A case of Costello syndrome with delayed eruption of primary teeth

Costello syndrome is very rare and a multiple malformation syndrome that includes characteristic craniofacial features, skin abnormality, severe failure to thrive, congenital cardiac abnormalities including hypertrophic cardiomyopathy, predisposition of papillomas and malignant tumors, and neurologic abnormalities. We report on the oral manifestation of a 27-month-old Japanese boy with this syndrome associated with delayed eruption of primary teeth. The oral characteristics of our patient included thick lips, high-arched palate, and macroglossia, but abnormal tooth morphology and missing were not observed. The data on the mesiodistal diameter of the primary teeth and the dental arch were similar to the mean values of Japanese infants aged 3–4 years old, except that the mesiodistal diameter of the lower primary canines and distance between the buccal cusp tips of the primary first molars of the mandible was smaller.