Perioperative Care and Airway Management for a Patient With Sagliker Syndrome

In this report, we present a case of a patient with a history of complex airway anatomy secondary to Sagliker syndrome (SS) who presented with acute exacerbation of chronic respiratory failure. The patient’s difficult airway, complicated medical comorbidities, and poor psychosocial status posed a unique challenge for providing safe care during an emergency. The perioperative anesthesia service (PAS), led by critical care anesthesiologists, coordinated a multidisciplinary airway management plan. The PAS team also assisted this medically complex patient with her decision-making process. A 37-year-old female with SS, which is characterized by irreversible disfiguring of head and neck anatomy secondary to end-stage renal disease (ESRD) and poorly controlled hyperparathyroidism, presented with acute exacerbation of chronic respiratory failure due to hypervolemia. The patient’s respiratory status rapidly deteriorated despite aggressive hemodialysis, requiring transfer to the ICU. Given the challenging anatomy and poor respiratory reserve in this patient, the PAS team helped coordinate a comprehensive airway plan that involved transnasal fiberoptic intubation, and in case of emergency, extracorporeal membrane oxygenation (ECMO) as a bridge to a surgical airway. During the decision-making process, the patient was found to be in psychological distress and had limited insights into her condition. The PAS team helped facilitated multidisciplinary goals-of-care discussions for the patient and her family. Fortunately, the patient’s oxygenation improved with noninvasive oxygen support and aggressive hemodialysis without the need for intubation. She was discharged with outpatient follow-up appointments arranged to discuss long-term management. This is the first reported case of SS in the United States. The early involvement by the PAS team helped coordinate a multidisciplinary care plan for this patient with a difficult airway and complex comorbidities. This report highlights an innovative airway algorithm for a potentially “cannot-intubate, cannot ventilate" complex airway, and the PAS team’s role in providing support for the patient’s physical and psychological needs, suggesting that a comprehensive perioperative service can improve the quality and safety of care, not only for surgical patients but also for medically complex patients as well.