Severity of Mandibular Dysmorphology in Treacher Collins Syndrome for Stratification of Perioperative Airway Risk
Carlos E. BarreroLarissa E. WietlisbachMatthew E. PontellLauren K. SalineroConnor S. WagnerJordan W. SwansonScott P. BartlettJesse A. Taylor
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Background: Patients with Treacher Collins syndrome (TCS) and attendant airway dysmorphology may be predisposed to airway complications in the perioperative period. However, limited data correlates severity of mandibular hypoplasia and airway status. This study aims to improve risk stratification for perioperative airway insufficiency in TCS by using a previously proposed mandibular severity index. Methods: Patient demographics, perioperative airway status, difficulty of intubation, and Cormack Lehane grade were collected and compared using a TCS mandibular hypoplasia severity grading scale in patients with TCS treated between 2000 and 2022. Results: Twenty-six patients underwent 222 procedures with institutional mandibular severity gradings as follows: 23% Grade I, 31% Grade II, 39% Grade III, 8% Grade IV. Our severity index was associated with intubation difficulty ( P <0.001) and difficult airway status ( P <0.001), with 72% of difficult airways found in grade III and grade IV patients. Mandibular retrusion and ramal hypoplasia subscores were positively correlated with measures of airway severity ( P <0.001), whereas the gonial angle was negatively correlated ( P <0.001). Age was negatively correlated with difficult visualization for endotracheal intubation ( P =0.02) but had no association with difficult airway status ( P =0.2). Conclusions: This study found a positive correlation between severity of maxillomandibular dysmorphology and perioperative airway difficulty in TCS patients. Our findings suggest that severely affected patients require heightened vigilance throughout life, as difficult airways may not completely resolve with aging. Given the risk of morbidity and mortality associated with airway complications, proper identification and preparation for challenging airways is critical for TCS patients.Keywords:
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Summary Upper airway obstruction and difficult tracheal intubation are often encountered in patients with Treacher Collins syndrome (mandibulofacial dysostosis). In this case report, the use of a laryngeal mask airway (LMA TM ) in a 10‐day‐old newborn with severe Treacher Collins syndrome and acute airway obstruction is described. It successfully relieved the airway obstruction and was left in situ for an exceptionally long period of 4 days. The difficult decisions with respect to the management of the airway and specifically the role of the laryngeal mask are described. In our opinion, in some newborns with severe mandibulofacial disorders and upper airway obstruction, where conservative airway management procedures have failed, the laryngeal mask can be considered not only to relieve the obstruction but also to buy time until there is full insight into the medical condition and its consequences.
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Pierre Robin sequence (PRS), also called Robin sequence, is a congenital anomaly characterized by a triad of micrognathia, glossoptosis, and upper airway obstruction. Infants with PRS can present with varying degrees of respiratory difficulty secondary to upper airway obstruction. There has been no consensus for the treatment of upper airway obstruction in infants with PRS, but recent studies recommend attempting non-surgical interventions before surgical treatment. In this case report, we present 3 cases of infants diagnosed with PRS who showed persistent respiratory difficulties after birth. Before considering surgical intervention, insertion of a nasopharyngeal airway was attempted in these infants. Following this procedure, symptoms of upper airway obstruction were relieved, and all infants were discharged without surgical interventions; the nasopharyngeal airway was removed 1 to 2 months later. To date, no infant has shown signs of upper airway obstruction. Nasopharyngeal airway insertion is a highly effective and less invasive treatment option for infants with PRS. However, it is not widely known and used in Korea. Nasopharyngeal airway insertion can be preferentially considered before surgical intervention for upper airway obstruction in such infants. Keywords: Pierre Robin syndrome; Upper airway obstruction; Nasopharyngeal airway
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Topicalisation of the airway by various techniques has routinely been recommended for awake fibre-optic bronchoscopy in cases of difficult airway. However, topicalisation by itself can cause airway obstruction by decreasing the tone of the laryngeal muscles and causing a dynamic air inflow obstruction. Two cases of difficult airway are illustrated where anaesthetising upper airway with nebulisation with 4% lignocaine (Xylocaine™) or 2% lignocaine (Xylocaine™) jelly resulted in stridor and upper airway obstruction. This is the first reported case of airway obstruction after lignocaine (Xylocaine™) jelly. We would like to highlight that topicalisation of airway, once thought as a relatively safe technique, can cause airway collapse if not detected and anticipated at the earliest. Pre-operative spirometry and airway ultrasonography can be useful in detecting the patients at risk of developing airway obstruction. Using a nasopharyngeal airway during topicalisation can serve as a valuable device in preventing total airway obstruction in susceptible patients.
