Trichodentoosseous (TDO) syndrome: case report and literature review

Trichodentoosseous (TDO) syndrome is an autosomal dominant condition characterized by dysplastic nails, curly hair, bone sclerosis of the long bones and calvarium, taurodontism, and enamel hypoplasia that occurs with hypomaturation/hypocalcification defects. To date, nine previous case reports of TDO in the dental literature have shown that while taurodontism and enamel defects are consistently present in TDO, the changes in the nails, hair, and bones may be variably expressed and occasionally not noted. Because of this, a few previous cases of TDO syndrome had been diagnosed simply as amelogenesis imperfecta. This case report highlights the typical clinical findings, diagnostic problems, and the clues to diagnosing this interesting condition. Clinical management of TDO is centered on preventing dental abscesses with stainless steel crowns and improving appearance with esthetic restorations.