Glossary
A
AirFlo – AirFlo, an app developed by the North American Airways Collaborative (NoAAC), was a patient airflow monitoring tool (now discontinued) that measured and recorded a patient’s peak expiratory flow (PEF) generating data for the Vanderbilt Study.
AmnioFix – This product is an amnion tissue (stem cells) indicated for the treatment of acute and chronic wounds to enhance healing. It is being used by some doctors to try and slow the occurrence of scar tissue. It is still early days for this experimental treatment (around 12 months), so success rates are unknown.
Antinuclear antibody (ANA) – ANA is a blood test used to detect the presence of antinuclear antibodies, which may be associated with specific autoimmune diseases such as systemic lupus erythematosus (SLE).
Anti-neutrophil cytoplasmic antibodies (ANCA) – ANCA (p-ANCA and c-ANCA) is a blood test for granulomatosis with polyangiitis (GPA, formerly Wegener’s granulomatosis).
Awake injections – the common phrase for Awake Serial Intralesional Steroid Injections (SILSI), a procedure pioneered by Dr. Ramon Franco of Massachusetts Eye and Ear Infirmary, where submucosal injections of corticosteroids are made into a stenotic scar with the goal of modifying scars and decreasing operative interventions for SGS treatment (also see Steroid injections). (See The Laryngoscope 2017, Franco et al, “Awake Serial Intralesional Steroid Injections Without Surgery as a Novel Targeted Treatment for Idiopathic Subglottic Stenosis.”).
B
Balloon dilation – see Endoscopic tracheal dilation
Bite guard – A bite guard or intubation bite guard is a rigid plastic device inserted in the mouth over the teeth to protect dentition from accidental damage, such as chipping a front tooth, during a surgical procedure. Some SGS patients have had tooth damage and are advised to ensure their surgeon places a bite guard before every airway procedure.
Bronchoscopy – Bronchoscopy is an endoscopic technique of visualizing the inside of the airways for diagnostic and therapeutic purposes using an instrument called a bronchoscopy.
C
Cartilage tracheoplasty – is a form of laryngotrcheoplasty.
Chin stitch – A chin stitch (or Grillo stitch, named for the first surgeon to use this), is a now largely-outdated technique to help keep the head in position after a tracheal resection, only still used at a few centres.
Cilia – Cilia are hair-like structures that line the trachea and help move and expel normal mucus. SGS patients do not have any cilia where the stenotic scar is located, making it more difficult to expectorate.
Clinical trial – see Vanderbilt study
Corticosteroids – Corticosteroids inhibit fibroblast formation and reduce inflammation and can be administered orally by a tablet (e.g. Prednisone), by oral inhalation (e.g. nebulized albuterol, budesonide, i.e. Pulmicort) and by an injection (see Steroid injection and Awake injections). Common corticosteroids used in SGS injection treatment are triamcinolone acetate (Kenalog-40), dexamethasone (Decadron), methylprednisolone (Solu-Medrol) and betamethasone (Celestone).
Cotton-Myer Grading System – This is used to measure the severity of subglottic or tracheal stenosis and is determined with endotracheal tubes demonstrating a leak. It is named after the doctors who first proposed it in 1994.
The grades are –
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Grade 0. No or minimal (<10%) discernible stenosis
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Grade 1. Discernible obstruction between 10 and 50%
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Grade 2. Obstruction between 51 and 70%
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Grade 3. Obstruction between 71 and 99%
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Grade 4. No discernible lumen
Cricoid cartilage – The cricoid cartilage, or simply cricoid or cricoid ring, is the only complete ring of cartilage around the trachea.
Cricotracheal resection – Cricotracheal resection is a form of open anterior neck surgery. (See – Laryngoscope. 2018 Oct;128(10) –2268-2272. doi – 10.1002/lary.27263. Epub 2018 May 14. ‘Outcomes after cricotracheal resection for idiopathic subglottic stenosis’. Carpenter PS, Pierce JL, Smith ME.)
C-reactive protein (CRP) – CRP is a blood test for inflammation.
