Tracheal stenosis
Encyclopedia
Laryngotracheal stenosis refers to abnormal narrowing of the central air passageways. This can occur at the level of the larynx
Larynx
The larynx , commonly called the voice box, is an organ in the neck of amphibians, reptiles and mammals involved in breathing, sound production, and protecting the trachea against food aspiration. It manipulates pitch and volume...

, trachea
Vertebrate trachea
In tetrapod anatomy the trachea, or windpipe, is a tube that connects the pharynx or larynx to the lungs, allowing the passage of air. It is lined with pseudostratified ciliated columnar epithelium cells with goblet cells that produce mucus...

, carina
Carina of trachea
In anatomy, the carina is a cartilaginous ridge within the trachea that runs anteroposteriorly between the two primary bronchi at the site of the tracheal bifurcation at the lower end of the trachea .The mucous membrane of the carina is the most sensitive area of the trachea and larynx...

 or main bronchi
Bronchus
A bronchus is a passage of airway in the respiratory tract that conducts air into the lungs. The bronchus branches into smaller tubes, which in turn become bronchioles....

. In a small number of patients narrowing may be present in more than one anatomical location.

Nomenclature

Laryngotracheal stenosis (Laryngo-: Glottic Stenosis; Subglottic Stenosis; Tracheal: narrowings at different levels of the windpipe) 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
Subglottic stenosis
Subglottic stenosis is a congenital or acquired narrowing of the subglottic airway. Although it is relatively rare, it is the third most common congenital airway problem . Subglottic stenosis can present as a life-threatening airway emergency...

 is often therefore used to describe central airway narrowing in children, and laryngotracheal stenosis is more often used in adults.

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:
Main causes of adult laryngotracheal stenosis
Benign causes Malignant causes
Extrinsic compression
  • Thyroid goitre
    Thyroid disease
    -Hyper- and hypofunction:Imbalance in production of thyroid hormones arises from dysfunction of the thyroid gland itself, the pituitary gland, which produces thyroid-stimulating hormone , or the hypothalamus, which regulates the pituitary gland via thyrotropin-releasing hormone . Concentrations of...

  • Thymoma
    Thymoma
    Thymoma is a tumor originating from the epithelial cells of the thymus. Thymoma is an uncommon tumor, best known for its association with the neuromuscular disorder myasthenia gravis. Thymoma is found in 15% of patients with myasthenia gravis. Once diagnosed, thymomas may be removed surgically...

  • Mediastinal lymphadenopathy (e.g. TB
    Tuberculosis
    Tuberculosis, MTB, or TB is a common, and in many cases lethal, infectious disease caused by various strains of mycobacteria, usually Mycobacterium tuberculosis. Tuberculosis usually attacks the lungs but can also affect other parts of the body...

    )
  • Vascular anomalies
  • Thyroid cancer
    Thyroid cancer
    Thyroid neoplasm is a neoplasm or tumor of the thyroid. It can be a benign tumor such as thyroid adenoma, or it can be a malignant neoplasm , such as papillary, follicular, medullary or anaplastic thyroid cancer. Most patients are 25 to 65 years of age when first diagnosed; women are more affected...

  • Lung cancer
    Lung cancer
    Lung cancer is a disease characterized by uncontrolled cell growth in tissues of the lung. If left untreated, this growth can spread beyond the lung in a process called metastasis into nearby tissue and, eventually, into other parts of the body. Most cancers that start in lung, known as primary...

    /lymphomas
    Lymphoma
    Lymphoma is a cancer in the lymphatic cells of the immune system. Typically, lymphomas present as a solid tumor of lymphoid cells. Treatment might involve chemotherapy and in some cases radiotherapy and/or bone marrow transplantation, and can be curable depending on the histology, type, and stage...

    -related mediastinal lymphadenopathy
  • Intrinsic narrowing
  • At the level of the larynx (glottis)
    • Bilateral vocal fold paralysis
    • Blunt/sharp laryngeal trauma
    • Foreign body inhalation
    • Sarcoidosis
      Sarcoidosis
      Sarcoidosis , also called sarcoid, Besnier-Boeck disease or Besnier-Boeck-Schaumann disease, is a disease in which abnormal collections of chronic inflammatory cells form as nodules in multiple organs. The cause of sarcoidosis is unknown...

    • Amyloidosis
      Amyloidosis
      In medicine, amyloidosis refers to a variety of conditions whereby the body produces "bad proteins", denoted as amyloid proteins, which are abnormally deposited in organs and/or tissues and cause harm. A protein is described as being amyloid if, due to an alteration in its secondary structure, it...

    • Bilateral vocal fold mobility impairment
      • Crico-arytenoid joint fixation
        • Rheumatoid arthritis
          Rheumatoid arthritis
          Rheumatoid arthritis is a chronic, systemic inflammatory disorder that may affect many tissues and organs, but principally attacks synovial joints. The process produces an inflammatory response of the synovium secondary to hyperplasia of synovial cells, excess synovial fluid, and the development...

        • Intubation-related joint fixation
      • Inter-arytenoid scarring
        • Intubation-related
    • Infections (e.g. Diphtheria
      Diphtheria
      Diphtheria is an upper respiratory tract illness caused by Corynebacterium diphtheriae, a facultative anaerobic, Gram-positive bacterium. It is characterized by sore throat, low fever, and an adherent membrane on the tonsils, pharynx, and/or nasal cavity...

      , epiglottitis
      Epiglottitis
      Epiglottitis is an inflammation of the epiglottis - the flap that sits at the base of the tongue, which keeps food from going into the trachea . Due to its place in the airway, swelling of this structure can interfere with breathing and constitutes a medical emergency...

