Overcorrected Midface Advancement to Improve Airway Problems in Severe Pfeiffer Syndrome Types II and III.

ABSTRACT

INTRODUCTION: Treatment of patients with severe Pfeiffer syndrome types II and III is difficult. The purpose of this article is to present our method of overcorrecting midface advancement to improve airway problems in such patients. MATERIALS AND METHODS: One boy and two girls with types II and III Pfeiffer syndrome and who underwent Le Fort III midface advancement using our previously described corrected cephalometric analysis and distraction system were included in the study. RESULTS: The authors overcorrected by advancing the midface to make it look as similar as possible to an adult face. While the overcorrected midface advancement widened the upper airway spaces in the 3 patients, the tracheostomy that had already been placed during infancy could not be closed, probably because of an underlying tracheal abnormality or tracheomalacia. DISCUSSION: Overcorrected midface advancement cannot enable tracheostomy closure, probably because of severe tracheal anomalies, such as tracheomalacia, below the tracheostomy. However, with the possibility of gradual improvement of the tracheomalacia with age, closure of the tracheostomy can eventually be expected. Therefore, efforts to close a tracheostomy should be pursued even if the probability of its removal is low. CONCLUSION: Overcorrected midface advancement did not enable tracheostomy closure, probably because of severe tracheal anomalies such as tracheomalacia. However, the severe exophthalmos and angle III malocclusion were improved, and with the possibility of gradual improvement of the tracheomalacia with age, closure of the tracheostomy can eventually be expected. Therefore, efforts to close a tracheostomy should be pursued even if the probability of its removal is low.