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Congenital Stridor

Last Updated: May 10, 2006
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Synonyms and related keywords: congenital stridor, congenital croup, chronic congestion, obstruction of airway, noisy breathing

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Author: Clement L Ren, MD, Chief, Division of Pediatric Pulmonology, Associate Professor, Department of Pediatrics, Golisano Children's Hospital at Strong

Clement L Ren, MD, is a member of the following medical societies: American Academy of Pediatrics

Editor(s): Thomas Scanlin, MD, Chief, Division of Pediatric Pulmonary & Cystic Fibrosis, Assistant Professor, Department of Pediatrics, Robert Wood Johnson University Medical Group; Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine.com, Inc; Charles Callahan, DO, Professor, Deputy Chief of Clinical Services, Walter Reed Army Medical Center; Mary E Cataletto, MD, Associate Director, Division of Pediatric Pulmonology, Winthrop University Hospital; Associate Professor, Department of Clinical Pediatrics, State University of New York at Stony Brook; and Michael R Bye, MD, Professor of Clinical Pediatrics, Columbia University College of Physicians and Surgeons; Acting Director, Department of Pediatric Pulmonary Medicine, Columbia University Medical Center

Disclosure


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Background: Stridor is a clinical sign characterized by musical, monophonic, audible breath sounds (noisy breathing) caused by oscillations of narrowed large extrathoracic airways. The presence of stridor indicates a partial obstruction of the upper airways, glottis, or trachea. The pitch of the stridor is determined by the degree of airway obstruction and the velocity of the airflow. The exact quality of the sound in terms of pitch and loudness varies depending on the specific cause. Congenital stridor is present at birth or within the first few weeks (4-6) of life.

Pathophysiology: Stridor results from partial obstruction of an airway with turbulent flow characteristics. Such respiratory tract areas are the upper airway, glottis, and trachea. The obstruction can be fixed or variable. Variable extrathoracic obstructions are primarily associated with inspiratory stridor. This is because during inspiration extrathoracic intraluminal airway pressure is negative relative to atmospheric pressure, leading to collapse of supraglottic structures. In contrast, stridor caused by intrathoracic obstructions tends to be more prominent on expiration. On expiration, intrathoracic pressure is positive and tends to collapse the airway.

Mortality/Morbidity: With the exception of bilateral vocal cord paralysis and subglottic hemangioma, congenital stridor is rarely caused by a life-threatening or fatal lesion. However, significant airway obstruction can lead to respiratory distress. Significant obstruction can also lead to failure to thrive secondary to increased work of breathing.

Age: Congenital stridor is present either at birth or shortly afterward.


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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 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

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
    • Inspiratory versus expiratory
    • Pitch and loudness
  • Respiratory examination
    • Lower respiratory tract sounds (eg, wheezes, crackles)
    • Retractions
    • Quality of voice or crying
  • Other physical findings
    • Skin lesions (eg, hemangiomas)
    • Neurologic status

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. 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. 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 typically is biphasic. Acquired forms usually arise from endotracheal (ET) intubation.
    • Other laryngeal abnormalities leading to stridor include webs, cysts, hemangiomas, papillomata, and laryngotracheoesophageal clefts.
  • 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.
    • 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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Laryngomalacia
Pulmonary Artery Sling
Stridor
Subglottic Stenosis
Tracheomalacia
Vascular Ring, Double Aortic Arch
Vascular Ring, Right Aortic Arch


Other Problems to be Considered:

GER
Foreign body aspiration
Laryngeal stenosis

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Related Articles
Laryngomalacia

Pulmonary Artery Sling

Stridor

Subglottic Stenosis

Tracheomalacia

Vascular Ring, Double Aortic Arch

Vascular Ring, Right Aortic Arch


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Imaging Studies:

  • Chest radiography may be helpful in diagnosing a vascular ring if a right-sided aortic arch is observed.
  • Standard neck radiography is rarely helpful unless a large mass is responsible for the obstruction. High-kilovoltage radiography can highlight the tracheal structures better and may provide more information. These radiographs use a higher radiation dose.
  • Computed tomography (CT) scanning of the neck and chest may be helpful, especially if the radiology facility can perform airway reconstruction imaging. However, the need for cooperation in generating such images limits the utility of CT imaging in infants and young children with congenital stridor.
  • Barium esophagraphy can be helpful in diagnosing vascular rings if an indentation in the esophagus is present. The pattern of indentation may also be helpful in indicating what type of vascular anomaly may be present. However, the lesion of anomalous innominate artery does not yield abnormal findings on an esophagram.

