Treacher Collins syndrome is a rare craniofacial cleft syndrome, which includes zygomatic, maxillary, and mandibular dysplasia. Intubation is difficult in these patients, which is a major anesthetic consideration given that... [ view full abstract ]
Treacher Collins syndrome is a rare craniofacial cleft syndrome, which includes zygomatic, maxillary, and mandibular dysplasia. Intubation is difficult in these patients, which is a major anesthetic consideration given that many of these children require multiple anesthetics. Potential airway challenges include upper airway obstruction, macroglossia, limited mouth opening, retrognathia, cleft lip and palate, and palatopharyngeal incompetence. Review of literature on airway management describes direct laryngoscopy, laryngeal mask airway (LMA), fiberoptic bronchoscopic intubation, intubating LMA, retrograde intubation and tracheostomy.
Our patient was delivered at an outside hospital to a 42 year old gravida 4 at 31 weeks gestation via emergency cesarean section for non-reassuring fetal heart tones; perinatal course complicated by premature rupture of membranes and prolapsed cord. At time of delivery, patient noted to have severe retrognathia and mid face hypoplasia consistent with Treacher Collins syndrome. Neonate's birth weight was 1608 grams with APGAR scores 2,2,2,4 at 1, 5, 10 and 15 minutes respectively. Resuscitative measures included positive pressure ventilation, 4 rounds of chest compressions, 3 normal saline fluid boluses and 1 bolus of 10% dextrose-water. Greater than 5 attempts at intubation by attending pediatrician and on call anesthesiologist. Minimal improvement with CPAP. Prior to arrival at UC Davis Medical Center, critical care transport nurse orally intubated under direct laryngoscopy; patient was inadvertently extubated when stylet was removed. Transport team secured the airway with size 1 Air-Q LMA. Vital signs on arrival at UC Davis Medical Center significant for SpO2 readings in 70-percentile. After stabilization in NICU, patient went for tracheostomy for a more definitive airway given worsening hypoxia, cyanosis and respiratory acidosis. We continued positive pressure ventilation via Air-Q LMA using NICU ventilator for perioperative airway management to minimize disruption in ventilation. We deferred fiberoptic bronchoscopy because of patient size. Spontaneous ventilation was maintained using short-acting reversible anesthetic agents (fentanyl, midazolam) in case patient developed ventilation difficulties. Surgeons infiltrated lidocaine prior to incision. After successful cannulation with a 3.0 neonatal Shiley tracheostomy, patient color and saturations improved. Post-tracheostomy airway evaluation deferred due to significant airway swelling after removal of LMA. Consideration for airway management in future difficult airway patients may include bedside tracheostomy given risks transporting critical airways.
