. Zalzal GH, Anon JB, Cotton RT. Epiglottoplasty for the treatment of laryngomalacia. Ann Otol Rhinol Laryngol. 1987 Jan-Feb. 96(1 Pt 1):72-6. [Medline].

  10. Cotton RT. Pediatric laryngotracheal stenosis. J Pediatr Surg. 1984 Dec. 19(6):699-704. [Medline].

  11. Cotton RT, Evans JN. Laryngotracheal reconstruction in children. Five-year follow-up. Ann Otol Rhinol Laryngol. 1981 Sep-Oct. 90(5 Pt 1):516-20. [Medline].

  12. Cotton RT, Schreiber JT. Management of laryngotracheoesophageal cleft. Ann Otol Rhinol Laryngol. 1981 Jul-Aug. 90(4 Pt 1):401-5. [Medline].

  13. McSwiney PF, Cavanagh NP, Languth P. Outcome in congenital stridor (laryngomalacia). Arch Dis Child. 1977 Mar. 52(3):215-8. [Medline].

  14. Nielson DW, Heldt GP, Tooley WH. Stridor and gastroesophageal reflux in infants. Pediatrics. 1990 Jun. 85(6):1034-9. [Medline].

  15. Orenstein SR, Kocoshis SA, Orenstein DM, Proujansky R. Stridor and gastroesophageal reflux: diagnostic use of intraluminal esophageal acid perfusion (Bernstein test). Pediatr Pulmonol. 1987 Nov-Dec. 3(6):420-4. [Medline].

  16. Parnell FW, Brandenburg JH. Vocal cord paralysis. A review of 100 cases. Laryngoscope. 1970 Jul. 80(7):1036-45. [Medline].

  17. Richardson MA, Cotton RT. Anatomic abnormalities of the pediatric airway. Ear Nose Throat J. 1985 Jan. 64(1):47-60. [Medline].

  18. Stanger P, Lucas RV Jr, Edwards JE. Anatomic factors causing respiratory distress in acyanotic congenital cardiac disease. Special reference to bronchial obstruction. Pediatrics. 1969 May. 43(5):760-9. [Medline].

  19. Weiss LN. The diagnosis of wheezing in children. Am Fam Physician. 2008 Apr 15. 77(8):1109-14. [Medline].

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Breath sound assessment. Video courtesy of Therese Canares, MD, and Jonathan Valente, MD, Rhode Island Hospital, Brown University.
 
 
 
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