Congenital ankylosis of the temporomandibular joint: resultant upper airway obstruction and cor pulmonale.

Cor pulmonale and severe congestive heart failure secondary to chronic upper airway obstruction developed in a three-year-old girl with congenital ankylosis of the temporomandibular joint complicated by frequent respiratory infections. Nearly absent mouth opening, micrognathia, and mandibular retroposition with resultant glossoptosia obstructed the airway. Medical treatment followed by a tracheostomy and bilateral condylectomy relieved the obstruction allowing normal function of the temporomandibular joint. The clinical, electrocardiographic, radiologic, and hemodynamic findings returned to normal. Congenital ankylosis of the temporomandibular joint has not been previously reported as a cause of cor pulmonale secondary to upper airway obstruction.