Laryngotracheal stenosis


Laryngotracheal stenosis refers to abnormal narrowing of the central air passageways. This can occur at the level of the larynx, trachea, carina or main bronchi.
In a small number of patients narrowing may be present in more than one anatomical location.

Presentation

The most common symptom of laryngotracheal stenosis is gradually-worsening breathlessness particularly when undertaking physical activities. The patient may also experience added respiratory sounds which in the more severe cases can be identified as stridor but in many cases can be readily mistaken for wheeze. This creates a diagnostic pitfall in which many patients with laryngotracheal stenosis are incorrectly diagnosed as having asthma and are treated for presumed lower airway disease. This increases the likelihood of the patient eventually requiring major open surgery in benign disease and can lead to tracheal cancer presenting too late for curative surgery to be performed.

Causes

Laryngotracheal stenosis is an umbrella term for a wide and heterogeneous group of very rare conditions. The population incidence of adult post-intubation laryngotracheal stenosis which is the commonest benign sub-type of this condition is approximately 1 in 200,000 adults per year. The main causes of adult laryngotracheal stenosis are:
Benign causesMalignant causes
Extrinsic compression
  • Thyroid cancer
  • Lung cancer/lymphomas-related mediastinal lymphadenopathy
    • Intrinsic narrowing
  • At the level of the larynx
  • * Bilateral vocal fold paralysis
  • * Blunt/sharp laryngeal trauma
  • * Foreign body inhalation
  • *Sarcoidosis
  • * Amyloidosis
  • * Bilateral vocal fold mobility impairment
  • ** Crico-arytenoid joint fixation
  • *** Rheumatoid arthritis
  • *** Intubation-related joint fixation
  • ** Inter-arytenoid scarring
  • *** Intubation-related
  • * Infections
  • * Respiratory papillomatosis
  • * Large ball-valving vocal polyps
  • * Congenital laryngeal stenosis
  • ** Laryngeal atresia
  • ** Congenital laryngeal webs
  • At the level of subglottis/trachea
  • * Intubation/tracheostomy-related
  • * Granulomatosis with polyangiitis
  • * Idiopathic Progressive Subglottic Stenosis
  • * Amyloidosis
  • * Tracheopathia osteoplastica
  • * Tracheomalacia
  • ** Expiratory Dynamic Airway Collapse
  • ** Tracheobronchomalacia
  • *** Relapsing polychondritis
  • *** Tracheal ring damage due to COPD
  • *** Tracheal ring weakness
  • * Benign tumors
  • * Tracheal trauma / rupture
  • * Congenital subglottic/tracheal anomalies
  • ** Complete tracheal rings
  • ** Congenital subglottic/tracheal webs
  • ** Subglottic haemangioma
  • ** Subglottic / tracheal cysts
  • At the level of carina or main bronchi
  • * Granulomatosis with polyangiitis
  • * Foreign body inhalation
  • * Tuberculosis
  • * Following Photodynamic Therapy
  • Head and neck cancers
  • Primary tracheal cancers
  • Erosive thyroid cancer
  • Erosive esophageal cancer
  • Lung cancer causing central airway obstruction

    • Diagnosis

    Patient history,
    CT scan of neck and chest and
    Fibre-optic bronchoscopy.

    Treatment

    The optimal management of laryngotracheal stenosis is not well defined, depending mainly on the type of the stenosis.
    General treatment options include
    1. Tracheal dilation using rigid bronchoscope
    2. Laser surgery and endoluminal stenting
    3. Tracheal resection and laryngotracheal reconstruction
    Tracheal is used to temporarily enlarge the airway. The effect of dilation typically lasts from a few days to 6 months. Several studies have shown that as a result of mechanical dilation may occur a high mortality rate and a rate of recurrence of stenosis higher than 90%.
    Thus, many authors treat the stenosis by endoscopic excision with laser and then by using bronchoscopic dilatation and prolonged stenting with a T-tube.
    There are differing opinions on treating with laser surgery.
    In very experienced surgery centers, tracheal resection and reconstruction is currently the best alternative to completely cure the stenosis and allows to obtain good results. Therefore, it can be considered the gold standard treatment and is suitable for almost all patients.
    The narrowed part of the trachea will be cut off and the cut ends of the trachea sewn together with sutures. For stenosis of length greater than 5 cm a stent may be required to join the sections.
    Late June or early July 2010, a new potential treatment was trialed at Great Ormond Street Hospital in London, where Ciaran Finn-Lynch received a transplanted trachea which had been injected with stem cells harvested from his own bone marrow. The use of Ciaran's stem cells was hoped to prevent his immune system from rejecting the transplant, but there remain doubts about the operation's success, and several later attempts at similar surgery have been unsuccessful.

    Nomenclature

    Laryngotracheal stenosis is a more accurate description for this condition when compared, for example to subglottic stenosis which technically only refers to narrowing just below vocal folds or tracheal stenosis. In babies and young children however, the subglottis is the narrowest part of the airway and most stenoses do in fact occur at this level. Subglottic stenosis is often therefore used to describe central airway narrowing in children, and laryngotracheal stenosis is more often used in adults.