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1/19. Bilateral congenital choanal atresia and absence of respiratory distress.

    Bilateral congenital choanal atresia is considered a lethal congenital malformation in an obligatory nasal breathing neonate. Described herein are two cases of bilateral choanal atresia associated with craniofacial anomalies who did not present respiratory distress in the neonatal period. Our first patient had a complete unilateral cleft lip which facilitated oropharyngeal respiration. The second patient presented wory distress in the neonatal period by providing an oropharyngeal airway.
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ranking = 1
keywords = airway
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2/19. Treacher Collins syndrome with acute airway obstruction.

    Treacher Collins syndrome presents with cranio-facial deformities of variable expressions and severity, but rarely is it associated with acute airway obstruction of such severity that it necessitates surgical intervention. The authors describe their experience with such a neonate, who presented with acute airway obstruction successfully managed with a tracheostomy. The relevant English literature is reviewed.
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ranking = 41.265071548832
keywords = airway obstruction, airway, obstruction
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3/19. Anaesthetic implications of Nager syndrome.

    Nager acrofacial dysostosis is an oromandibular hypogenesis syndrome with associated limb abnormalities. Although it shares some phenotypic features with Treacher-Collins syndrome, it is recognized as a separate disorder. The physical features of Nager syndrome include down slanted palpebral fissures, malar hypoplasia, a high nasal bridge, atretic external auditory canals, cleft palate and micrognathia. Preaxial limb malformations include absent or hypoplastic thumbs, hypoplasia of the radius and shortened humeral bones. Of primary concern to the anaesthetist are the midface and mandibular manifestations which may complicate perioperative airway management. These problems may also manifest in the postoperative period with airway obstruction. Associated defects have included vertebral malformations with reports of cervical spine involvement, congenital cardiac defects and upper limb defects affecting the preaxial or radial side. We describe a 7-year-old boy with Nager syndrome who required anaesthetic care during placement of a syringopleural shunt for drainage of a spinal cord syrinx. The perioperative implications of this disorder are reviewed.
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ranking = 7.8775119248053
keywords = airway obstruction, airway, obstruction
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4/19. Difficult paediatric intubation when fibreoptic laryngoscopy fails.

    We report an unusual problem with fibreoptic bronchoscopy in an 8-year-old girl with Negar syndrome. She had a history of difficult airway since birth, and had undergone mandibular distraction for severe obstructive sleep apnoea when she was aged 2 years. Nagar syndrome is a Treacher-Collins like syndrome with normal intelligence, conductive bone deafness and problems with articulation. The patients have malar hypoplasia with down slanting palpebral fissures, high nasal bridge, micrognathia, absence of lower eyelashes, low set posteriorly rotated ears, preauricular tags, atresia of external ear canal, cleft palate, hypoplasia of thumb, with or without radius, and limited elbow extension. Protracted attempts with a fibreoptic bronchoscope failed to visualize the glottis, and this was only possible when the tube was guided to the larynx by blind nasal intubation. Apparently, the healing of the wounds for the mandibular distraction in the mandibular space on the inside of the rami of the mandible had caused differential fibrosis on either side of the hyoid, leading to a triplane distortion of the larynx with a left shift, clockwise rotation to a 2-8 o'clock direction and a slight tilt towards the left pharyngeal wall. The large epiglottis overlying this had precluded a view of the larynx. Finally, the older technique of breathguided intubation facilitated fibreoptic bronchoscopy to achieve tracheal intubation.
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ranking = 1
keywords = airway
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5/19. The prolonged use of the laryngeal mask airway in a neonate with airway obstruction and Treacher Collins syndrome.

    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 (LMATM) 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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ranking = 62.116364536192
keywords = airway obstruction, airway, obstruction
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6/19. Use of the Augustine stylet anticipating difficult tracheal intubation in Treacher-Collins syndrome.

