eMedicine Specialties > Pediatrics: Surgery > Otolaryngology

Subglottic Stenosis: Differential Diagnoses & Workup

Author: John E McClay, MD, Assistant Professor, Department of Otolaryngology, Division of Pediatric Otolaryngology, Children's Medical Center, University of Texas Southwestern Medical School
Contributor Information and Disclosures

Updated: Oct 24, 2008

Differential Diagnoses

CHARGE Syndrome
Croup
Gastroesophageal Reflux

Other Problems to Be Considered

Consider any lesion that can cause stridor, including subglottic tumor (benign or malignant), subglottic hemangioma, glottic stenosis, tracheal stenosis, edema from gastroesophageal reflux (GER), and acute infection (eg, croup).

Workup

Laboratory Studies

  • Specific laboratory studies are not required in patients with subglottic stenosis (SGS).

Imaging Studies

  • The criterion standard for evaluation of the airway is direct laryngoscopy and direct bronchoscopy.
  • Certain radiographic examinations can help in obtaining a diagnosis and determining the severity of the disease. Usually, the initial radiographic study used to evaluate a child with airway obstruction is anteroposterior and lateral plain neck radiography. Frequently, in a child with SGS, the subglottis appears narrowed and peaked; this is often described as a steeple sign. In a patient with a thin web SGS, lateral plain film radiography may reveal a faint line.
  • Fluoroscopy is often performed in children with symptoms of airway obstruction.
    • Fluoroscopy can be used to diagnose lesions of the larynx and trachea.
    • When a barium-enhanced esophagram is added to the procedure, vascular malformations, along with gastroesophageal reflux disease (GERD), may be ruled out.
  • CT scans and MRIs are not often used in the primary evaluation of SGS.

Other Tests

  • Investigate any indication of GERD. Walner showed that children with SGS have a 3-fold increase in GERD compared with the general pediatric population.15
  • Currently, the best test in evaluating for GER is dual-channel pH probe testing. One probe is placed above the lower esophageal sphincter, and another is placed at the area of the cricopharyngeus near the larynx.
  • Walner and Cotton recommend treating GER for 1 month before and 12 months after airway reconstructive surgery, even if only mild disease is present.15,16
  • If moderate or severe GERD is diagnosed, start medical therapy and confirm disease resolution with another pH probe test prior to surgery.
  • Do not perform laryngeal reconstruction until GER has resolved.
  • If reconstruction is being considered, pediatric laryngologists frequently perform tests to rule out GER, even in the absence of symptoms, because the disease may affect the outcome.
  • One concern is that an "allergic" esophagitis may occur and may affect the outcome of surgery.
    • To evaluate for this entity, a esophagogastroduodenoscopy (EGD) is performed with biopsies of the proximal and distal esophagus, stomach, and duodenum.
    • If more than 15 eosinophils are found in the mucosa per high power field, the patient may have "allergic" esophagitis.
    • Evaluation and treatment for GERD must have taken place prior to this evaluation because reflux may elicit eosinophils as well.
    • If "allergic" esophagitis is discovered, then treatment with weeks to months of oral steroids or orally applied inhaled steroids is performed to help diminish the affects of the disease and possibly allow for a better success rate of laryngeal reconstruction.

Procedures

In a child with mild or moderate airway obstruction, perform flexible fiberoptic nasopharyngoscopy and laryngoscopy in the clinic or the emergency department (ED). If extreme airway obstruction is observed or if an active supraglottic infectious process is suspected in a young child, flexible endoscopy may be deferred in favor of formal rigid bronchoscopy in the operating room (OR). However, flexible fiberoptic nasopharyngoscopy may be performed in a controlled setting in the OR because determination of the nature of the supraglottis and glottis in awake, unsedated patients is crucial.

