In Apert syndrome, a staged strategy can be used in the least severe cases or when the environment does not allow for major craniofacial procedures (absence of intensive care unit, unavailability of distractors, etc.…). When a monobloc is indicated due to significant exorbitism and obstructive sleep apnea syndrome (OSAS), we prefer internal distraction at an ideal age of around 30 months. In older patients, an external distractor could be used. To avoid failures and complications, a rigorous and standardized technique is mandatory. Neurosurgical collaboration is mandatory, and it is preferable that the procedure be carried out in a reference center that treats a high volume of syndromic craniofacial patients (French CRANIOST, European ERN CRANIO). Exorbitism correction is always obtained with the monobloc procedure. However, when dealing with OSAS, the improvement obtained by the Monobloc is sometimes insufficient to fully correct the most severe cases. In that case of incomplete improvement, an early Le Fort III will restore the airways to normalize Apnea–Hyponea Index (AHI). In all cases, additive procedures will be necessary at adolescence.

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Monobloc Advancement with Internal Distraction

  • Eric Arnaud,
  • Roman H. Khonsari,
  • Giovanna Paternoster

摘要

In Apert syndrome, a staged strategy can be used in the least severe cases or when the environment does not allow for major craniofacial procedures (absence of intensive care unit, unavailability of distractors, etc.…). When a monobloc is indicated due to significant exorbitism and obstructive sleep apnea syndrome (OSAS), we prefer internal distraction at an ideal age of around 30 months. In older patients, an external distractor could be used. To avoid failures and complications, a rigorous and standardized technique is mandatory. Neurosurgical collaboration is mandatory, and it is preferable that the procedure be carried out in a reference center that treats a high volume of syndromic craniofacial patients (French CRANIOST, European ERN CRANIO). Exorbitism correction is always obtained with the monobloc procedure. However, when dealing with OSAS, the improvement obtained by the Monobloc is sometimes insufficient to fully correct the most severe cases. In that case of incomplete improvement, an early Le Fort III will restore the airways to normalize Apnea–Hyponea Index (AHI). In all cases, additive procedures will be necessary at adolescence.