Cuff manometer – a device used during general anaesthesia, whether administered using endotracheal intubation or a laryngeal mask airway (LMA), to measure tracheal perfusion pressure (20-30 mmHg). Without a cuff manometer the cuff pressure can increase leading to a situation where tracheal mucosa is no longer perfused, which, in turn, leads to necrosis with subsequent scarring of the tissue and narrowing of the lumen (tracheal stenosis). All patients are advised to request the use of a cuff manometer when undergoing general anaesthesia.
D
Dilation – see Endoscopic tracheal dilation
Direct laryngoscopy – A direct exam of subglottic area via endoscopy (and “in-office scope”).
Dyspnea – Shortness of breath, also known as dyspnoea, is a feeling like one cannot breathe well enough with sensations including extra effort/work, chest tightness and/or air hunger.
E
Endoscope – a slender, tubular optical instrument used as a viewing system for examining an inner part of the body and, with an attached instrument, for biopsy or surgery.
Endoscopic balloon dilation – see Endoscopic tracheal dilation
Endoscopic resection – see Endoscopic tracheal resection
Endoscopic tracheal dilation – In cases of subglottic stenosis and tracheal stenosis, endoscopic tracheal dilation or dilatation is the mechanical widening, stretching, or enlarging of the opening of the trachea using a balloon dilator or tracheal dilators (with or without cuts or laser, with or without topical or injected corticosteroids, with or without Mitomycin-C).
Endoscopic tracheal reconstruction – Named for Jan Maddern (the first patient to undertake this procedure), “the Maddern” is an endoscopic transoral surgical procedure where the scar is removed, and a skin graft is placed temporarily into the trachea for two weeks to encourage healthy cells to regrow in place of the diseased tissue.
Endoscopic tracheal resection – A multidisciplinary team at Mayo Clinic in Rochester, USA developed this technique which involves transoral resection of the scar tissue using a laser. Longitudinal research by Vanderbilt University and the NoAAC has found this resection technique to deliver long lasting results for patients without compromising voice. It is slowly being adopted by other centres globally. (This technique is described in detail in: Maldonado F, et al. Idiopathic subglottic stenosis – An evolving therapeutic algorithm. Laryngoscope. 2014;124 –498.)
Endoscopy – An endoscopy is a procedure to look inside the body using an endoscope to examine the interior of a hollow organ or cavity of the body.
Endotracheal intubation – Endotracheal or tracheal intubation, usually simply referred to as intubation, is the placement of an endotracheal tube into the trachea to maintain an open airway or to serve as a conduit through which to administer certain drugs.
Endotracheal tube (ETT) – A flexible plastic tube placed into the trachea during endotracheal intubation to establish and maintain airway patency, prevent aspiration into the lungs, i.e. to “secure” the airway and allow for mechanical ventilation. ETT sizes (e.g., 5.5, 6.0, 6.5, etc.) refer to the inner diameter of the tube measured in mm and vary by and within gender. Standard practice is to use a 7.0 ETT for women, but it may be too large for some airways, and improper ETT sizing is known to cause subglottic stenosis. SGS patients may wish to add their recommended ETT size to a Medical Alert ID.
Erythrocyte sedimentation rate (ESR) – ESR is a blood test for inflammation.
F
Fibroblast – a cell in connective tissue which produces collagen and other fibres.
Flow Volume Loop – A flow volume loop is a visual representation of spirometry data that shows whether airflow is appropriate for a particular lung volume. SGS patients have “flattened” flow volume loops.
Flutter valve – Flutter valve is an airway clearance device to help expel mucus by combining positive expiratory pressure (PEP) therapy with airway vibrations.
G
Glottis – The glottis is the opening between the vocal folds.
Granulomatosis with polyangiitis (GPA) – GPA, formerly Wegener’s Granulomatosis, is an auto-immune collagen vascular disease.
