Surgical protocol for pierre robin sequence

摘要

Pierre Robin Sequence (PRS) is described by the triad: micro-retrognathia, glos-soptosis and upper airway obstruction, associated with U/V- shaped cleft palate. PRS can be shown isolated or aggravated by other syndrome. Prenatal diagnosis is possible from 20th weeks of gestation by ultrasonography and MRI, for parents counseling about possible respiratory distress, feeding problems. A multidisciplinary team approach is mandatory for the treatment of these patients. Several options have been provided, but nowadays no international guidelines exist. Our protocol is based on: low dose CT scan to evaluate micrognathia and upper airway narrowing, polisomnography to register any sleep apnea episodes. Parents are taught to feed children with fractionated meal and to place child in prone position to improve airways space. In severe cases we perform mandibular distraction osteogenesis in order to lengthen mandible. Lastly, patients undergo palatoplasty when they are about 9 months.