A multidisciplinary and individualized airway management in pediatric ROHHAD syndrome: A case report

Rapid-Onset Obesity with Hypothalamic Dysfunction, Hypoventilation, and Autonomic Dysregulation (ROHHAD) syndrome is a rare pediatric disorder that presents significant clinical challenges due to characteristics such as rapid-onset obesity, hypothalamic dysfunction, hypoventilation, and autonomic dysregulation. Respiratory involvement significantly worsens clinical course and frequently requires ventilatory support. We describe the airway management of a nine-year-old boy with ROHHAD syndrome who weighed 170 kg and stood 155 cm tall and was admitted for elective tracheostomy due to inadequate bi-level positive airway pressure (BiPAP) support. Preoperative airway assessment demonstrated adequate mouth opening; however, oxygen saturation dropped precipitously to 14 % within 15–20 s following BiPAP removal, precluding complete oropharyngeal evaluation. Due to anatomical challenges and technical difficulties, the team decided to proceed with fiberoptic bronchoscopy-assisted intubation during mask ventilation. Consequently, rapid intubation using videolaryngoscopy was performed with high-flow apneic nasal oxygenation. The team modified the tracheostomy tube assembly to adapt to the significant soft tissue depth, ensuring that the tube remained stable and minimizing the displacement risk. Multidisciplinary preparation included readiness for emergency front-of-neck access in the event that a person cannot intubate or cannot oxygenate. Intubation and tracheostomy were successfully performed without major complications. This case highlights the need to address both the anatomical and physiological challenges in ROHHAD syndrome. Strategic preoperative planning, rapid adaptation during airway management, and effective multidisciplinary collaboration are pivotal to achieve successful outcomes in this highly vulnerable patient population.