Abstract
Introduction: Frank ter Haar syndrome (FTHS) is a rare and complex multisystem congenital genetic disorder that leads to craniofacial, cardiac, and skeletal abnormalities. We report the anesthesia and airway management of a child with FTHS who was referred for repair of atrial septal defect (ASD) and ventricular septal defect (VSD). Case Presentation: The patient exhibited craniofacial and skeletal abnormalities, including craniosynostosis, micrognathia, a prominent forehead, hypertelorism, and anteverted nostrils. These features raised the possibility of a difficult airway. Conclusions: For patients with potential difficult airways undergoing elective surgery, the procedure should be postponed until all necessary equipment for managing a difficult airway is available.