Role of preoperative multidetector computed tomography airway reconstruction in anaesthetic management of mucopolysaccharidosis type IV

Abstract

Mucopolysaccharidoses (MPS) are a group of lysosomal storage disorders that often present with a difficult airway. The trachea is usually narrowed and flattened, making the choice of correct technique and endotracheal tube (ETT) size crucial. Multidetector computed tomography (MDCT) images have been used to assess the airway of children who are considered at risk for difficult intubation. However, it has not been standardised in preoperative assessment of MPS. We report a case in whom after failed fibreoptic intubation, titrated doses of injection propofol were given and direct laryngoscopy was performed. A 6.0 mm internal diameter (ID) and later a 5.0 mm ID ETT were tried but could not be negotiated. Subsequently, the airway was secured with a 4.5 mm ID ETT. Postoperatively, MDCT airway reconstruction demonstrated that the narrowest part of airway measured 4.3 mm. Anaesthesiologists often face a multitude of challenges in these patients. Our experience gained in retrospect suggests that a detailed preoperative evaluation including MDCT airway reconstruction, followed by meticulous planning, is essential for a safe outcome.