Prolonged Ventilator Dependency in a Pediatric ICU Patient With Pfeiffer Syndrome Following Le Fort I Osteotomy and Distraction Osteogenesis: A Case Report

Pfeiffer syndrome is a rare autosomal dominant disorder characterized by craniosynostosis and midface hypoplasia, often leading to significant airway challenges and respiratory complications during anesthesia management. This case report describes a four-year-old female with Pfeiffer syndrome who underwent Le Fort I osteotomy with distraction osteogenesis and concurrent ventriculoperitoneal shunt revision. Postoperatively, the patient experienced an extended pediatric ICU (PICU) stay with complex airway management, requiring prolonged mechanical ventilation and sedation. This case highlights the complexities in managing patients with Pfeiffer syndrome undergoing craniofacial surgery and emphasizes the importance of early sedation weaning, sedation windows, use of shorter active sedatives, timely tracheostomy, and multidisciplinary education to improve respiratory outcomes.