Respiratory arrest in Treacher-Collins syndrome: implications for dental management: case report.

T reacher-Collins syndrome (TCS) or mandibular dysostosis, is a rare autosomal dominant craniofacial malformation that has been described extensively in the scientific literature, 1 with more than 250 cases reported. 2 Most reports to date have focused on the facial morphology of this syndrome: antimongoloid slanting of palpebral fissures, malformed auricles, mandibular and zygomatic bone hypoplasia, receding chin, and others.2 More recent studies1’ 3-5 expressed a particular interest in the morphology of anatomical structures related to the upper airway in these patients that can develop early respiratory problems. 5 In numerous reports of TCS patients with a history of respiratory failure, the phenomenon had been attributed to glossoptosis, or to the "lethality of the gene", rather than to an actual restriction of the entire pharyngeal airway. 1 In their study using videofluoroscopy, Shprinzen et al. 1 disclosed marked narrowing of the airway in 11 patients with TCS. In several of them, the pharynx was less than 1 cm in width, and it was thought that the reduced airway in these patients might help to explain the frequent reports of neonatal death associated with the syndrome. The researchers concluded that pharyngeal hypoplasia is a primary feature of the TCS. Johnson et al. 3 discovered that patients with TCS are at high risk for upper airway obstruction with associated symptoms like sleep apnea due to the micrognathia that causes the tongue to fall posteriorly onto the oropharynx and obstruct the airway. In a recent comprehensive study, Arvystas and Shprinzen 5 gathered the applicable information concerning the lifethreatening upper airway compromise in TCS and related it to the combination of basicranial kyphosis, narrowing of the pharynx, and the severe retrognathism and ramal height defficiency. Dental treatment on posterior teeth in patients with TCS may be extremely difficult since the mandibular defects dictate a very narrow mouth opening. The purpose of this paper is to report two episodes of the dental treatment of a 7 1/2-year-old boy with TCS. One was respiratory arrest during ambulatory treatment, and the other, a difficult intubation at subsequent session under general anesthesia. The events and recommendations for treatment are discussed.