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Presence of congenital tumors in the esophagus or mediastinum.

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Odontoma Dysphagia Syndrome.

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Less than 10 cases have been described. Autosormal dominant inheritance was suspected in 5 cases, while at least one case seemed to be sporadic.

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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.

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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 23:770, 1967.  [PubMed: 5229432]
Schonberger W: Multiple odontomas (odontomatosis) and dysphagia in father and son—A syndromic connection? Z Kinderheilkd 117:101, 1974.  [PubMed: 4424740]
Schmidseder R, Hausamen JE. Multiple odontogenic tumors and other anomalies. An autosomal dominant inherited syndrome. Oral Surg Oral Med Oral Pathol 39:249, 1975.  [PubMed: 1054451]

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