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11/19. Anaesthesia for Treacher Collins syndrome using a laryngeal mask airway.

    patients with Treacher Collins syndrome pose a serious problem to anaesthetists in maintaining their airway because of retrognathia. Two patients with Treacher Collins syndrome undergoing tympanoplasty are reported in whom a laryngeal mask was used in place of an endotracheal tube for airway maintenance.
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ranking = 1
keywords = airway
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12/19. sleep apnoea syndrome associated with maxillofacial abnormalities.

    Four cases with Obstructive sleep Apnoea syndrome (OSAS) are presented. They consisted of two cases with TMJ ankylosis with micrognathia, one case with Treacher Collins syndrome, and one case with the Long face syndrome. Standard and specific cephalometric parameters were obtained to detect the site of the obstruction. Polysomnographic studies yielded information regarding the patient's sleep-wake state, respiratory and cardiac functioning, pre- and post-operatively. A temporary tracheostomy corrected the symptoms in one patient but the syndrome recurred when it was closed. Surgical correction of the maxillofacial anomalies will re-establish normal sleep patterns preventing OSAS.
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ranking = 0.016044856291584
keywords = obstruction
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13/19. Respiratory inductance plethysmography and pulse oximetry in the assessment of upper airway patency in a child with Goldenhar's syndrome.

    The anaesthetic management of a child with Goldenhar's syndrome and upper airway dysmorphology is presented. She had a history of severe dyspnoea due to deterioration of cor pulmonale caused by upper airway obstruction. The patency of the upper airway and oxygenation were evaluated during the perioperative period with respiratory inductive plethysmography (RIP) and pulse oximetry, which did not show severe upper airway obstruction or oxygen saturation below 80 per cent. Tracheal intubation was performed under inhalational anaesthesia with spontaneous breathing. This case suggests that RIP and pulse oximetry may be useful monitoring devices in the anaesthetic management of patients with upper airway problems as in Goldenhar's syndrome.
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ranking = 3.4591706416018
keywords = airway obstruction, airway, obstruction
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14/19. Anaesthesia for Treacher Collins and Pierre Robin syndromes: a report of three cases.

    We present three patients with Treacher Collins or Pierre Robin syndromes who had historical and physical evidence of airway obstruction, difficulty feeding, and sleep disturbances. These preoperative findings correlated with difficult airway management intraoperatively. Based on this experience, we recommend that children with obstructive symptoms have laryngoscopy prior to anaesthetic induction. If the glottic opening is visualized, inhalational induction can proceed. If the glottic structures cannot be visualized, then the anaesthetist must choose between awake oral or nasal intubation, elective tracheostomy, or fiberoptic intubation. In all cases, a tracheostomy tray should be ready and a surgeon experienced in paediatric tracheostomy should be in attendance. After intubation, anaesthesia is best maintained with oxygen and a potent inhalational agent. Extubation should only be done with the patient fully awake and with emergency airway equipment immediately available. Postoperatively, these patients should be transferred to an intermediate care area or intensive care unit where they can be observed closely since delayed complications of airway obstruction are common in this group of patients.
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ranking = 2.6258373082684
keywords = airway obstruction, airway, obstruction
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15/19. Obstructive sleep apnea in Treacher-Collins syndrome.

    Studies of the Treacher-Collins syndrome have emphasized hearing and surgical considerations. Although craniofacial anomalies have been associated with respiratory disorders in infancy, the presence of such problems in older children has not been emphasized. An eight-year-old with Treacher-Collins syndrome presented a history of recent behavioral problems at home, poor attention span and performance in school, daytime somnolence, and sleep apnea with relatively long periods of chest movement but no airflow. He also had abnormal sleep behavior consisting of rocking to and fro on his hands and knees, often to such an extent that his nose became abraded. ICU monitoring with observation and recording of sleep patterns and sounds, and fluoroscopy of his upper airway utilizing cineradiography while asleep confirmed the diagnosis of obstructive sleep apnea. The patient subsequently underwent an orthognathic surgery consisting of insertion of rib bone grafts after anterior advancement of his mandible. This procedure resulted in disappearance of the obstructive sleep apnea and associated symptoms. Because of micrognathia, patients with Treacher-Collins syndrome are at high risk for developing obstructive sleep apnea. Surgical correction of their deformities can result in improvement in cosmetic appearance as well as in resolution of the obstructive episodes with improvement in performance and behavior.
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ranking = 0.16666666666667
keywords = airway
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16/19. Orotracheal intubation through the laryngeal mask airway in paediatric patients with Treacher-Collins syndrome.

