Abstract
BackgroundPallister–Hall syndrome is a rare disorder characterized by hypothalamic hamartoma, hypopituitarism, bifid epiglottis, and micrognathia.Case presentationWe describe the airway management under general anesthesia of a 15-year-old female with Pallister–Hall syndrome whose airway was compromised with bifid epiglottis and acquired subglottic stenosis. The three options considered for airway management were tracheal intubation, a supraglottic device, and surgical tracheotomy. Tracheal intubation provides a secured airway, but extubation can be difficult. A supraglottic device minimizes airway injury, but it does not completely protect the airway from aspiration.ConclusionsThe patient’s airway was successfully managed using a supraglottic device with aspiration prophylaxis. Airway management devices should be selected according to each patients’ individual circumstances.
Highlights
Pallister–Hall syndrome is a rare disorder characterized by hypothalamic hamartoma, hypopituitarism, bifid epiglottis, and micrognathia.Case presentation: We describe the airway management under general anesthesia of a 15-year-old female with Pallister–Hall syndrome whose airway was compromised with bifid epiglottis and acquired subglottic stenosis
Airway management in patients with Pallister–Hall syndrome (PHS) is challenging due to craniofacial anomalies such as micrognathia, hard palate malformation, cleft larynx, gingival cysts, bifid epiglottis and uvula, and mandibular hypoplasia
We describe the airway management of a female pediatric patient with PHS, in whom the airway was compromised with bifid epiglottis and acquired subglottic stenosis
Summary
Pallister–Hall syndrome (PHS) is a rare disorder with a wide spectrum of severity that is characterized clinically by hypothalamic hamartoma, hypopituitarism, bifid epiglottis, imperforate anus, and polydactyly [1, 2]. There are numerous reports which describe airway management in patients with subglottic stenosis [6,7,8]. It is challenging for anesthesiologists to manage airways with subglottic stenosis that have other airway abnormalities. The airway abnormalities other than subglottic stenosis make it difficult to manage the airways. We describe the airway management of a female pediatric patient with PHS, in whom the airway was compromised with bifid epiglottis and acquired subglottic stenosis. Pre-anesthetic problems included (1) difficult intubation due to micrognathia and bifid epiglottis, (2) predisposition to aspiration, and (3) airway stenosis due to prolonged intubation during infancy. The subglottic stenosis was the reason that only a small-sized endotracheal tube could be inserted when the patient was 11 years old. The postoperative course was uneventful, and the patient was discharged the day after the procedure
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