Apert Syndrome (Acrocephalosyndactyly Type 1 and 2)

15:1,000,000 live births

Equal M:F ratio

Aspiration

Bronchospasm

Resp depression

Airway obstruction

Difficult mask, airway, or IV access

Elevated intracranial pressure, temperature dysregulation, and seizures

Corneal abrasions (due to exophthalmos)

PACU and perioperative monitoring for apnea

Cardiac anomalies (10% of cases)

Anatomic anomalies (regional/neuraxial anesthesia)

Apert syndrome is a disorder identified by synostoses of the cranium, vertebral bodies, and digits. It is caused by a mutation in the FGFR-2 gene.

Two major manifestations are bicoronal synostosis and maxillary hypoplasia. High-arched V-shaped palates and cleft palates are common.

Strabismus, syndactyly, and conductive and neuronal hearing loss are common manifestations.

Cognitive delay (IQ <70) see in 67% of pts.