2IntroductionSubglottic stenosis (SGS) is a congenital or acquired narrowing of the subglottic airway.Acquired SGS is the most common acquired anomaly of the larynx in children.The most common abnormality requiring tracheotomy in children younger than 1 year.In the early 20th century SGS was rare.3- Most cases occurred in adults4- This resulted from the increased survival of low-birth-weight infants and the increased use of intubation in this population. In addition, long term intubation became accepted as an alternative to tracheotomy
3IntroductionIn 1965, McDonald and Stocks introduced long-term intubation as a treatment method for neonates in need of prolonged ventilation for airway support.In the 1960s, the incidence of acquired SGS began to dramatically increase in the neonatal population.New attention on the pediatric larynx, and airway reconstruction and expansion techniques were developed.
4AnatomyThe subglottis is defined as the area extending from the lower surface of the true vocal cords to the lower surface of the cricoid cartilage. In adults this corresponds to approximately 10 mm inferior to the anterior commissure and 5 mm inferior to the posterior commissure.
5Anatomy Infant vs. Adult At birth, the infant larynx is approximately one third the size of the adult larynx.The narrowest portion of the airway in the older child and adult is the glottic aperture, while in the infant is the subglottis.The infant larynx is positioned higher in the neck than the adult larynx.
6Anatomy Infant vs. Adult The structures of the infant larynx are more pliable and less fibrous making the infant airway more susceptible to narrowing from edema and less easily palpable.One millimeter of edema circumferentially in the subglottis reduces the cross-sectional area by 60%.
8Congenital SGS Accounts for 5% of all cases. The third most common congenital airway problem (after laryngomalacia and vocal cord paralysis). Thought to be secondary to failure of laryngeal lumen to recanalize.Divided into membranous or cartilaginous types.
9Congenital SGSDefined as subglottic diameter < 4.0 mm in full term infants and < 3.5 mm in premature infants.It is considered if there is no Hx of intubation or other forms of laryngeal trauma.Associated with other congenital malformations.
10Acquired SGS Accounts for 95% of all cases. Numerous causes including intubation (90%), trauma, infection/inflammation, thermal or caustic injuries to the subglottic mucosa.All studies published after 1983 reported an incidence of neonatal SGS in intubated neonates as < 4.0%, and all studies published after 1990 reported an incidence of < 0.63% (Walner, 2001).
11Acquired SGSPathogenesis: mucosal pressure leading to ulceration leading to chondritis and finally deposition of fibrous material.Risk factors of developing SGS in intubated patients:Prolonged intubation (most important).Size of endotracheal tube.Increased motion of tube.Repeated intubations.Birth weight less than 1500g.Infection.GERD.Systemic illness, malnutrition, anemia, and hypoxia.
13PresentationChildren with SGS have an airway obstruction that may manifest in several ways.If the stenosis is severe and congenital, the patient will present with airway distress at birth.In neonates, SGS may manifest as stridor and obstructive breathing after extubation that requires reintubation.In children and adults usually present with a history of prior intubation with symptoms of progressive shortness of breath and stridor.
14Presentation The stridor in SGS usually is biphasic. Depending on the severity, SGS can cause patients to have decreased subglottic pressure and a hoarse or a weak voice.Question parents about: duration, progression of Sx, change in voice or cry, hx of prematurity, birth trauma, hx of intubation, feeding problems, recent trauma or foreign body exposure, hx of GERD.
15Examination V/S : Assess presence of tachypnea, ↓ SpO2. Evaluate the child's initial overall appearance:Presence of increased breathing difficulty, especially during periods of heightened emotion.Presence of cyanosis, suprasternal, substernal, intracostal retractions, or nasal flaring.Evaluate presence of stridor & type.Evaluate voice or crying quality for weakness, hoarseness, breathiness, or complete absence.
16ExaminationA complete head and neck exam should be performed on all patients.Flexible fiberoptic nasopharyngolaryngoscopy:Vocal cord mobility.Reflux changes.Subglottic abnormalities.Other glottic and supraglottic abnormalities may be detected.
18Examination Evaluate the child's neurological status. In the presence of tracheotomy, evaluate the patient's breathing while tracheotomy is occluded.Auscultate the child's lung field and neck to elicit any airway obstructive symptoms and to evaluate the status of their pulmonary function.Identify associated facial abnormalities such as cleft palate, choanal atresia, retrognathia, and facial deformities.