H
Huff coughing – an alternative to deep coughing if you have trouble clearing your mucus. See section on reducing inflammation and mucus
I
Idiopathic subglottic stenosis (SGS or ISS) – refers to narrowing of the upper trachea of unknown cause. The disease is rare, with an estimated incidence of 1 per 400,000 person-years. SGS accounts for approximately 19 percent of patients with laryngotracheal stenosis, with the majority having an identifiable cause such as an iatrogenic injury, neck or throat trauma, or an inflammatory connective tissue disease. The exact pathogenesis of ISS is unknown.
IL-23/IL-17A Axis – The relationship between Interleukin-23 (IL-23) and Interleukin 17A (IL-17A), small proteins called cytokines, is referred to as the IL-23/IL-17A axis. The aberrant mucosal immune activation seen in the large airway of SGS patients helps us understand the molecular pathogenesis of SGS and may lead to more directed targeting with drugs (see The Laryngoscope 2016, Gelbard et al, “Idiopathic Subglottic Stenosis is Associated with Activation of the Inflammatory IL-17A/IL-23 Axis”).
Indirect laryngoscopy – uses a small mirror held at the back of your throat. The health care provider shines a light on the mirror to view the throat area. This is a simple procedure. Most of the time, it can be done in the provider's office while you are awake. A medicine to numb the back of your throat may be used.
In-office injections – see Awake injections
Intubation – see Endotracheal intubation
J
Jet ventilation – Used in conjunction with general anaesthesia, jet ventilation is a transoral means of ventilation during laryngeal surgery and procedures on the upper airway.
K
Kenalog - see Corticosteroids
L
Laryngeal mask airway (LMA) – also known as laryngeal mask— is a medical device that keeps a patient’s airway open during anaesthesia or unconsciousness and can be used as an alternative to intubation during non-airway surgery.
Laryngoscope – A laryngoscope is a rigid or flexible endoscope passed through the mouth and equipped with a source of light and magnification, for examining and performing local diagnostic and surgical procedures on the larynx.
Laryngoscopy – Laryngoscopy is endoscopy of the larynx, a part of the throat, used to view the vocal folds and glottis.
Laryngotracheal reconstruction – Laryngotracheal (luh-ring-go-TRAY-key-ul) reconstruction surgery widens your windpipe (trachea) to make breathing easier. Laryngotracheal reconstruction involves inserting a small piece of cartilage — stiff connective tissue found in many areas of your body — into the narrowed section of the windpipe to make it wider.
Laryngotracheal stenosis (LTS) – Laryngotracheal stenosis, a fixed extrathoracic obstruction, is the abnormal narrowing of the central air passageways, which can occur at the level of the larynx, trachea, carina or main bronchi. SGS patients may also have stenosis at the glottis and/or supraglottic stenosis.
Laryngotracheoplasty – a form of open anterior neck surgery that includes both cartilage tracheoplasty and slide tracheoplasty. It is also known as laryngotracheal reconstruction.
Larynx – The larynx or voice box is an organ in the top of the neck involved in breathing, producing sound, and protecting the trachea against food aspiration. It houses the vocal folds, and manipulates pitch and volume, which are essential for phonation.
Living with Idiopathic Subglottic Stenosis – “Living with Idiopathic Subglottic Stenosis” is a Facebook support group for patients and their families started in 2009 by Catherine Anderson, the NoAAC Patient Partner.
M
Maddern – see Endoscopic tracheal reconstruction
Mayo protocol – The Mayo protocol is an SGS treatment approach that combines antibiotics to suppress inflammation (e.g., Bactrim, azithromycin or erythromycin), corticosteroids to inhibit fibroblast formation and reduce inflammation, and proton-pump inhibitors (PPIs) to reduce gastric acid reflux (gastroesophageal reflux disease or GERD).
Mitomycin-C – Mitomycin-C is a chemotherapeutic agent that can be applied to a stenotic scar at the time of an endoscopic tracheal dilation, although there is no scientific evidence of its effectiveness.
Mucus plug – Mucus impaction or plugging occurs when mucus becomes thick and sticky creating a mass that is difficult to expectorate blocking the airway and possibly resulting in death. Mucus plugs can be avoided by respiratory therapy, medication, and mucolytic therapy.
