Previously, severe upper airway obstruction in patients with retrognathia and glossoptosis has been managed with tracheostomy. However, tracheostomy is associated with significant morbidity. In recent years, mandibular distraction has become an alternative management strategy in infants, but these applications have been limited to patient populations with retrognathia and glossoptosis. The authors present 2 unique cases of patients with KAT6B-related gene disorders, who present with a paradox of tongue-based airway obstruction in the absence of retrognathia. In both cases mandibular distraction osteogenesis with an obliquely oriented vector was successfully performed and both children avoided the need for tracheostomy.
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