Articles: pierre-robin-syndrome-therapy.
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Br J Oral Maxillofac Surg · Dec 2013
Case ReportsManagement of airway and feeding difficulties in a neonate with Pierre Robin sequence: a case report.
Pierre Robin sequence is a congenital disorder that affects neonates, and is characterised by the classic triad of micrognathia, glossoptosis, and cleft of the secondary palate. We present the case of a child with Pierre Robin sequence who had severe respiratory distress and feeding problems, and describe a simple technique for forward traction of the mandible to relieve the respiratory distress and activate the tongue.
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Pierre Robin sequence (PRS) is a congenital anomaly presenting with micrognathia, glossoptosis and a cleft palate. This study describes a decade's experience of the management of upper airway obstruction (UAO) in PRS patients with a nasopharyngeal airway (NPA). ⋯ There is a spectrum of UAO in PRS. This study reports on long-term outcomes in 104 children with PRS and airway obstruction. In most children (86.5%), airway obstruction was managed by conservative measures or with an NPA for a few months. The natural history shows that with normal growth, airway compromise resolves without immediate surgical intervention as advocated by some practitioners. Few PRS children require a tracheostomy.
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Case Reports
Pierre Robin sequence: challenges in the evaluation, management and the role of early distraction osteogenesis.
The challenges for the management of Pierre Robin Sequence (PRS) are the evaluation and management of airway and feeding difficulty from glossoptosis and associated cleft palate. ⋯ Primary management of airway insufficiency in patients with PRS can be managed in a prone position with or without nasopharyngeal airway, prolonged intubation, tongue-lip adhesion, mandibular distraction osteogenesis and tracheostomy. The present study confirmed that proper conservative management can be used to manage most of the patients with PRS. However, early mandibular distraction should be considered when (a) indicated in patients with respiratory insufficiency to avoid tracheostomies or (b) successfully decannulating tracheostomies. Interdisciplinary team management is needed to ensure proper evaluation, improve care and optimum outcome.
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Plast. Reconstr. Surg. · Jul 2011
The GILLS score: part I. Patient selection for tongue-lip adhesion in Robin sequence.
The compromised airway in Robin sequence demands prompt operative intervention. Tongue-lip adhesion is one alternative; however, the outcome of this technique is variable. The purpose of this study was to identify variables that preoperatively predict the success of adhesion in Robin sequence patients with life-threatening respiratory distress. ⋯ Risk, II.(Figure is included in full-text article.).
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The Robin sequence, previously known as the Pierre Robin syndrome, is characterized by the sequence of clinical events that result from a small mandible. The tongue becomes posteriorly displaced (glossoptosis) and obstructs the airway. The obstructing tongue also makes oral feeding difficult and, in severe cases, impossible. ⋯ Ideally, treatment should be individualized. Patients who will have catch-up growth of the mandible will only need a tongue-lip or nasopharyngeal airway as a temporary measure. Patients who we know will not have catch-up growth will benefit from early distraction osteogenesis.