Mucolytic therapy – helps expel mucus, can include a combination of mucolytic medication (Over the counter (OTC) expectorant cough syrup, throat lozenge with glycerine, pectin, or slippery elm not menthol or eucalyptus; amino acid N-Acetyl Cysteine (NAC) or NAC as a capsule or nebulized NAC, e.g. Mucomyst) and the use of an airway clearance technique (‘huff’ cough ‘huffing’ exercise) or device (flutter valve).
Mycobacterium tuberculosis (MtbC) – MtbC is a pathogenic bacteria in the family Mycobacteriaceae and the causative agent of tuberculosis, yet rather than MtbC, a variant member of the MtbC complex or a closely related novel mycobacterium has been found to be present in SGS tracheal biopsy specimens (see The Laryngoscope 2016, Gelbard et al, “Molecular Analysis of Idiopathic Subglottic Stenosis for Mycobacterium Species”).
N
Nebulizer – A nebulizer is a saline and/or drug delivery device used to administer medication (or saline) in the form of a mist inhaled into the airway to thin mucus making it easier to expectorate.
North American Airway Collaborative (NoAAC) – NoAAC (noaac.net) is a collective of medical centres who are collaborating to investigate the underlying cause of SGS and seeking to provide patients with information that will improve their quality of life through informed decision making. Its leadership team of multi-disciplinary clinicians, surgeons, patient representatives, and health care research personnel volunteers its time and expertise. NoAAC was founded at Vanderbilt University and its director is Dr. Alexander Gelbard. The NoAAC Patient Partner Catherine Anderson established “Living with Idiopathic Subglottic Stenosis” and the NoAAC Patient Representative Kesi-Dorner Wright established the Tracheal Stenosis Foundation.
O
Open anterior neck surgery – includes four surgical procedures – cricotracheal resection, Laryngotracheoplasty (cartilage tracheoplasty and slide tracheoplasty), REACHER and tracheostomy.
Otolaryngology – Otolaryngology is a surgical subspecialty that deals with the ear, nose, and throat (ENT) and related structures of the head and neck. Doctors who specialize in this area are called otolaryngologists, ENT doctors, ENT surgeons or head and neck surgeons.
P
Patient Centered Outcomes Research Institute (PCORI) – PCORI (pcori.org) funds research that can help patients and those who care for them make better-informed decisions about the healthcare choices they face every day, guided by those who will use that information. PCORI funded the Vanderbilt Study.
Peak expiratory flow (PEF) – PEF is a person’s maximum speed of expiration measured with a peak flow meter.
Peak flow meter – A peak flow meter is a portable, hand-held device that measures how well your lungs expel air. It measures your ability to push air out of your lungs in one fast blast by blowing hard through a mouthpiece (i.e. the force of airflow through the bronchi and thus the degree of any airway obstruction). It gives you a numeric reading on a scale; peak flow readings are higher when the airway is open, and lower when the airway is narrowed (constricted).
Phonation – Phonation is the process by which the vocal folds produce certain sounds through periodic vibration.
Pulmonary Function Test (PFT) – PFT, also called spirometry or lung function testing, is a group of tests that evaluate the respiratory system, including the function of lung capacity, chest wall mechanics, etc.
Pulse oximeter – Pulse oximeter is a device placed on your finger to measure oxygen saturation.
R
REACHER (Retrograde, endoscopically assisted cricoid hypertrophic epithelial resection) – REACHER is an open anterior neck surgical procedure pioneered by Dr Robert Lorenz of Cleveland Clinic, Ohio USA, where the diseased lining of the cricoid is removed and the outer cartilage is preserved.
Rough Guide – Idiopathic Subglottic Stenosis – A Rough Guide for Beginners by Catherine Anderson, the NoAAC Patient Partner, is a reference manual for SGS patients and their doctors.
S
Saline – also known as saline solution, is a mixture of sodium chloride in water (vials come in 0.9% and 3.0% strengths) that can be administered orally with a nebulizer.
