Perioperative airway management in mucopolysaccharidosis type II: a case report

Introduction Mucopolysaccharidosis type II (MPS II) is a lysosomal storage disorder associated with the accumulation of glycosaminoglycans in the body’s connective tissues. Head and neck structures involvement is nearly always present and is with progressive course up to life-threatening airway problems. ENT examination is considered essential for the determination of the course of the disease and in the preoperative setting.

Case report Reported is a case of 18 y/o male patient with MPS type II. The patient presented in our institution due to severe dysphagia. A decision for percutaneous endoscopic gastrostomy (PEG) under general anaesthesia was taken. The preoperative airway examination included flexible nasolaryngoscopy and CT imaging of the upper and lower airway tract. Single-slice dynamic CT was used to determine the tracheal lumen on multiple levels. Several airway management strategies were discussed and the multidisciplinary team was prepared accordingly. The first-choice option for ventilation was a laryngeal mask (LMA).

Results The LMA provided optimal respiration. The surgery and the immediate postoperative period were uneventful. The patient was monitored overnight in the ICU. On day 3 after the surgery, the recovery was complicated with pneumonia. On the 7th postoperative day, the patient was discharged.

Conclusion General anaesthesia in a patient with Hunter syndrome is a high-risk procedure and requires extreme caution and an interdisciplinary approach. Preoperative ENT evaluation and strategies for airway management both pre- and postoperatively are of leading importance.

Publication History

Article published online:
10 June 2020