Background: The prone position presents several concerns for the pediatric anesthesiologist, such as prevention of pressure related injuries, avoidance of undetected line infiltration, proper airway securement to inhibit unanticipated extubation, and limited access to the patient in critical events. However, the possibility of endotracheal tube kinking in pediatric patients is rarely discussed in the multitude of concerns about prone procedures. Here, we present a case report detailing the anesthetic management of a patient that experienced endotracheal tube kinking in the prone position during a posterior fossa mass resection. Our conclusion is that pediatric anesthesiologists must be cognizant of the possibility of endotracheal tube kinking in patients who are undergoing procedures in the prone position with significant neck flexion. We recommend using either an appropriately sized reinforced endotracheal tube or a nasotracheal intubation to decrease the potential of intraoperative tube kinking.

Congenital central hypoventilation syndrome (CCHS) is a rare genetic disorder affecting respiratory control and autonomic nervous system function caused by variants in the paired-like homeobox 2B (PHOX2B) gene. Although most patients are diagnosed in the newborn period, an increasing number of patients are presenting later in childhood, adolescence, and adulthood. Despite hypoxemia and hypercapnia, patients do not manifest clinical features of respiratory distress during sleep and wakefulness. CCHS is a lifelong disorder. Patients require assisted ventilation throughout their life delivered by positive pressure ventilation via tracheostomy, noninvasive positive pressure ventilation, and/or diaphragm pacing. At different ages, patients may prefer to change their modality of assisted ventilation. This requires an individualized and coordinated multidisciplinary approach. Additional clinical features of CCHS that may present at different ages and require periodic evaluations or interventions include Hirschsprung’s disease, gastrointestinal dysmotility, neural crest tumors, cardiac arrhythmias, and neurodevelopmental delays. Despite an established PHOX2B genotype and phenotype correlation, patients have variable and heterogeneous clinical manifestations requiring the formulation of an individualized plan of care based on collaboration between the pulmonologist, otolaryngologist, cardiologist, anesthesiologist, gastroenterologist, sleep medicine physician, geneticist, surgeon, oncologist, and respiratory therapist. A comprehensive multidisciplinary approach may optimize care and improve patient outcomes. With advances in CCHS management strategies, there is prolongation of survival necessitating high-quality multidisciplinary care for adults with CCHS.