Self-help group tracheal stenosis – self-help group for patients suffering from ISGS in German-speaking countries. Contact at www.trachealstenose.com or trachealstenose@gmail.com.
Slide tracheoplasty – a type of laryngotracheoplasty, is a surgical procedure used for more severe grades of long-segment tracheal stenosis or complete tracheal rings where the narrowed trachea is divided across the middle of the stenosis. The back of the lower tracheal segment and front of the upper tracheal segment are cut, and the opened ends are then slid onto each other and sutured in place to create an airway that is twice as wide and half as long.
SLT/SLP – see speech and language therapist/pathologist
Speech and Language Therapist/Pathologist – Provide treatment, support and care for people who have difficulties with communication, eating, drinking and swallowing.
Spirometry – Spirometry, the most common pulmonary function test, measures lung function, specifically the amount and/or speed of air that can be inhaled and exhaled.
Steam inhalation – Steam inhalation is a method of introducing warm, moist air into the lungs via the nose and throat for therapeutic benefit such as thinning mucus by breathing into a portable device. Essential oils can also be used.
Stenosis – an abnormal narrowing or contraction of a body passage or opening.
Steroid injection – refers to the submucosal injection of corticosteroids into the stenotic scar administered either in an office setting (externally through the neck or via transnasal endoscope) or in an operative setting with or without dilation (under moderate sedation or under general anaesthesia via transoral endoscope or transnasal videoscope).
Stoma – any opening in the body. A patient with a tracheotomy has a stoma in their trachea.
Stridor – Stridor is a high-pitched breath sound (commonly called the “Darth Vader” sound) resulting from turbulent air flow in the larynx or lower in the bronchial tree, caused by a narrowed or obstructed airway.
Subglottic stenosis – Narrowing of the subglottis is known as subglottic stenosis.
Subglottis – the subglottis or subglottic region is the lower portion of the larynx, extending from just beneath the vocal folds down to the top of the trachea.
Supraglottic stenosis – Supraglottic stenosis is narrowing of the airway above the glottis.
T
Trachea – or windpipe is a cartilaginous tube that connects the pharynx and larynx to the lungs, allowing the passage of air, and branches into the two primary bronchi.
Tracheal reconstruction – a surgical procedure to remove a portion of the trachea with a long stenotic scar and to insert a stent or piece of rib into the neck to replace the lost cartilage and prevent the neck from collapsing.
Tracheal resection – a surgical procedure to remove a portion of the narrowed portion of a patient’s trachea or windpipe through an incision in the neck and reattaching the upper and lower healthy parts with stitches.
Tracheal stenosis – see Laryngotracheal stenosis
Tracheal T-tube – a silicone stent for the trachea with an external limb.
Tracheal Stenosis Foundation (TSF) – TSF is a non-profit organization founded by patient Kesi Dorner-Wright that raises awareness and holds an (almost) annual patient-centered conference in the USA whose speakers include members of the NoAAC leadership team.
Tracheotomy – also known as tracheostomy is a surgical procedure that consists of making an incision on the anterior aspect of the neck and opening a direct airway through an incision in the trachea.
V
Vanderbilt Study – The commonly called Vanderbilt Study is an ongoing clinical trial (2015-2018) formally known as the ‘NoAAC PR-02 SGS Clinical Trial - Treatment Alternatives in Adult Rare Disease; Assessment of Options in Idiopathic Subglottic Stenosis’ (https –//clinicaltrials.gov/ct2/show/NCT02481817). Dr. Alexander Gelbard of Vanderbilt University is the study’s Principal Investigator.
Videoscope – A videoscope, also called a transnasal endoscope or distal chip video esphagoscope, is a medical device that records video of a procedure, such as transnasal steroid injections.
Vocal folds – Vocal folds or vocal cords are composed of twin folds of mucous membrane stretched horizontally, from back to front, across the larynx.
W
Wegener’s Granulomatosis (WG) - see Granulomatosis with polyangiitis (GPA)
Z
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