Functional outcomes in patients with facial dysostosis and severe upper airway obstruction
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