Bronchoscopic functional assessment of severe distal tracheal disease in Morquio type A syndrome

Morquio type A syndrome (mucopolysaccharidosis type IVA) is associated with progressive tracheal disease which may not be fully characterised by pre-operative imaging alone. We describe an adolescent undergoing posterior cervical fusion in whom pre-operative computed tomography demonstrated severe distal tracheal narrowing. Intra-operative flexible bronchoscopy provided functional assessment, informed tracheal tube exchange, confirmed positioning and guided subsequent airway management. The case illustrates the complementary role of flexible bronchoscopy alongside static imaging in peri-operative airway assessment and the importance of reassessing airway risk at each anaesthetic encounter in patients with progressive airway disease.