Nine of 56 patients with mucopolysaccharidoses (MPS) showed small tracheal diameters on their frontal chest radiographs. Autopsy
of an MPS I-H (Hurler disease) patient demonstrated that the small calibre was secondary to deposition of glycosaminoglycan
(mucopolysaccharide). Autopsies of two patients with other storage diseases, one with geleophysic dysplasia and one with mucolipidosis
II, also exhibited compromise of their airways because of storage material accumulation.
Paper presented at the European Pediatric Radiology Society Meeting in Paris, France, 5 May 1983