Congenital Laryngotracheal Diseases

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Congenital Laryngotracheal Diseases

Pediatric Otolaryngology
Author Credentials 

John Bent, MD
Director of Pediatric Otolaryngology-Head & Neck Surgery
Program Director, Department of ORL-HNS
Professor, Albert Einstein College of Medicine
Montefiore Medical Center
The Children's Hospital at Montefiore
Bronx, NY 

 

Module Summary

Airway problems may present very early in life, particularly with congenital disorders affecting the laryngotracheal complex. The clinician’s understanding of the relationship between the anatomical characteristics of each condition along with its associated symptomatology facilitates an optimaltreatment strategy. Although medical therapies are limited in many of these anatomic anomalies, they should still be considered in select cases. Surgical therapies remain the mainstay of treatment when indicated, and matching of patient selection to the procedure is critical for a successful outcome.

Module Learning Objectives 
  1. Explain the clinical presentation of common congenital diseases of the larynx and trachea in children.
  2. Describe the pathophysiologic development of congenital laryngotracheal diseases.
  3. Detail treatment strategies, including surgical options, for congenital laryngotracheal diseases.

Embryology

Learning Objectives 

To understand the embryologic development of the laryngotracheal complex.

References 
  1. Brown OE. Structure and function of the upper airway. In: Wetmore RF, Muntz HR, McGill TJ, Editors, Pediatric Otolaryngology: Principles and Practice Pathways. Thieme Medical Publishers, Inc., New York (2000):685-687.

Anatomy

Learning Objectives 

To describe the pertinent anatomical characteristics of the pediatric airway and how these findings relate to symptomatology.

References 
  1. Brown OE. Structure and function of the upper airway. In: Wetmore RF, Muntz HR, McGill TJ, Editors, Pediatric Otolaryngology: Principles and Practice Pathways. Thieme Medical Publishers, Inc., New York, NY; (2000):679-683.
  2. Bent JP, Alexander RE, Eavey RD. The Pediatric Larynx. In: Fried MP, Ferlito A, The Larynx, Chapter 3, Plural Publishing, Inc., San Diego, CA; (2008): 73-84.

Pathogenesis

Learning Objectives 

To understand the pathophysiology of common congenital laryngotracheal diseases:

  1. Laryngotracheomalacia
  2. Vocal fold mobility disorders
  3. Congenital subglottic stenosis
  4. Tracheal stenosis
  5. Laryngotracheal clefts
References 
  1. de Long AL, Friedman EM. Vocal cord paralysis. In: Wetmore RF, Muntz HR, McGill TJ, Editors, Pediatric Otolaryngology: Principles and Practice Pathways. Thieme Medical Publishers, Inc., New York, NY; (2000):787-800.
  2. Hartnick CJ, Cotton RT. Congenital laryngeal anomalies. Laryngeal atresia, stenosis, webs and clefts. Otolaryngol Clin North Am. 2000 Dec;33(6):1293 -308. [EBM Level 5]
  3. Hughes CA, Dunham ME. Congenital anomalies of the larynx and trachea. In: Wetmore RF, Muntz HR, McGill TJ, Editors, Pediatric Otolaryngology: Principles and Practice Pathways. Thieme Medical Publishers, Inc., New York, NY; (2000):777-786.
  4. Wetmore RF. Management of acute airway obstruction. In: Wetmore RF, Muntz HR, McGill TJ, Editors, Pediatric Otolaryngology: Principles and Practice Pathways. Thieme Medical Publishers, Inc., New York, NY; (2000):845-6.

Basic Science

Learning Objectives 

To describe important laboratory investigations that have shaped understanding of congenital airway anomalies.

References 
  1. Jacobs IN, Finkel RS. Laryngeal electromyography in the management of vocal cord mobility problems in children. Laryngoscope. 2002;112:1243-8.
  2. Thompson DM. Abnormal sensorimotor integrative function of the larynx in congenital laryngomalacia: a new theory of etiology. Laryngoscope. 2007 Jun;117(6 Pt 2 Suppl 114):1-33. 
  3. Holinger LD. Histopathology of congenital subglottic stenosis. Ann Otol Rhinol Laryngol. 1999;108(2):101-11.