19Radiologythe initial radiographic study used to evaluate a child with airway obstruction is an anteroposterior and lateral plain neck radiography.Neck AP x-ray of patient with post-intubation subglottic stenosis, as shown by the narrowing in the tracheal lumen marked by the arrow.
20Radiology Airway fluoroscopy & Ba swallow can be helpful. CT scans and MRIs are not often used in the primary evaluation of SGS.
21Other Important Investigations Evaluate the presence of GERD by use of dual-channel pH probe testing.The current criteria for diagnosing lower esophageal acid reflux is: a pH of < 4.0 for > 10% of the time.
22DiagnosisThe gold standard for diagnosis of any laryngotracheal abnormalities is direct laryngoscopy and tracheobronchoscopy under general anesthesia.Important things to document: The outer diameter of the largest bronchoscope or endotracheal tube that can be passed through the stenotic segment.The location and length of the stenosis.Other separate sites of stenosis.Other airway anomalies in infants.Reflux changes.
23GradingThere are two widely excepted staging systems for classifying subglottic stenosis: Myer-Cotton grading system.McCaffrey system.
25Stage Description McCaffrey system (1992) I II III IV Classifies laryngotracheal stenosis based on the subsites involved and the length of the stenosis.StageDescriptionILesions are confined to the subglottis or trachea and < 1cm longIILesions are isolated to the subglottis and > 1 cm longIIISubglottic/tracheal lesions not involving the glottisIVLesions involve the glottis
29Management Medical Observation Tracheostomy Airway expansion procedures
30Medical Diagnosis and treatment of GERD Pediatric consultation with primary physicians and specialists (pulmonary, GI, cardiology etc.)AdultAssess general medical status.Consultation with primary physician and specialists.Optimize cardiac and pulmonary function.Control diabetes.Discontinue steroid use if possible before LTR.
31ObservationPatients (children and adults) with Cotton-Myer grade I and mild grade II SGS may be managed with close observation (Walner and Cotton,1999)If no retractions, feeding difficulties, or episodes of croup requiring hospitalization.Should be followed with growth curves by a pediatrician and/or neonatologist.It is recommend to repeat endoscopy every months to measure the diameter of the airway to ensure that it is enlarging as the child grows.
32TracheostomyOften the initial step in treatment of pediatric acquired SGS.Allows these patients time to grow before definitive surgical treatment is performed.Cotton and Walner recommend waiting until the infant is at least 10 kg before performing airway reconstruction. Allow for optimization of pulmonary function in patients with BPDOften not performed in infants with congenital SGS.2-5% mortality in children (Lesperance, 1996)Accidental decannulation and plugging
33Airway Expansion Procedures The site, grade, and length of stenosis are the major factors in determining which surgical procedure will be used for reconstruction.The goal is to maintain vocal function and allow for early decannulation with subsequent unrestricted activity.
34Airway Expansion Procedures Contraindications:AbsoluteTracheostomy dependent (aspiration, severe BPD).Severe GERD refractive to surgical and medical therapy.RelativeDiabetes.Steroid use.Cardiac, renal or pulmonary disease.
36EndoscopicMild stenosis (Cotton-Myer grades I and II) can usually be treated with endoscopic techniques such as dilation or CO2 laser resection.Endoscopic dilationPracticed frequently before advent of LTR.Requires multiple repeat procedures.Low success rate.Reported success rates with endoscopic laser resection of Grade I and II stenosis range from 66-80%.
37Endoscopic Factors associated with failure Previous attempts Circumferential scarringLoss of cartilage supportExposure of cartilagePosterior inlet scarring with arytenoid fixationCombined laryngotracheal stenosis with vertical length >1cm
38Endoscopic treatment of laryngeal and tracheal stenosis has mitomycin C improved the outcome? PEREPELITSYN et al, 2004Results indicate that a statistically significant increase, from <20% to 75%, in the success rate of endoscopic treatment of acquired upper airway stenosis when topical mitomycin C is added to the treatment regimen.
39Mitomycin Does Not Prevent Laryngotracheal Repeat Stenosis After Endoscopic Dilation Surgery: An Animal Study Eliashar et al, The LaryngoscopeMitomycin exerts a benefit equal to that of normal saline when applied to a well established laryngotracheal stenosis in dogs and does not prevent repeat stenosis after endoscopic dilation surgery.
