Anesthesia for children with complete trisomy 18 (Edwards syndrome): A cohort review of 84 anesthesia encounters in nine patients.

Background: Trisomy 18 or Edwards syndrome is the second most common aneuploidy with a prevalence between 1/3000 and 1/10 000 live births. The syndrome encompasses malformations of the central nervous, cardiac, respiratory, gastrointestinal, and genitourinary systems. Trisomy 18 carries a poor prognosis with 90% of patients not surviving beyond 1 year of age; however, the current trend toward more aggressive supportive care may prolong survival. The limited anesthesia literature highlights the abnormal airway anatomy but generally describes uneventful airway management and perioperative course.

Aim: Our goal was to review all anesthesia encounters recorded for eleven trisomy 18 patients treated at Children's Wisconsin during the study period to explore the frequency of anesthesia encounters and to improve our understanding of the perioperative risks.

Methods: We performed a retrospective chart review of all patients with trisomy 18 who were treated at our institution between 2012 and 2017. Records were screened for anesthesia encounters, perioperative critical events and complications, enrollment in palliative care, code status, and time of death.

Results: Eleven children were identified. Children were born between 2001 and 2016. Two children never required anesthesia care. Nine patients had a total of 84 anesthesia encounters for 121 surgical or diagnostic procedures or emergent intubations. Critical events and perioperative complications included difficult mask ventilation (n = 7), difficult intubation (n = 15), and mechanical or pharmacological cardiopulmonary resuscitation (n = 6). Five patients presented with difficult peripheral intravenous access. One patient died in the immediate postoperative period. On five occasions, patients required emergent intubation outside of the operating room.

Conclusion: Difficult airway management and respiratory compromise were critical concerns during the perioperative period in our patient population, and the inability to ventilate could lead to cardiorespiratory arrest. This case series provides a comprehensive, longitudinal view of complete trisomy 18 patients in the perioperative period and adds information for counseling families and care providers.