Incidence

Learning Objectives 

To describe the incidence of congenital laryngotracheal disorders.

References 
  1. Hughes CA, Dunham ME. Congenital anomalies of the larynx and trachea. In: Wetmore RF, Muntz HR, McGill TJ, Editors, Pediatric Otolaryngology: Principles and Practice Pathways. Thieme Medical Publishers, Inc., New York, NY; (2000):777-786.
  2. Wetmore RF. Management of acute airway obstruction. In: Wetmore RF, Muntz HR, McGill TJ, Editors, Pediatric Otolaryngology: Principles and Practice Pathways. Thieme Medical Publishers, Inc., New York, NY; (2000):845-6.
  3. Zoumalan R, Maddalozzo J, Holinger LD. Etiology of stridor in infants. Ann Otol Rhinol Laryngol 2007 May;116(5):329-34.  [EBM Level 4]
  4. Denoyelle F, Garabedian EN, Roger G, Tashjian G. Laryngeal dyskinesia as a cause of stridor in infants. Arch Otolaryngol Head Neck Surg. 1996;122:612-16.
  5. Smith RJH, Smith MCF, Glossop LP, Bailey CM, Evans JNG. Congenital vascular anomalies causing tracheoesophageal compression. Arch Otolaryngol. 1984;110:82-7.

Genetics

Learning Objectives 

To understand the relationship between any genetic syndromes which include congenital laryngotracheal disorders in their constellation of symptoms.

References 
  1. Hughes CA, Dunham ME. Congenital anomalies of the larynx and trachea. In: Wetmore RF, Muntz HR, McGill TJ, Editors, Pediatric Otolaryngology: Principles and Practice Pathways. Thieme Medical Publishers, Inc., New York, NY; (2000):777-786.
  2. Miyamoto RC, Cotton RT, Rope AF, Hopkin RJ, Cohen AP, Shott SR, Rutter MJ. Association of anterior glottic webs with velocardiofacial syndrome (chromosome 22q11.2 deletion). Otolaryngol Head Neck Surg. 2004 Apr;130(4):415-7

Patient Evaluation

Learning Objectives 

To perform a thorough history and physical examination of a child or infant with suspected laryngotracheal disease.

References 
  1. Hughes CA, Dunham ME. Congenital anomalies of the larynx and trachea. In: Wetmore RF, Muntz HR, McGill TJ, Editors, Pediatric Otolaryngology: Principles and Practice Pathways. Thieme Medical Publishers, Inc., New York, NY; (2000):777-786.
  2. Lim FY, Crombleholme TM, Hedrick HL, Flake AW, Johnson MP, Howell LJ, Adzick NS. Congenital high airway obstruction syndrome: natural history and management. J Pediatr Surg. 2003 Jun;38(6):940-5. [EBM Level 4]
  3. Wetmore RF. Management of acute airway obstruction. In: Wetmore RF, Muntz HR, McGill TJ, Editors, Pediatric Otolaryngology: Principles and Practice Pathways. Thieme Medical Publishers, Inc., New York, NY; (2000):852-60.
  4. Portier F, Marianowski R, Morrisseau-Durand MP, Zerah M, Manac’h, Y. Respiratory obstruction as a sign of brainstem dysfunction in infants with Chiari malformations. Int J Pediatr Otorhinolaryngol. 2001;57:195-202.

Imaging

Learning Objectives 

To describe the role of radiographic imaging in evaluation of infants and children with suspected laryngotracheal disease.

