Table 6.

Prevention of Secondary Manifestations in Individuals with Apert Syndrome

Manifestation/ConcernPreventionConsiderations/Other
Papilledema / Cognitive impairment Timely surgical treatment of craniosynostosis
Exposure keratopathy & corneal scarring due to ocular proptosis Ocular lubricantsMonitoring of eye surfaces, eye alignment, & vision recommended to prevent & ↓ visual loss
Perioperative respiratory complications 1 Anesthesia eval before any surgical intervention for those w/potential airway challenges may improve communication & outcomes.Risks related to anesthesia are higher in those w/Apert syndrome. 2
Aspiration pneumonia w/subsequent chronic lung disease Aspiration precautions (e.g., thickened feeds, limiting oral intake)Clinical feeding eval &/or video fluoroscopic swallowing study may help determine aspiration risk.
Spinal cord injury & neurologic sequelae
  • Vigilance regarding spine precautions
  • Consultation w/spine surgeon will guide precautions & positioning prior to surgery & anesthesia.
In those w/cervical spine anomalies
1.

Most commonly upper-airway obstruction (6.1%) [Barnett et al 2011]

2.

While upper-airway obstruction is the most common, lower respiratory-tract complications during anesthesia have also been reported [Elwood et al 2001].

From: Apert Syndrome

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