P036: DIFFICULT AIRWAY: HADJU-CHENEY SYNDROME
Joohi Khan, DO, Gianella Russo, MD; Kendall Regional Medical Center
Introduction: Hadju-Cheney syndrome is an exceedingly rare autosomal dominant congenital disorder which affects connective tissue. It is characterized by excessive bone resorption leading to osteoporosis. Facial abnormalities include a retrognathia, orbital hypertelorism, low-set ears and hypoplastic frontal sinuses, some patients may even present with cleft palate. The prevalence of this disorder is unknown, fewer than 100 affected individuals have been described in literature. Several factors indicate a difficult ventilation and intubation in a patient with this disorder. Positioning of the head to optimally align the pharyngeal and tracheal axes may be difficult with a misshapen head. Facial asymmetry or underdevelopment may make it hard to attain a good seal between the face and a mask. Patients with high arched palates or cleft palates may have a smaller oral cavity making intubation more complex . This stresses the importance of following the difficult airway algorithm.
Case Description: 15 yo male with past medical history of suspected Hadju-Cheney syndrome, and central sleep apnea, past surgical history of tonsillectomy and adenoidectomy and physical exam midface hypoplasia, retrognathia, presented to our hospital after sustaining a fall from his bicycle. Patient was found unresponsive and cyanotic by a passerby. Patient was ventilated by bag mask and transferred to an outside hospital. Patient could be adequately ventilated however proved to be difficult intubation. After multiple attempts to secure the airway at the outside hospital, the airway was secured via blind ETT placement through LMA. Patient was then transferred to our level 1 trauma center. Upon arrival ETT placement was confirmed, secured and LMA removed. Patient was found to have a traumatic brain injury and was admitted to the pediatric intensive care unit. Upon improvement of his injury, a discussion was held between the intensivist and pediatric anesthesiologist to attempt extubation. Although patient met extubation criteria, given the high likelihood of difficulty securing the airway should the patient fail extubation, the decision was made to extubate the patient while securing the airway with a ventilating airway exchange catheter. After thirty minutes, the patient was diaphoretic, tachypneic with accessory muscle use and an arterial blood gas showed respiratory acidosis. Therefore, the patient was reintubated over the exchange catheter while using a glidescope in an attempt to visualize the airway. Patient was then referred to specialized pediatric hospital for ENT evaluation of possible tracheostomy and mandibular surgery.
Discussion: Whenever a difficult airway is anticipated, proper measures should be directly on hand including a glidescope, fiberoptic scope, as well as preparation for the possibility of a surgical airway. Extubation should be proceeded with extreme caution, and appropriate safety measures should be in place, consider the use of an airway exchange catheter which will allow for ventilation and reintubation if needed.