Airway Management of Hunter Syndrome: a case series

Abstract

Type II mucopolysaccharidoses is an X-linked autosomal recessive disease, causing glycosaminoglycan accumulation, leading to repeated surgeries. Airway substrate deposits present a challenge for anesthesiologists. This case series describes airway characteristics and management in pediatric Hunter syndrome. Seven pediatric Hunter syndrome cases undergoing general anesthesia were studied. Preanesthetic assessment revealed obstructive sleep apnea and potential airway difficulties, including limited mouth openings, macroglossia, tonsiloadenoid hypertrophy, and restricted neck mobility. Visualizing the larynx was challenging during surgery, with hypersecretions, narrowed hypopharynx, and thickened vocal cords. Anesthesia plans incorporated sleep non-apnea as the preferred option, considering muscle relaxants if ventilation could be achieved with jaw thrust. Airway management strategies included using laryngeal mask airway (LMA) for difficult mask ventilation, employing C-MAC video laryngoscopy and bougie for first-pass intubation in two cases, and performing fiberoptic bronchoscopy through LMA with bougie-assisted endotracheal intubation in one case. Experienced anesthesiologists should prepare thoroughly for airway management in pediatric Hunter syndrome patients.

Keywords

• airway management
• case report
• hunter syndrome