Anesthetic management for bronchoscopic intervention in a patient with mucopolysaccharidosis and short stature complicated by mediastinal mass-induced airway stenosis: a case report

Abstract

Mucopolysaccharidosis (MPS) is a group of inherited lysosomal storage disorders associated with difficult airway management. Critical tracheal compression from an anterior mediastinal mass presents an additional challenge during interventional bronchoscopy, when the airway is shared by the ventilation and procedural devices. A 33-year-old woman with MPS (height, 100 cm; weight, 21.5 kg) and progressive dyspnea had a 50 × 22 mm anterior mediastinal mass and severe tracheal stenosis, with minimum luminal dimensions of approximately 13 × 1 mm. Bronchoscopic intervention was performed under general anesthesia using a laryngeal mask airway (LMA) and jet ventilation. Desaturation during normal-frequency jet ventilation (NFJV) resolved after superimposed high-frequency jet ventilation (SHFJV) was initiated. A 14-mm covered tracheal stent was removed because it was disproportionate to the patient’s airway, and balloon dilatation reduced the residual stenosis to approximately 70%. The patient was discharged on postoperative day 3 with symptomatic improvement. In this patient with MPS and critical tracheal stenosis, an LMA-based shared-airway technique with immediate access to SHFJV was used successfully during bronchoscopic recanalization.