An increased risk of upper airway obstruction (UAO) is seen in up to 95% of patients with facial dysostosis. Secondary to respiratory problems are feeding difficulties and increased nutritional requirements. Little has been described regarding these outcomes in this patient population. Hence, a retrospective cohort study was performed to gather data on functional outcomes. Eighteen patients with facial dysostosis and severe UAO were included. The median follow-up time was 3.42 years. A tracheostomy tube was placed in 13 patients, of whom 10 subsequently underwent mandibular distraction. Three of the five patients without a tracheostomy underwent mandibular distraction as the primary surgical treatment; the remaining two patients were treated conservatively with oxygen supplementation. At presentation, 13 patients had feeding difficulties. Overall malnutrition was present in 16 patients during follow-up. At the end of follow-up, severe UAO was present in 12 patients, feeding difficulties in seven patients, and malnutrition in four patients, while two patients died. In conclusion, patients with facial dysostosis have a high prevalence of severe UAO, feeding difficulties, and malnutrition. Importantly, mandibular distraction has limited success in treating severe UAO in these patients. Close follow-up by a specialized craniofacial team is of paramount importance to manage the long-term consequences.

distraction osteogenesis, enteral nutrition, mandibulofacial dysostosis, nutritional status, obstructive sleep apnoea, tracheostomy, Treacher Collins syndrome,
International Journal of Oral and Maxillofacial Surgery

van der Plas, P.P.J.M. (P. P.J.M.), Yang, S. (S.), Streppel, M, Pullens, B, Versnel, S.L, Koudstaal, M.J, … Joosten, K.F.M. (2020). Functional outcomes in patients with facial dysostosis and severe upper airway obstruction. International Journal of Oral and Maxillofacial Surgery. doi:10.1016/j.ijom.2020.11.018