40Anterior cricoid split The anterior cricoid split (ACS) procedure was originally described for a neonate who has had multiple failed extubations instead of performing a tracheostomy (Cotton and Seid, 1980).Strict criteria for ACS have been established by Cotton and include:Extubation failure on two occasions or more due to laryngeal pathologyPatient weight > 1500 gramsMinimal supplemental oxygen requirementsNo active respiratory infectionGood pulmonary and cardiac function
41Anterior cricoid split The procedure is performed after direct laryngoscopic and bronchoscopic confirmation of the diagnosis.A vertical midline incision is made through the cricoid cartilage and first two tracheal rings as well as the lower thyroid cartilage.This allows the cartilages to spring open and allow edematous mucosa to drain, increasing airway size.Prolene stay sutures are placed on either side of the cricoid cartilage and the skin is re-approximated after placement of a drain.
43Anterior cricoid split Child remains intubated 7-14 days in ICU.Abx and antireflux meds while intubated & steroids in the first 48 hours.Complications include pneumothorax, pneumomediastinum, subcutaneous emphysema, wound infection, and persistent SGS.Success rate of 75.9%
44Laryngotracheal Reconstruction Laryngotracheal reconstruction involves scar division with distraction of the edges by interposition of graft material (augmentation) to widen the airway lumen.Used In older pediatric patients with higher grade stenosis.There are several techniques depending on the location and severity of the stenosis.
45Laryngotracheal Reconstruction It can be performed in2 stages with a tracheostomy and formal stenting orIn a single stage by using the endotracheal tube as a stent (single-stage LTR, SS-LTR).Laryngeal stents include: teflon stents [Aboulker stent (short or long)], and silastic stents(Montgomery stent).
46Single Stage LTRIntroduced in 1991 by Seid et al who reported the use of single-stage laryngotracheoplasty (LTP).Their approach to the airway resembles ACS; however, instead of leaving the area anterior to the fibrosis, a piece of costal cartilage was placed.Remove ET tube when air leak at 20 cm H2O.the procedure failed in a patient who had complete glottic and SGS (grade IV).
47LTR with stenting For severe SGS (grade III-IV). Introduced in 1972 by Fearon and Cotton.Widely used.Tracheostomy and stent in place for several weeks to months.Zalzal and Cotton have reported success rates higher than 90%.
48LTR with stentingAnterior: adequate for isolated anterior subglottic stenosis.Anterior/posterior: for circumferential or posterior SGS.Four quadrant division: for complete SGS.
50LTR with stenting Complications: Dysphagia Aspiration Granulation tissueDislodgement of stent
51Graft materialCartilage is preferred because it has a lower rate of resorption, are easy to carve, and are viable without a vascular pedicle.Rib and auricular most commonly used.
52Post-operative Care Abx Antireflux Often discharged in several days Intensive care unitIntensivist familiar with these casesAbxAntirefluxOften discharged in several daysRepeat endoscopy after 3-4 weeks for stent evaluationOnce the stent is removed, follow-up bronchoscopies are performed to confirm the stenosis has not recurred before the patient is decannulated.
53Cricotracheal resection First reported in 1970 and popularized in the 90s.More technically challenging than split procedures.This technique is indicated if there is severe deformity of the cricoid making grafting very likely to fail.95% decannulation rate (Monnier et al., 2003)
54Cricotracheal resection The best candidates for CTR are patients with severe SGS (grade III-IV) without associated glottic pathologic conditions and with a margin of at least 4 mm in the healthy airway below the vocal folds and above the stenosis.Entails removing the anterior half of cricoid lamina as well as the mucosa covering the posterior cricoid lamina.First several tracheal rings are removed.Two ends of the airway are brought back together.
56Cricotracheal resection Complications:Anastamosis failureGranulation tissueRLN injuryArytenoid prolapseRestenosisWound infectionNeed for further proceduresRe-intubation
57PreventionIt is important for the primary physician, anesthatist and/or intensivist to be aware of SGS as a complication of endotracheal intubation. This starts with education.Choosing the appropriate size ETT should be determined by the patients age and size.Blind intubations should always be avoided if possible.
58PreventionOnce intubated, multiple measures should be taken to prevent the tube from moving.There is no definitive safe period for intubation.Antireflux medications.