Morquio's syndrome. A case report and review of clinical findings

Morquio's syndrome is a genetic disorder caused by the lack of the enzyme responsible for the breakdown of the mucopolysaccharide keratin sulfate. The resulting buildup of keratin sulfate causes a specific pattern of abnormalities, including short stature, spinal deformity, cardiac abnormalities, corneal opacities, deafness and dental abnormalities. The specific dental findings in the patient described here are as follows: the posterior teeth were tapered and had pointed. cusps; the maxillary anterior teeth were spaced and flared, and on radiographs, the enamel was less than 25 percent of normal thickness but of normal radiodensity. Despite these findings, the patient exhibited a low rate of caries. This paper is a review of medical and dental findings in the Morquio's patient and how this may affect dental management.