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ITALIAN JOURNAL OF MAXILLOFACIAL SURGERY
A Journal on Maxillofacial Surgery
Official Journal of the Italian Society of Maxillofacial Surgery
Rivista Italiana di Chirurgia Maxillo-Facciale 2002 April;13(1):45-9
Mandibular distraction osteogenesis using external devices in the Pierre Robin sequence. Report of a case and review of the literature
Bertolini F., Brevi B., Baj A., Bianchi B., Sesenna E.
Chair and Division of Maxillo-Facial Surgery, General Hospital, Parma
The Pierre Robin Sequence (PRS) is an anomaly consisting of micrognathia with glossoptosis, respiratory obstruction, and consequent feeding difficulties. It can be present as an isolated anomaly or may represent part of a syndrome. Patients with PRS may have respiratory obstruction shortly after birth or during the following weeks. These symptoms gradually subside as mandibular growth and improved control of the tongue musculature develop. The goal of treatment is to avoid permanent tracheostomy and gavage feeding. Many conservative and surgical therapeutic modalities have been proposed. Mild cases are often managed conservatively by placing the little patient in an adapted bed, holding the head in true prone position and/or using gavage feeding when oral feeding is not tolerated. However, when severe respiratory distress ensues despite positional treatment, surgical intervention to relieve the obstruction is mandatory. The technique of distraction in adult patients has been modified in infants with upper airway obstruction and obstructive sleep apnea, such as in the PRS, to permit an immediate sagittal advancement of the mandible and the tongue. We present the case of a 3-month-old patient affected by PRS who underwent external mandibular distraction osteogenesis to treat the upper airway obstruction with a good functional outcome.