Presence of congenital tumors in the esophagus or
Odontoma Dysphagia Syndrome.
Less than 10 cases have been described.
Autosormal dominant inheritance was suspected in 5 cases, while at least one case seemed
to be sporadic.
Odontoma is an undifferentiated mass (hamartoma)
arising from tooth germ or surrounding tissue. The three reported cases had
multiple odontomas (odontomatosis) associated with severe dysphagia. The
child reported by Bader died at the age of 6 years after esophageal surgery.
Histology revealed leiomyomatosis of the muscularis propria of the stenotic
esophagus. The child also had stenosis of the intrathoracic descendent aorta
with calcifications, chronic interstitial myocarditis, pyelonephritis, and
hepatic sclerosis. Another report describes a father and three children with
multiple odontomas that were present at birth or became apparent in infancy.
One child died of pneumonia at the age of 3 months. The father and one son also had
severe dysphagia from stenosis and dysmotility of the inferior part of the
esophagus. No other changes were reported.
Chronic aspirations may compromise
pulmonary function. Possible cardiac, renal, and hepatic involvement should
be assessed prior to intervention. Depending on the size and location of the
odontomas, mask ventilation and intubation may be difficult.
Bader G: Odontomatosis (multiple odontomas). Oral Surg Oral Med Oral Pathol
Schonberger W: Multiple odontomas (odontomatosis) and dysphagia in father
and son—A syndromic connection? Z Kinderheilkd
Schmidseder R, Hausamen JE. Multiple odontogenic tumors and other anomalies.
An autosomal dominant inherited syndrome. Oral Surg Oral Med Oral Pathol