LAUSR

Learning Objectives: Hunter syndrome (MPS II) is a rare genetic disorder with accumulation of glycosaminoglycan resulting in short stature, hepatosplenomegaly, joint stiffness, cardiac valvular disease and airway involvement including macroglossia, tracheal deformities, laryngomalacia, and obstructive sleep apnea. Methods: A 19-year-old man with attenuated MPS II and known critical airway, but no previous illnesses, presented with respiratory distress secondary to influenza and presumed status asthmaticus. He was treated with maximal asthma therapies which did not provide much benefit. Given his extremely difficult airway and the high likelihood of cardiac arrest with induction, the decision was made to place ECMO sheaths preemptively then fiberoptically intubate him in a controlled setting in the operating room with only local anesthesia. He tolerated the procedures well, however, he continued to have worsening ventilation and hemodynamic instability. Inhaled anesthesia was attempted but unsuccessful so he was cannulated to veno-venous ECMO. He successfully separated from ECMO after 10 days, but required high ventilatory pressures to prevent airway collapse. A tracheostomy attempt was unsuccessful as his cricoid was substernal and unable to be mobilized. Bronchoscopy revealed nearly 95% collapse of the tracheal lumen. After four weeks of no progress, he was transferred to Cincinnati Children’s Hospital where he underwent a partial manubriectomy and tracheostomy. Due to significant collapse of the distal trachea and bronchi, the tracheostomy tube was positioned at the carina and two left bronchial stents were employed. He was weaned from the ventilator, transferred back two months later, and eventually discharged home with intact neurologic function. Results: Our patient had severe airway abnormalities that were not previously clinically significant until this acute illness triggered airway decompensation. Patients with MPS II in respiratory distress should be presumed to have severe airway anomalies until proven otherwise, and many cannot be intubated by conventional means. Earlier interventions including intubation and even ECMO should be anticipated as in our patient, whose care required not only multidisciplinary but multi-institutional coordination.