Mandibular Distraction in Robin Sequence With Multi-Level Airway Disease: Always Contraindicated?

In patients with Pierre Robin sequence (PRS), understanding the mechanism of airway obstruction and the potential for concurrent airway lesions is important for determining appropriate intervention. When concomitant airway anomalies are present with PRS, mandibular distraction osteogenesis alone is often contraindicated and unsuccessful at alleviating the obstruction, mandating the need for tracheostomy. Herein, the authors present the second-reported case of PRS with concomitant complete tracheal rings and highlight the importance collaborative surgical efforts to avoid tracheostomy. Our patient was born with signs and symptoms of PRS and concomitant complete tracheal rings. At birth, he developed respiratory compromise that was resistant to invasive and noninvasive intervention and was transferred to our care. The severity of his airway compromise necessitated operative intervention. Due to the patient's complex medical condition, avoiding the long-term sequelae of a tracheostomy and performing mandibular distraction was favorable. However, due to his multi-level airway disease, a 2-staged approach was planned: sliding tracheoplasty to release the tracheal rings, followed by mandibular distraction to correct the micrognathia. The patient tolerated both procedures and was successfully distracted and eventually weaned into room air. When managing patients with PRS in the setting of severe respiratory distress, securing the airway and carefully evaluating for concomitant airway anomalies is crucial. Even in the setting of multi-level airway disease where mandibular distraction is typically contraindicated, by directly addressing each anatomic level of airway obstruction, the airway can be safely secured and the resultant consequences of tracheostomy can be avoided. Careful planning with a multidisciplinary team is essential.