      )
    • Respiratory papillomatosis
      Papillomatosis
      Papillomatosis is skin surface elevation caused by hyperplasia and enlargement of contiguous dermal papillae.- See also :* Skin lesion* Skin disease* List of skin diseases* Papilloma* Laryngeal papillomatosis...

    • Large ball-valving vocal polyps

    • At the level of subglottis/trachea
      • Intubation/tracheostomy-related (commonest cause)
      • Wegener's Granulomatosis
        Wegener's granulomatosis
        Wegener's granulomatosis , more recently granulomatosis with polyangiitis , is an incurable form of vasculitis that affects the nose, lungs, kidneys and other organs. Due to its end-organ damage, it is life-threatening and requires long-term immunosuppression...

      • Idiopathic Progressive Subglottic Stenosis
      • Amyloidosis
        Amyloidosis
        In medicine, amyloidosis refers to a variety of conditions whereby the body produces "bad proteins", denoted as amyloid proteins, which are abnormally deposited in organs and/or tissues and cause harm. A protein is described as being amyloid if, due to an alteration in its secondary structure, it...

      • Benign tumors (e.g. Carcinoid)
      • Tracheal trauma / rupture

    • At the level of carina or main bronchi
      • Wegener's Granulomatosis
        Wegener's granulomatosis
        Wegener's granulomatosis , more recently granulomatosis with polyangiitis , is an incurable form of vasculitis that affects the nose, lungs, kidneys and other organs. Due to its end-organ damage, it is life-threatening and requires long-term immunosuppression...

      • Foreign body inhalation
      • Tuberculosis
        Tuberculosis
        Tuberculosis, MTB, or TB is a common, and in many cases lethal, infectious disease caused by various strains of mycobacteria, usually Mycobacterium tuberculosis. Tuberculosis usually attacks the lungs but can also affect other parts of the body...

      • Following Photodynamic Therapy
        Photodynamic therapy
        Photodynamic therapy is used clinically to treat a wide range of medical conditions, including malignant cancers, and is recognised as a treatment strategy which is both minimally invasive and minimally toxic...

  • Head and neck (especially laryngeal or supraglottic) cancers

    • Primary tracheal cancers
    • Erosive thyroid cancer
      Thyroid cancer
      Thyroid neoplasm is a neoplasm or tumor of the thyroid. It can be a benign tumor such as thyroid adenoma, or it can be a malignant neoplasm , such as papillary, follicular, medullary or anaplastic thyroid cancer. Most patients are 25 to 65 years of age when first diagnosed; women are more affected...

    • Erosive esophageal cancer
      Esophageal cancer
      Esophageal cancer is malignancy of the esophagus. There are various subtypes, primarily squamous cell cancer and adenocarcinoma . Squamous cell cancer arises from the cells that line the upper part of the esophagus...

    • Lung cancer
      Lung cancer
      Lung cancer is a disease characterized by uncontrolled cell growth in tissues of the lung. If left untreated, this growth can spread beyond the lung in a process called metastasis into nearby tissue and, eventually, into other parts of the body. Most cancers that start in lung, known as primary...

       causing central airway obstruction

    Presentation

    Symptoms can include, but are not limited to, dyspnea
    Dyspnea
    Dyspnea , shortness of breath , or air hunger, is the subjective symptom of breathlessness.It is a normal symptom of heavy exertion but becomes pathological if it occurs in unexpected situations...

     (shortness of breath) and stridor
    Stridor
    Stridor is a high pitched wheezing sound resulting from turbulent air flow in the upper airway. Stridor is a physical sign which is produced by narrow or obstructed airway path. It can be inspiratory, expiratory or biphasic . Inspiratory stridor is common...

     (wheezing). Several patients have reported being initially diagnosed with asthma
    Asthma
    Asthma is the common chronic inflammatory disease of the airways characterized by variable and recurring symptoms, reversible airflow obstruction, and bronchospasm. Symptoms include wheezing, coughing, chest tightness, and shortness of breath...

    .

    Treatment

    General treatment options include
    1. Tracheal dilation using rigid bronchoscope
    2. Laser surgery
    3. Tracheal resection
      Segmental resection
      Segmental resection is a surgical procedure to remove part of an organ or gland. It may also be used to remove a tumor and normal tissue around it. In lung cancer surgery, segmental resection refers to removing a section of a lobe of the lung.- External links :* entry in the public domain NCI...

       and reconstruction


    Tracheal dilation is used to temporarily enlarge the airway. The effect of dilation typically lasts from few days to 6 months.

    There are differing opinions on treating with laser surgery
    Laser surgery
    Laser surgery is surgery using a laser to cut tissue instead of a scalpel. Examples include the use of a laser scalpel in otherwise conventional surgery, and soft tissue laser surgery, in which the laser beam vaporizes soft tissue with high water content...

    .

    Tracheal resection is so far the best alternative to cure the stenosis completely. 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
    Stent
    In the technical vocabulary of medicine, a stent is an artificial 'tube' inserted into a natural passage/conduit in the body to prevent, or counteract, a disease-induced, localized flow constriction. The term may also refer to a tube used to temporarily hold such a natural conduit open to allow...

    may be required to join the sections.

    Late June or early July of 2010, a new potential treatment was pioneered at Great Ormond Street Hospital in London, where Ciaran Finn-Lynch (aged 11) received a transplanted trachea which had been injected with stem cells harvested from his own bone marrow. The use of Ciaran's stem cells is hoped to prevent his immune system from rejecting the transplant.
    The source of this article is wikipedia, the free encyclopedia.  The text of this article is licensed under the GFDL.
     
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