Tracheal narrowing in children and adults with mucopolysaccharidosis type IVA: evaluation with computed tomography angiography

ConclusionNarrowing, deviation and abnormal shape of the trachea at the thoracic inlet are common in children and adults with MPS IVA, with a trend toward increased narrowing with advancing age in children. A W- or T-shaped trachea is associated with focal tracheal narrowing. Crowding of the thoracic inlet, due to vascular tortuosity and thyroid position, appears to play a major role.
Source: Pediatric Radiology - Category: Radiology Source Type: research