References 
  1. Zawin JK. Radiologic Evaluation of the upper airway. In: Wetmore RF, Muntz HR, McGill TJ, Editors, Pediatric Otolaryngology: Principles and Practice Pathways. Thieme Medical Publishers, Inc., New York, NY; (2000):695-700, 715-20, 725-27.
  2. Walner DL, Ouanounou S, Donnelly LF, Cotton RT. Utility of radiographs in the evaluation of pediatric upper airway obstruction. Ann Otol Rhinol Laryngol. 1999;108:378-83.
  3. Rimell FL, Shapiro AM, Meza MP, Goldman S, Hite S, Newman B. Magnetic resonance imaging of the pediatric airway. Arch Otolaryngol Head Neck Surg. 1997;123(9):999-1003.

Pathology

Learning Objectives 

To understand pathologic differences between the varying congenital laryngotracheal disorders.

Treatment

Learning Objectives 

To identify treatment strategies in children suspected of having a congenital laryngotracheal disorder.

References 
  1. Olney DR, Greinwald JH, Smith RJH, Bauman NM. Laryngomalacia and its treatment. Laryngoscope. 1999;109:1770-5.

Medical Therapies

Learning Objectives 

To describe the role of medical therapy in common congenital laryngotracheal disorders:

  • Laryngomalacia
References 
  1. Wetmore RF. Management of acute airway obstruction. In: Wetmore RF, Muntz HR, McGill TJ, Editors, Pediatric Otolaryngology: Principles and Practice Pathways. Thieme Medical Publishers, Inc., New York, NY; (2000):852-60.
  2. Giannoni C, Sulek M, Friedman EM, Duncan NO. Gastroesophageal reflux associated with laryngomalacia: a prospective study. Int J Ped Otorhinolaryngol. 1998;43:11-20.
  3. Halstead L. Gastroesophageal reflux: a critical factor in pediatric subglottic stenosis. Otolaryngol Head Neck Surg. 1999;120:683-8.

Pharmacology

Learning Objectives 

To understand the basis for medical therapy for laryngomalacia.

References 
  1. Walner DL, Stem Y, Gerber MR, Rudolph C, Baldwin CY, Cotton RT. Gastroesophageal reflux in patients with subglottic stenosis. Arch Otolaryngol Head Neck Surg 1998 May;124(5):551-5. [EBM Level 3]
  2. Wetmore RF. Infant and pediatric apnea. In: Wetmore RF, Muntz HR, McGill TJ, Editors, Pediatric Otolaryngology: Principles and Practice Pathways. Thieme Medical Publishers, Inc., New York, NY; (2000):830-831.

Surgical Therapies

Learning Objectives 

To describe the role and approach for surgical treatments for congenital laryngotracheal disorders:

  1. Laryngomalacia
  2. Vocal fold immobility:
    1. Unilateral
    2. Bilateral
  3. Congenital subglottic stenosis
  4. Tracheal stenosis
  5. Tracheobronchomalacia
  6. Laryngotracheal clefts
References 
  1. Cotton RT. Management of subglottic stenosis. Otolaryngol Clin North Am. 2000 Feb;33(1):111-30. [EBM Level 5]
  2. Zur KB, Carroll LM. Recurrent laryngeal nerve reinnervation in children: Acoustic and endoscopic characteristics pre-intervention and post-intervention. A comparison of treatment options. Laryngoscope. 2015 Dec;125 Suppl 11:S1-15. 
  3. Mair EA, Parsons DS. Pediatric tracheobronchial and major airway collapse. Ann Otol Rhinol Laryngol. 1992;101:300-9.
  4. Triglia JM, Guys JM, Louis-Borrione C. Tracheomalacia caused by arterial compression in esophageal atresia. Ann Otol Rhinol Laryngol. 1994;103:516-21.
  5. Hartnick CJ, Hartley BE, Lacy PD, Liu J, Willging JP, Myer CM, Cotton RT. Surgery for pediatric subglottic stenosis: disease-specific outcomes. Ann Otol Rhinol Laryngol 2001 Dec;110(12):1109-13. [EBM Level 3]
  6. McMurray JS, Myer, III CM. Management of chronic airway obstruction. In: Wetmore RF, Muntz HR, McGill TJ, Editors, Pediatric Otolaryngology: Principles and Practice Pathways. Thieme Medical Publishers, Inc., New York, NY; (2000):863-5,871-80.

