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In this systematic review, current evidence for the multidisciplinary treatment of Treacher Collins syndrome is provided, recommendations for treatment are made, and a proposed algorithm for treatment is presented. Although some topics are well supported, others, especially ocular, nasal, speech, feeding, and swallowing problems, lack sufficient evidence. In addition, craniofacial surgical reconstruction lacks a sufficient level of evidence to provide a sound basis for a full treatment protocol. Despite the rarity of the syndrome, more research is needed to compare outcomes of several surgical treatments, especially in orbitozygomatic/maxillary regions.

Objective The aim of this study is to assess prevalence, severity, and duration of respiratory distress following palatoplasty in children with Robin sequence and to evaluate perioperative management. Design Retrospective chart review study. Data were collected for patients who were born between 2009 and 2012 and underwent palatoplasty in the Sophia Children's Hospital-Erasmus Medical Center. Results Of the 75 patients with cleft palate, 30 with Robin sequence and a control group of 45 cleft without Robin sequence underwent palatoplasty. Prior to closure, 26 of 30 patients with Robin sequence had been treated by prone positioning, and four needed additional treatment. The mean age at closure was 12.4 months for patients with Robin sequence and 10.9 months for patients without Robin sequence (P = .05). On the basis of the results of preoperative polysomnography with palatal plate, closure was postponed in two patients with Robin sequence. In the Robin sequence group, eight of the 30 patients developed postoperative respiratory distress within 48 hours and one patient, after 7 days; whereas none within the non-Robin sequence group developed respiratory distress. In all nine cases of Robin sequence the obstructive problems resolved within a few days, with four children requiring a temporary nasopharyngeal tube (NPT). There were no significant differences between preoperative polysomnography results of the nine patients with Robin sequence who developed postoperative respiratory distress compared with those patients with Robin sequence who did not. Conclusion Despite delayed closure compared with children without Robin sequence, 30% of the children with Robin sequence developed respiratory distress following palatoplasty, which resolved within a few days. This study emphasizes the need for close perioperative monitoring of patients with Robin sequence who undergo palatoplasty.

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