The procedures are described as follows:

  • Flexible fiberoptic nasopharyngoscopy and laryngoscopy
    • During flexible fiberoptic nasopharyngoscopy and laryngoscopy, topical anesthesia and decongestion can be accomplished in older infants and children with topical Afrin and lidocaine. A 3-mm endoscope can be used, even in an infant. Pass the endoscope into both nasal cavities to access pyriform aperture stenosis, midnasal stenosis, choanal atresia or stenosis, lesions of the nose and nasopharynx, and the adenoid pad.
    • Pass the endoscope into the superior oropharynx and hypopharynx. The hypopharynx and larynx can be assessed. Identify the structure and position of the supraglottis. Evaluate the epiglottis and arytenoids for malacia or stenosis. Evaluate the position and movement of the true vocal cords. Evaluate edema or erythema of the true vocal cords, epiglottis, and arytenoids.
  • Flexible endoscopy
    • This can be performed with the patient in the supine or sitting position. The supine position often results in the obstruction of certain supraglottic processes. If the goal is to obtain the best visualization of the true vocal cords and supraglottis, place a child (even an infant) in the sitting position with his or her neck extended.
    • If the child is older, the voice can be evaluated, and videostroboscopy can be performed to assess the vocal cord waveform and vocal cord mobility.
    • Occasionally, the subglottis can be visualized with flexible endoscopy; however, rigid laryngoscopy and bronchoscopy are the safest procedures and offer the best visualization for the subglottis and tracheobronchial tree.
  • Rigid laryngoscopy and bronchoscopy
    • Rigid laryngoscopy and bronchoscopy is the best single test for evaluating airway obstruction in children. The otolaryngologist must have knowledge of the pediatric airway, and the OR must have adequate bronchoscopes and telescopes of various sizes. Prepare all equipment for bronchoscopy, including laryngoscopes, light sources, video documentation equipment, telescopes, and bronchoscopes prior to the child's arrival in the OR. Throughout the procedure, maintain good communication between anesthesiologists, surgical nursing staff, and physicians, so that any potential airway obstruction can be quickly assessed and addressed.
    • Do not further injure the pediatric airway. This point is of paramount importance. Use the smallest bronchoscope or telescope alone for evaluation of the subglottis in a child who does not require ventilation throughout the procedure. This practice allows good visualization without iatrogenic injury to the area. If ventilation is required throughout the evaluation, use a bronchoscope-telescope combination.
    • If a child has a tracheotomy or is not in extreme distress, the child can breathe spontaneously and inhale oxygen and anesthetics through an endotracheal tube in the pharynx while the airways are visualized with a laryngoscope and large telescope. Frequently, the true vocal cords are anesthetized with lidocaine prior to evaluation to help prevent laryngospasm.
    • Determine the size of the child's airway by using endotracheal tubes. Myers and Cotton have established a scale for SGS severity that is based on the child's age and the size of the endotracheal tube that can be placed in the airway with an air leak pressure of less than 20 cm of water.
    • Evaluate the subglottis and glottis for fixation, scarring, granulation, edema, paralysis or paresis, and other abnormalities. Evaluate the distance and caliber of the stenosis. Apply the Myers and Cotton staging system only to circumferential SGS. Glottic stenosis and SGS often occur together and must be considered when reconstruction is planned.
    • Evaluate the maturity of the stenosis. If a firm white scar is present, the stenosis is mature. If the stenosis has a granular or erythematous appearance, GERD, viral infection, allergic esophagitis, or another inflammatory process may be present.
    • Examine the area below the subglottis into the trachea and bronchi for secondary lesions. The suprastomal area is important because pathological stenosis or malacia can influence the choice of surgical procedure. In severe SGS, viewing the suprastomal area requires the passage of a tiny telescope through a narrow subglottis or a telescope or bronchoscope through a tracheotomy site, if available.

Staging

Myers and Cotton devised a classification scheme for grading circumferential subglottic stenosis from I-IV. The scale is based on a percentage of stenosis established by the age of the patient and the size of the endotracheal tube that can be placed in the airway with an air leak less than 20 cm of water pressure.

The percentage of stenosis is evaluated by using endotracheal tubes of different sizes. The largest endotracheal tube that can be placed with an air leak less than 20 cm of water pressure is recorded and evaluated against a scale that has previously been constructed by Myers and Cotton. This grading system mainly applies to circumferential stenosis and does not apply to other types of SGS or combined stenoses, although it can be used to obtain a rough estimate.