Rehabilitation

Learning Objectives 

To understand the long term outcomes of treatment for congenital laryngotracheal disease in children.

Staging

Learning Objectives 

To describe common staging systems used to classify some congenital laryngotracheal disorders:

  • Laryngomalacia
  • Subglottic stenosis
  • Tracheomalacia
  • Laryngotracheal clefts

Case Studies

  1. You are called urgently to the NICU. A 2-hour-old 3-kg male infant, the product of a full-term and uncomplicated pregnancy and uneventful delivery, has biphasic high-pitched stridor. His is mildly tachypneic with nasal flaring and moderate suprasternal retractions.  His oxygen saturation is 99% on room air. Routine exam of nose, oral cavity and oropharynx shows no obstruction. He has good breath sounds on auscultation and his CXR is clear. Do you think it is more likely that his obstruction is at, above, or below his larynx? You opt to perform transnasal flexible fiberoptic laryngoscopy. You see that he has a prominent anterior glottic web with a pinpoint posterior glottic lumen. Is this an emergency? How would you manage it? Once patient is stabilized, what genetic anomaly would you want to exclude?
  2. A 5-week-old male infant presents with inspiratory stridor that manifested shortly after birth. It has worsened. Child’s birth weight was 2.9kg and he currently weighs at the 10th percentile. He was never intubated. His parents report that he has trouble swallowing and sleeping. They have taken him to the ER twice because of concern about his breathing, but no abnormalities were found. He is currently sleeping very comfortably in his mother’s arms. He has no birthmarks. You do flexible fiberoptic laryngoscopy: the artyepiglottic folds prolapse anteriorly and medially with inspirations, so severely that the glottis is not visible in any phase of respiration. Child becomes markedly stridorous when agitated. You diagnose laryngomalacia. Is this child at increased risk of GERD? Of a second airway lesion? What would you do next?

Complications

Learning Objectives 

To describe potential complications of both treated and untreated congenital laryngotracheal disease.

References 
  1. Alvarez-Neri H, Penchyna-Grub J, Porras-Hernandez JD, Blanco-Rodriguez G, Gonzalez R, Rutter MJ. Primary cricotracheal resection with thyrotracheal anastomosis for the treatment of severe subglottic stenosis in children and adolescents. Ann Otol Rhinol Laryngol. 2005 Jan;114(1 Pt 1):2-6.  [EBM Level 3]
  2. Baker S. Kelchner L, Weinrich B, Lee L, Willging P, Cotton R, Zur K. Pediatric laryngotracheal stenosis and airway reconstruction: a review of voice outcomes, assessment and treatment issues. J Voice. 2006 Dec;20(4): 631-41. [EBM Level 5]
  3. Wright CD, Graham BB, Grillo HC, Wain JC, Mathisen DJ. Pediatric tracheal surgery. Ann Thorac Surg 2002 Aug;74(2):308-13; discussion 314. [EBM Level 3]
  4. Yellon RF, Parameswaran M, Brandom BW. Decreasing morbidity following laryngotracheal reconstruction in children. Int J Pediatr Otorhinolaryngol. 1997 Aug 20;41(2):145-54. [EBM Level 3]

Review

Review Questions 
  1. What are the 3 most common causes of congenital airway obstruction?
  2. Is laryngomalacia more common in boys or girls?
  3. Which form of subglottic stenosis, assuming equal lumen size, does better with observation alone: congenital or acquired? Which form is more common?
  4. What are 2 congenital airway lesions associated with tracheo-esophageal fistula?
  5. What is the recommended surgical treatment for an infant with a 3-cm segment of severe tracheal stenosis secondary to congenital tracheal rings?
  6. A barium swallow show an indentation on the posterior esophageal wall is diagnostic of what congenital vascular anatomic anomaly?
Resources 

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  1. Annual Meeting Webcast (AMW):