Other Tests:

  • A multichannel sleep study that measures airflow, chest wall excursion, oxygen saturation, and heart rate can provide useful information about the severity of obstruction.
  • An arterial blood gas (ABG) can demonstrate the presence of carbon dioxide retention or chronic hypoxemia.
  • In cases of suspected gastroesophageal reflux, 24-hour mid esophageal pH monitoring may be helpful in establishing the diagnosis.

Procedures:

  • Fiberoptic laryngoscopy and bronchoscopy, valuable diagnostic tools for the evaluation of congenital stridor, offer several important advantages over radiographic imaging, including the following:
    • Lesions can be visualized directly. Evidence of inflammation or bleeding can be observed. Characteristics of the lesion, such as vascularity, can be determined.
    • Biopsies and bronchoalveolar lavage samples can be taken if necessary.
    • The examination is conducted while the patient is actively breathing, allowing assessment of dynamic events.
  • Fiberoptic direct laryngoscopy can be performed in the office. However, in the pediatric population this procedure can be performed most safely while the patient is sedated. Bronchoscopy in a child requires intravenous conscious sedation or general anesthesia.
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Medical Care: Medical care is primarily supportive because many causes of congenital stridor resolve spontaneously over time. For those that do not, such as vascular rings, surgical treatment usually is definitive. However, in some patients, tracheomalacia persists for some time after such a repair. In severe cases of congenital stridor, nonsurgical therapy may have a role prior to definitive surgical correction.

  • Supplemental oxygen can be used to treat hypoxemia.
  • Noninvasive positive pressure mechanical ventilation can maintain a patent airway in cases of airway collapse, such as tracheomalacia.
  • If an airway cannot be maintained by noninvasive positive pressure mechanical ventilation, intubation with mechanical ventilation may be indicated.

Surgical Care: Surgical management is dependent on the specific lesion causing stridor.

  • In general, indications for surgical correction include the following:
    • Inability to maintain a patent airway
    • Feeding difficulties or failure to thrive
    • Inability to maintain adequate oxygenation
  • Some of the surgical procedures used to treat congenital stridor include the following:
    • Epiglottoplasty for laryngomalacia
    • Tracheostomy for severe subglottic stenosis or tracheomalacia
    • Division of a vascular ring
    • Tracheoplasty for complete tracheal rings
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Complications:

  • The primary complication from congenital stridor is airway compromise and respiratory failure.
  • Other complications include failure to thrive secondary to increased work of breathing.

Prognosis:

  • The prognosis for congenital stridor depends on the specific cause. In general, it is very good.
  • For conditions such as laryngomalacia, the condition is self-limited and resolves on its own.
  • For other conditions, such as subglottic stenosis, surgical correction is curative.
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Medical/Legal Pitfalls:

  • The major medical pitfall in evaluation of congenital stridor is failure to make the diagnosis.
    • Infants are frequently diagnosed with congestion or asthma.
    • True viral upper respiratory infections in infants younger than 1 month are rare.
    • Persistently abnormal noisy breathing is unlikely to be associated with a viral illness and warrants further investigation.
  BIBLIOGRAPHY Section 9 of 9   Click here to go to the previous section in this topic Click here to go to the top of this page
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  • 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].
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  • Cotton RT: Pediatric laryngotracheal stenosis. J Pediatr Surg 1984 Dec; 19(6): 699-704[Medline].
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  • Nielson DW, Heldt GP, Tooley WH: Stridor and gastroesophageal reflux in infants. Pediatrics 1990 Jun; 85(6): 1034-9[Medline].
  • 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].
  • Parnell FW, Brandenburg JH: Vocal cord paralysis. A review of 100 cases. Laryngoscope 1970 Jul; 80(7): 1036-45[Medline].
  • Richardson MA, Cotton RT: Anatomic abnormalities of the pediatric airway. Ear Nose Throat J 1985 Jan; 64(1): 47-60[Medline].
  • 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].
  • 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].

Congenital Stridor excerpt