  • The system contains 4 grades, as follows:
    • Grade I - Obstruction of 0-50% of the lumen obstruction
    • Grade II - Obstruction of 51-70% of the lumen
    • Grade III - Obstruction of 71-99% of the lumen
    • Grade IV - Obstruction of 100% of the lumen (ie, no detectable lumen)
  • Evaluate the subglottis and the glottis for any fixation, scarring, granulation, edema, paralysis or paresis, or other abnormalities.
  • Evaluate the distance and the caliber of the stenosis.
  • Only apply the Myers-Cotton staging system to circumferential SGS. Often, glottic stenosis and SGS occur together and must be considered when planning reconstruction.

More on Subglottic Stenosis

Overview: Subglottic Stenosis
Differential Diagnoses & Workup: Subglottic Stenosis
Treatment & Medication: Subglottic Stenosis
Follow-up: Subglottic Stenosis
Multimedia: Subglottic Stenosis
References
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Further Reading

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Keywords

subglottic stenosis, laryngeal stenosis, SGS, cricoid ring, acquired SGS, acquired subglottic stenosis, syphilis, tuberculosis, typhoid fever, diphtheria, endotracheal intubation, pressure necrosis, exercise intolerance, prematurity, airway obstruction, inspiratory stridor, biphasic stridor, gastroesophageal reflux disease, GERD, bronchopulmonary dysplasia, cleft palate, choanal atresia, retrognathia, subglottic tumor, subglottic hemangioma, glottic stenosis, tracheal stenosis, edema, gastroesophageal reflux, GER, acute infection, croup

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Contributor Information and Disclosures

Author

John E McClay, MD, Assistant Professor, Department of Otolaryngology, Division of Pediatric Otolaryngology, Children's Medical Center, University of Texas Southwestern Medical School
John E McClay, MD is a member of the following medical societies: American Academy of Otolaryngic Allergy, American Academy of Otolaryngology-Head and Neck Surgery, American College of Surgeons, and American Medical Association
Disclosure: Nothing to disclose.

Medical Editor

Orval Brown, MD, Director of Otolaryngology Clinic, Professor, Department of Otolaryngology-Head and Neck Surgery, University of Texas Southwestern Medical Center at Dallas
Orval Brown, MD is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, American Academy of Pediatrics, American Bronchoesophagological Association, American College of Surgeons, American Medical Association, American Society of Pediatric Otolaryngology, Society for Ear, Nose and Throat Advances in Children, and Society of University Otolaryngologists-Head and Neck Surgeons
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from broker recommendation; Avanir Pharma Stock Investment from broker recommendation

Managing Editor

Alan D Murray, MD, Pediatric Otolaryngologist, ENT for Children; Full-Time Staff, Medical City Dallas Children's Hospital; Consulting Staff, Department of Otolaryngology, Medical Center of Lewisville, Children's Medical Center at Dallas, Cook Children's Medical Center; Full-Time Staff, Texas Pediatric Surgery Center, The Pediatric Surgery Center
Alan D Murray, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Otolaryngology-Head and Neck Surgery, American Academy of Pediatrics, American College of Surgeons, American Society of Pediatric Otolaryngology, Society for Ear, Nose and Throat Advances in Children, and Texas Medical Association
Disclosure: Nothing to disclose.

CME Editor

Paul D Petry, DO, FACOP, FAAP, Consulting Staff, Freeman Pediatric Care, Freeman Health System
Paul D Petry, DO, FACOP, FAAP is a member of the following medical societies: American Academy of Osteopathy, American Academy of Pediatrics, American College of Osteopathic Pediatricians, and American Osteopathic Association
Disclosure: Nothing to disclose.

Chief Editor

Glenn C Isaacson, MD, FACS, FAAP, Professor of Otolaryngology-Head and Neck Surgery and Pediatrics, Temple University School of Medicine
Glenn C Isaacson, MD, FACS, FAAP is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, American Academy of Pediatrics, American Bronchoesophagological Association, American College of Surgeons, American Laryngological Rhinological and Otological Society, American Society of Pediatric Otolaryngology, and Society of University Otolaryngologists-Head and Neck Surgeons
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