    Treacher-Collins syndrome is a familial and congenital condition often associated with a difficult airway. Although the condition is rare, the anesthesia care provider may encounter it on occasion. This report describes a patient with Treacher-Collins syndrome scheduled to undergo facial reconstruction (fore-head plasty, brow lift, and rhinoplasty) as the sixth of multiple operations. A nasotracheal intubation using the stylet component of a recently introduced airway device, the Augustine Guide, was successfully performed. This is believed to be the first reported use of this method using the Augustine stylet.
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ranking = 2
keywords = airway
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7/19. Fiberoptic tracheal intubation through a laryngeal mask airway in a child with Treacher Collins syndrome.

    Treacher Collins syndrome (TCS) is a rare inherited condition characterized by bilateral and symmetric abnormalities of structures within the first and second bronchial arches. The mechanism of inheritance is autosomal dominant with variable expressivity. Because of this variability in expression, some affected individuals exhibit virtually no overt clinical manifestations. However, most children with TCS present with the following classic facial features: down-sloping palpebral fissures, colobomata of the lower eyelid, scanty lower eyelashes, malar hypoplasia, and micro- or retrognathia. cleft palate is present in up to 35% of patients and an additional 30-40% have congenital palatopharyngeal incompetence. Abnormalities of the ear are very common and vary from minor malformations to severe microtia and hearing loss. hearing loss may be due to atresia of the auditory canals or ossicular malformation of the middle ear. Despite these many development abnormalities, TCS patients are usually of normal intelligence. We report the case of a 3 1/2-yr-old patient with TCS undergoing cleft palate repair and discuss fiberoptic intubation through a laryngeal mask airway using two endotracheal (ETT) tubes secured via an ETT connector.
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ranking = 5
keywords = airway
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8/19. Mandibular lengthening by distraction for airway obstruction in treacher-collins syndrome: the long-term results.

    Mandibular lengthening by distraction was performed in a 6-year-old severely affected Treacher-Collins syndrome patient who was tracheostomy dependent. As previously reported, this procedure permitted tracheostomy removal once distraction was complete. Now that the patient is skeletally mature, the long-term results of this intervention are reported with regard to his clinical outcome and an assessment of the anatomical changes in the upper airway during growth. Although the distraction could be considered a success in that it enabled permanent decannulation and improved the minimum cross-sectional area of the upper airway, there was no further increase in the minimum cross-sectional area of the upper airway during childhood growth. It is significant that the abnormal growth pattern of the mandible, which is characteristic of this syndrome, did not alter from its preoperative pattern once distraction was completed.
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ranking = 30.510047699221
keywords = airway obstruction, airway, obstruction
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9/19. Management of the difficult pediatric airway with Shikani Optical Stylet.

    The airways of children with many craniofacial and mandibulofacial malformations often present unique challenges to the anesthesiologists. We report the application of Shikani Optical Stylet (SOS) in facilitating the tracheal intubation of four children with history of difficult airway management. The SOS combines the benefits of the lightwand and fiberoptic bronchoscope.
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ranking = 6
keywords = airway
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10/19. Fiberoptic tracheal intubation through a classicial laryngeal mask airway under spontaneous ventilation in a child with Treacher Collins syndrome.

    This report cites the usefulness of fiberoptic scope-guided endotracheal tube intubation through a classical laryngeal mask airway (LMA) during spontaneous breathing. Treacher Collins syndrome (TCS) is a condition where airway management is stressful to anesthesiologists. We report a pediatric patient with TCS undergoing cleft palate repair. The patient had a history of sleep apnea syndrome, chronic lung disease, and congenital heart disease. intubation by rigid laryngoscopy was unsuccessful at the first attempt. One month later, under spontaneous ventilation, tracheal intubation was smoothly performed with the use of a fiberoptic scope through an LMA under intravenous anesthesia with propofol. Fiberoptic scope-guided endotracheal intubation through an LMA with the push of another ETT of the same size to curb the back slippage of the endotracheal tube already inserted in the trachea can be easily and safely performed under spontaneous breathing in a pediatric patient with TCS.
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ranking = 6
keywords = airway
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