    The laryngeal mask airway (LMA) is useful as an airway intubator (conduit) for an intubating tracheal bougie or fibreoptic bronchoscope, over which a tracheal tube is passed. However, in our paediatric patients with Treacher-Collins syndrome, only the latter technique was successful. This was attributed to the fact that a posteriorly protruded tongue displaced the LMA, made the glottis move considerably anterior and interfered with the attempts to enter the trachea with a bougie. Downward displacement of the epiglottis, which can sometimes impair the intubation technique through the LMA, was not observed in our patients. Partial obstruction of a tracheal tube within the LMA occurred in one of the patients.
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ranking = 1.0160448562916
keywords = airway, obstruction
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17/19. Mandibular lengthening by distraction for airway obstruction in Treacher-Collins syndrome.

    Mandibular lengthening by distraction was performed in a 6-year-old tracheostomy-dependent Treacher-Collins syndrome patient. Detailed preoperative imaging revealed an occluded retrotongue base pharyngeal airway, which, following mandibular distraction, became patent and permitted tracheostomy removal. Mandibular distraction as a technique must be targeted toward clinical problems--management of upper-airway obstruction may be one such scenario.
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ranking = 5.8979266040044
keywords = airway obstruction, airway, obstruction
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18/19. Cranial base and face in mandibulofacial dysostosis.

    Using longitudinal roentgencephalometry, we studied craniofacial growth in two children with mandibulofacial dysostosis. In one child, data were supplemented by three-dimensional reconstructions of CT scans and stereolithographic models of the craniofacial skeleton. Progressive basilar kyphosis was found in both children, the hypothesized caused being bending of the cranial base at the level of the sphenofrontal suture. Such bending acting in concert with abnormal growth of the mandible led to impairment of the airway. We advocate careful monitoring of craniofacial growth and respiratory function in mandibulofacial dysostosis from birth through adolescence.
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ranking = 0.16666666666667
keywords = airway
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19/19. Diagnostic difficulties in cases of sudden death in infants with mandibular hypoplasia.

    Infants with mandibular hypoplasia are at risk of sudden death from cardiorespiratory arrest secondary to upper airway obstruction. To evaluate diagnostic difficulties that may occur at autopsy in such infants, the autopsy files at the Adelaide Children's Hospital (ACH) for 36 years, 1959 to 1994, were reviewed. Eight cases were identified (age range, 2 days to 10 months; mean age, 2.2 months; male/female ratio, 5:3). In all cases, death was considered most likely due to airway obstruction related to mandibular hypoplasia or its treatment. Although death occurred in the hospital in five cases, one infant suddenly collapsed at home while feeding and died, and two infants were unexpectedly found dead in their cribs at home. Three infants had defined genetic syndromes. Although all the infants had histories of antemortem airway obstruction, one infant had normal oxygen saturation studies before hospital discharge, and one infant had a tracheostomy. Acute bronchopneumonia was an exacerbating factor in one case. Assessment of mandibular size is important in any infant who dies unexpectedly; and if hypoplasia is found, careful review of the clinical details for evidence of airway obstruction is necessary to help distinguish these cases from sudden infant death syndrome (SIDS). Sudden death may, however, occur in infants with mandibular hypoplasia in spite of apparent clinical stability before death with no significant recent episodes of oxygen desaturation.
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ranking = 4.5850079498702
keywords = airway obstruction, airway, obstruction
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