Background and objectives: Pierre Robin Sequence (PRS) is a congenital abnormality characterized by mandibular hypoplasia, glossoptosis and often secondary palate cleft. As a consequence of these physical alterations, patients often presents varying degree of upper airway obstruction and feeding difficulties. Although mortality is now lower than in the past, chronic hypoxia has been reported to place many infants at risk for permanent brain damage. To avoid this, several treatment options are described in literature. Despite this, no international guidelines about the management of PRS currently exist. The aim of this study is to describe a Fast and Early Mandibular OsteoDistraction (FEMOD) protocol used by authors in cases of PRS with severe airway obstruction.
Methods: 46 consecutive patients affected by severe PRS were treated in our department from 2008 to 2012. The evaluation of these patients was conducted by a multidisciplinary team and consisted in complete history and physical examination, low dose computed tomographic scan (CT), polisomnography and O 2 –CO 2 saturation monitoring. Based on this evaluation, only 38 of 46 patients were included in the study. In 29 cases, FEMOD protocol was performed few days after birth (average 11 days) because of severe respiratory crisis at birth and endotracheal intubation was required.
Results: Tracheotomy was avoided in all patients who had not previously. Polisomnography and O 2 –CO 2 saturation performed before and after distraction shown an improvement of AHI index and saturation of central apnoea. Before surgery all patients were fed by gavage and nasogastric tube, but after surgery all infants were able to feed by baby bottle and have gained weight.
Conclusions: Based on our results, rapid mandibular distraction osteogenesis (2 mm/day) immediately after birth (mean 11 days) represents an excellent treatment in PRS infants with micrognathia and severe airway obstruction.