Odontoma dysphagia syndrome explained

Synonyms:	Odontomatosis-aortae esophagus stenosis syndrome

Odontoma dysphagia syndrome (Bader syndrome) is a rare syndrome (<10 cases reported to date) first described in 1967.^[1] The cause is not known but it is suspected to be genetic in origin.

Presentation

This syndrome is characterized by clustering of teeth (compound odontoma, dysplasia and aplasia of teeth), slight craniofacial abnormalities, and dysphagia. The dysphagia may be due to an over growth of muscle tissue or strictures in the oesophagus. Benign tumors (odontomas) may form at the roots of the teeth.

Cause

The cause is thought to be genetic and it may inherited in an autosomal dominant fashion.^[2] A possible link to chromosome 11 (11q13.3) a region that included fibroblast growth factors 3 and 4 has been reported.^[3]

Notes and References

1. Bader G (1967) Odontomatosis (multiple odontomas). Oral Surg Oral Med OralPathol 23:770
2. Schönberger W (1974) Multiple odontomas (Odontomatosis) and dysphagia in father and son-a syndromic connection? Z Kinderheilkd 117(2):101-108
3. Ziebart T, Draenert FG, Galetzka D, Babaryka G, Schmidseder R, Wagner W, Bartsch O (2012) The original family revisited after 37 years: odontoma-dysphagia syndrome is most likely caused by a microduplication of chromosome 11q13.3, including the FGF3 and FGF4 genes. Clin Oral Investig