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Anesthetic management of an infant with trisomy 14 mosaicism undergoing tracheostomy placement

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Anesthetic Management of an Infant with Trisomy 14 Undergoing Tracheostomy Placement

Tommy Nguyen, MD; Jana Ghulmiyyah, MD; Evan Salant, MD

 

BACKGROUND

•Trisomy 14 is a rare congenital disorder with only a few hundred reported cases around the world
•It is associated with developmental and craniofacial abnormalities, including growth and psychomotor restriction, broad nose, micrognathia, cleft palate, highly arched palate, short neck, and congenital heart disease
•Almost all reported cases involve mosaicism, as complete trisomy 14 is highly lethal in utero
 
PMH
•Our patient was a 5-month old ex-34-week male weighing 3.7kg, who presented for tracheostomy placement
•Shortly after birth, he had a 3-month stay in the NICU
•Past medical history of mosaic trisomy 14, laryngomalacia, chronic lung disease with baseline SpO2 87%, hypotonia, PFO and failure to thrive requiring a G-tube
 
HPI
•Admitted to PICU for acute respiratory failure 2/2 viral bronchiolitis
•Required nightly CPAP and daytime nasal cannula, supplemented with nebulizers
•Given his dysmorphic facial features, ENT was consulted and performed direct laryngoscopy and rigid bronchoscopy with an anterior commissure laryngoscope
•Findings notable for micrognathia, retrognathia, nasal stenosis, laryngomalacia, and omega-shaped epiglottis with a grade 2-3 view of vocal cords
•Consented for tracheostomy with consideration for future supraglottoplasty
 
INDUCTION
•Pt arrived in OR on room air in no distress with a 24g IV in the L hand
•He was placed on standard ASA monitors, including EKG, BP cuff on the R arm, and pulse oximetry on the L foot, with a backup pulse oximeter on the contralateral foot
•Due to the tenuous airway, the anesthesia and ENT teams jointly agreed on an awake intubation to minimize the risk of respiratory compromise
 
INTUBATION
•After preoxygenation, anesthesia team proceeded with the awake intubation with a size 3.5 oral rae tube using a CMAC and a MAC #0 video laryngoscopy blade, revealing a transient view of the arytenoids and a grade I view of the vocal cords
•Upon attempts to pass the tube through the cords, Pt frequently coughed and subsequently desaturated, and he subsequently improved when the tube was withdrawn
•Because he was unable to tolerate awake intubation, the decision was made to fully induce the patient for optimal conditions using 5mg propofol and 3mg rocuronium
•Patient was intubated successfully by the surgeon via rigid telescope with an anterior commissure laryngoscope
•Placement was confirmed with end-tidal CO2, and the tube was secured at a depth of 11cm.
 
MAINTENANCE
•Surgical anesthesia was maintained with an additional 10mg propofol, 5mcg fentanyl, and 1% sevoflurane
•During placement of the size 3.5 Shiley tracheostomy tube, the anesthesia team withdrew the oral rae tube until slightly above the vocal cords
•The endotracheal tube was subsequently removed after successful placement of the tracheostomy tube
 
CONSIDERATIONS
•Due to the high incidence of craniofacial abnormalities in patients with trisomy 14, the anesthesia and surgical teams must expect to manage associated difficult airways
•Careful history must also be taken to elucidate any congenital cardiac abnormalities
78th Annual PostGraduate Assembly in Anesthesiology (PGA78) 2024 » banner
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