Apert Syndrome Follow-up

Updated: Aug 21, 2018
  • Author: Harold Chen, MD, MS, FAAP, FACMG; Chief Editor: Maria Descartes, MD  more...
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Follow-up

Further Outpatient Care

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  • Carefully monitor postoperative complications.

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Further Inpatient Care

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  • Admit patients with Apert syndrome for surgical intervention.

  • Tracheostomy may be necessary for airway management.

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Transfer

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  • Transfer may be indicated for further diagnostic evaluation and surgical intervention.

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Complications

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  • Potential eye or brain injury

  • Wound infections

  • Leakage of cerebrospinal fluid or meningocele formation

  • Increased intracranial pressure and hydrocephalus

  • Airway obstruction, respiratory insufficiency, and sleep apnea

  • Treatment goals focused on preventing avoidable developmental delays (from raised intracranial pressure and sleep apnea) and reducing operative interventions may potentially improve developmental outcomes. [33]

  • A significant proportion of children have obstructive sleep apnea and may develop supraglottic airway obstruction on induction and emergence from anesthesia, due to the midface anatomical abnormalities associated with Apert syndrome. [34]

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Prognosis

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  • Prognosis largely depends on the age at operation. Craniosynostosis can result in brain compression and mental retardation unless relieved by early craniectomy. Innovations in craniofacial surgery have enabled children with Apert syndrome to achieve their full potential by maximizing their opportunities for intellectual growth, physical competence, and social acceptance; however, early surgical treatment of craniosynostosis may not alter intellectual outcome.

  • Prognosis depends on associated brain malformations. Malformations of the corpus callosum and size of the ventricles appear to play no role in the final intelligence quotient (IQ) score, though malformations of septum pellucidum have a significant effect.

  • Quality of the family environment is another factor involved in intellectual achievement. Only 12.5% of children with Apert syndrome who are institutionalized reach a normal IQ score, compared with 39.3% of children from a healthy family background.

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Patient Education

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