Worry About
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Difficult mask, airway, or IV access
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Elevated intracranial pressure, temperature dysregulation, and seizures
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Corneal abrasions (due to exophthalmos)
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PACU and perioperative monitoring for apnea
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Cardiac anomalies (10% of cases)
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Anatomic anomalies (regional/neuraxial anesthesia)
Overview
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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.
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Two major manifestations are bicoronal synostosis and maxillary hypoplasia. High-arched V-shaped palates and cleft palates are common.
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Strabismus, syndactyly, and conductive and neuronal hearing loss are common manifestations.
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Cognitive delay (IQ <70) see in 67% of pts.
Etiology
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Autosomal dominate disorder; however, most cases are sporadic mutations of the FGFR-2 gene.
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Sporadic mutations are associated with paternal age >40 y.
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