AIR-10
Postoperative airway emergency in a patient with an unknown tracheal cartilaginous sleeve
Bernadette A, Petrie M, Spilka J, Roper B
Naval Medical Center San Diego, San Diego, CA, US
Case Report:
A 12 month old, 10-kg male presented for craniosynostosis repair due to fusion of the metopic, left coronal, and sagittal sutures (trigoncephaly). He was born premature at 28 weeks by cesarean delivery due to twinning and briefly needed intubation after birth. He had an otherwise normal development and had a history of chronic sinusitis and asthma. After induction of general anesthesia, he was successfully intubated after 2 attempts. The operation was uneventful. The patient had an estimated blood loss of 100 mL and received 80 mL in packed red blood cells. Urine output was 40 mL and 440 mL of lactated ringer’s solution was administered. No vasoactive infusions were necessary. After discussion with the surgeon, the decision was made to extubate. The patient was then transported to the PICU monitored, with supplemental oxygen, and breathing spontaneously.
12 hours postoperatively, the on-call anesthesia team was emergently called to the patient’s bedside. The patient acutely developed respiratory distress, hypoxia, and bradycardia which resolved with bag mask ventilation. The decision was made to intubate, but was unsuccessful after multiple attempts with direct laryngoscopy and video laryngoscopy. ENT was called to the bedside and during an attempt at a fiberoptic intubation, the patient again became acutely hypoxic and bradycardic. Bag mask ventilation then became impossible. An emergent tracheostomy was performed at the bedside and the patient recovered. During tracheostomy revision the following day, the patient was found to have a tracheal cartilaginous sleeve. A genetics workup is currently pending.
Discussion:
Tracheal cartilaginous sleeve (TCS) is an airway malformation where a continuous tracheal cartilaginous structure envelops the airway in an O- or C-shaped manner (1). Syndromic causes of craniosynostosis have a strong correlation with TCS in over 150 described syndromes which may not be diagnosed in the patient (2). Importantly, the presence of TCS has been associated with premature death in almost all cases with a mean age of death of 3 years (1). Due to the rigidity of the trachea, airway dispensability is altered leading to turbulent air flow and obstruction (2). These changes are thought to lead to impairment in clearing secretions, increased mucus production, increased cough, and increased number of respiratory infections (3). Early ENT evaluation and elective tracheostomy bypasses the upper airway that can become obstructed leading to decreased airway complications and increased survival in these children (1).
Conclusions:
If a syndromic basis of craniosynostosis is suspected, we recommend such patients should be evaluated by otolaryngology preoperatively for an elective tracheostomy which could confer decreased perioperative morbidity and increased long-term survival to the patient.
References:
(1) Lertsburapa, et.al. Tracheal cartilaginous sleeve in patients with craniosynostosis syndromes: a meta-analysis. Journal of Pediatric Surgery. 2009
(2) Stater, Brian J, et.al. Tracheal cartilaginous sleeve association with syndromic midface hypoplasia. JAMA Otolaryngology-Head & Neck Surgery. 2015
(3) Lin, Sandra Y, et.al. Congenital Tracheal Cartilaginous Sleeve. Laryngoscope. 1995.
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