Dental caries history in nine children with chromosome 18p deletion syndrome.

Chromosome 18p deletion syndrome is caused by the deletion of a portion of genetic material on the short (p) arm of chromosome 18. Many of 100 prior case reports in the medical literature describing the dental health of subjects with this syndrome reported multiple caries associated with the syndrome. At the third annual international conference of The Chromosome 18 Registry & Research Society, dental examinations were carried out on nine children with chromosome 18p deletion syndrome and five of their unaffected siblings. The dental examination included an intra-oral evaluation of coronal decay and filled permanent teeth surfaces (DFS) and decayed and filled primary tooth surfaces (dfs) using a mouth mirror, explorer, and a high-intensity fiber optic light. An evaluation of the data revealed that five of nine children with 18p deletion syndrome (56%) were free of tooth decay or a history of tooth decay. Four of the nine (44%) had tooth decay or a history of tooth decay. The prevalence of decay was quite similar in the genetically unaffected siblings. Three of the five (60%) unaffected siblings of the children with 18p were free of tooth decay, whereas two of the five (40%) had tooth decay. One of the affected children had a missing mandibular left central incisor. None of the children had abnormally shaped teeth. The caries pattern seems to be similar to that reported in the NHANES III data collected in the United States from 1988-1991. Analysis of these preliminary data suggests that the risk for caries in chromosome 18p deletion syndrome may be lower than previously reported.