Perioperative complications in children with Apert syndrome: a review of 509 anesthetics.

OBJECTIVES: To perform a retrospective, anesthesia case note review in children with Apert Syndrome.

AIM: To identify perioperative complications in this group of patients.

BACKGROUND: Apert syndrome is a rare autosomal dominant disorder characterized by craniosynostosis, craniofacial anomalies, and severe symmetrical syndactyly (cutaneous and bony fusion) of the hands and feet. Children with this syndrome require general anesthetics for a number of different operations and procedures. Our institution has records of 71 children with Apert syndrome. Analysis of their general anesthetic records was undertaken, and the incidence of perioperative complications was investigated.

METHODS: A retrospective case note review was performed on 61 children with Apert syndrome over a 14-year period. There were a total of 509 general anesthetics administered to these children during this period of time.

RESULTS: There were a total of 31 perioperative respiratory complications occurring in 21 patients (6.1% of the total cases). Twenty-three of these complications were supraglottic airway obstruction (4.5% of total cases).

CONCLUSIONS: We found there to be a low incidence of major perioperative major complications in this group of patients. Nevertheless, a significant proportion of these children have obstructive sleep apnoea and may develop supraglottic airway obstruction on induction and emergence from anesthesia due to the associated mid-face anatomical abnormalities.