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Abstract We developed a technique to determine the site of upper airway obstruction in patients with idiopathic obstructive sleep apnea (OSA). This technique is based on the analysis of inspiratory airflow pressures at various levels of the pharyngeal airway during sleep. Pharyngeal pressure was measured by a moveable Millar catheter pressure transducer. The catheter's position in the airway was localized radiographically. Ten patients with OSA were tested: five patients were found to have upper airway obstruction at the level of the soft palate, and five had upper airway obstruction at the base of the tongue. We concluded that measuring airway pressures at multiple sites along the airway is useful in localizing the site of obstruction in patients with OSA, and may have important implications in terms of the patient's response to surgical treatment.
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The aim of the study was to evaluate noninvasive continuous positive airway pressure as first-line treatment for upper airway obstruction in neonates with Pierre Robin sequence. Forty-four neonates were enrolled and classified in 4 groups: ventilator-dependent patients (severe upper airway obstruction group), patients with clinical upper airway obstruction, an abnormal (moderate upper airway obstruction group) or normal polygraphy (mild upper airway obstruction group), and those with no clinical upper airway obstruction (no upper airway obstruction group). Five neonates in the severe upper airway obstruction group (n = 9) were successfully managed by noninvasive continuous positive airway pressure and 4 required a tracheotomy. The moderate upper airway obstruction group (n = 4) was successfully managed by noninvasive continuous positive airway pressure and the other patients by prone positioning. Continuous positive airway pressure represents an effective treatment of upper airway obstruction in Pierre Robin sequence and may be recommended in selected patients.Therapeutic, IV.
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Le Fort fractures are a part of the facial fracture spectrum, sustained from significant forces of impact to the midface. The mechanism of airway obstruction in Le Fort fractures is attributed to midface instability with posterior inferior displacement into the oropharyngeal airway. Otolaryngologists often participate in the evaluation and management of such patients, securing the airway, if necessary. It is important, therefore, to understand the mechanisms responsible for acute airway obstruction in these types of fractures. A retrospective review of 64 cases of Le Fort fractures, representing a 3-year period, was performed to determine the factors responsible for acute airway obstruction. The review disclosed that airway obstruction is due most often to hemorrhage into the upper airway from multiple sources, with inability to handle blood and the oral secretions. An emergency airway was required by one third of the patients with Le Fort fractures in this review.
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The most common indication for employing the fibreoptic technique for intubation is when a difficult intubation is anticipated. It may also be used when intubation unexpectedly proves difficult with a rigid laryngoscope in anaesthetized patients. However, failures with orotracheal fibreoptic intubation have been reported in up to 9.5% of cases, although only two possible equipment related causes of intubation difficulties have been identified. The Berman intubating airway and the Ovassapian intubation airway in fibreoptic orotracheal intubation have been compared in 65 patients. The study was randomized and for fibreoptic endoscopy each patient served as his or her own control. The results indicate that both airways can be used for orotracheal fibreoptic intubation in anaesthetized patients. The Berman airway offers easier visualization of the vocal cords than the Ovassapian airway, provided that the Berman airway is of an adequate size and positioned in the midline. Upon impingement of the intubation tube, completing the tracheal intubation is more difficult with the Berman airway than the Ovassapian airway.
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