Bader Syndrome

At a glance

Presence of congenital tumors in the esophagus or mediastinum.

Synonym

Odontoma Dysphagia Syndrome.

Incidence

While the occurrence of multiple odontomas has been reported multiple times, the association reported here with other findings makes this an own entity. Fewer than 10 cases have been described.

Genetic inheritance

Autosomal dominant inheritance was suspected in five cases, while at least one case seemed to be sporadic.

Clinical aspects

Odontoma is an undifferentiated hamartoma arising from tooth germ or surrounding tissue. Some reported cases had multiple maxillary and mandibular odontomas (odontomatosis) associated with severe esophageal stenosis and dysphagia. The child reported by Bader died at the age of 6 years after surgery for esophageal stenosis caused by leiomyomatosis of the muscularis propria. Other reported findings in some of these patients included hyperplasia of the esophageal myenteric plexus, stenosis of the intrathoracic descending aorta with calcifications, chronic interstitial myocarditis, chronic bronchitis with bilateral bronchiectases, chronic “cystopyelonephritis,” “splenitis,” and hepatic sclerosis. Another report described a father and three children (two sons and one daughter) with multiple odontomas that were present at birth or became apparent in infancy. One child died of pneumonia at the age of 1 week. The father and one son also had severe dysphagia from stenosis and dysmotility of the inferior part of the esophagus. A hepatopathy of unknown origin, pulmonary and aortic stenosis, recurrent aspiration pneumonias, and an increased susceptibility to infections (recurrent skin abscesses) appeared to be additional possible components of this disorder. The odontomas in this disorder seem to have a high recurrence rate, as some of the patients required multiple surgeries to have them removed. Odontoma is a hamartoma and the most common benign odontogenic tumor, composed of odontogenic epithelium and ectomesenchyme with calcifications. Two types can be distinguished patho-anatomically: the compound and the complex odontoma, with the former being approximately twice as common. While compound odontomas consist of tooth-like structures typically arranged in an orderly configuration imitating rudimentary teeth, complex odontomas lack tooth-like structures and consist of a disorganized pattern of dentin, enamel, enamel matrix, pulp, follicular tissue, and odontogenic epithelium. Mixed forms have also been described.

Precautions before anesthesia

Possible cardiac, renal, and hepatic involvement should be assessed preoperatively. Chronic aspirations may compromise pulmonary function. Assess for difficult airway management.

Anesthetic considerations

Depending on the size and location of the odontomas, mask ventilation and intubation may be very difficult. Maintenance of spontaneous ventilation is recommended until the airway has been secured. Alternative airway management options should be immediately available (eg, supraglottic airway device, fiberoptic bronchoscope), and adherence to an established difficult airway algorithm is advised. A surgeon familiar with surgical airway management and the necessary equipment should easily ...