Management of upper airway obstruction in syndromic craniosynostosis: A lifespan approach from childhood to adulthood.

Introduction: Syndromic faciocraniosynostoses are malformations of the skull and face caused by premature closure of one or more cranial sutures, characterized by hypoplasia of the maxilla, leading to upper airway obstruction.

Objective: The aim is to provide a comprehensive review on the management of upper ventilatory disorders in the context of craniosynostosis.

Methods and results: Initial management should look for upper airway obstruction through questioning, clinical examination with flexible laryngoscopy, and, if in doubt, sleep recording to look for obstructive sleep-disordered breathing. Treatment consists of re-establishing airway patency through non-surgical treatments such as non-invasive ventilation and correcting the obstruction through various surgical procedures: fronto-facial advancement, adenoidectomy, turbinoplasty, septoplasty, etc. A tracheotomy may be necessary during early childhood, pending permeabilization of the upper airways. The age at which surgery is performed varies, depending on the individual patient, and it is not uncommon for patients to require surgery in adulthood, either as part of the initial management or for additional surgeries to improve obstruction.

Conclusion: The multidisciplinary approach is the key for managing craniosynostoses, from diagnosis to treatment, from childhood to adulthood.