J Med Assoc Thai 2011; 94 (12):91

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Pierre Robin Sequence: Challenges in the Evaluation, Management and the Role of Early Distraction Osteogenesis
Chowchuen B Mail, Jenwitheesuk K , Chowchuen P , Prathanee B

Background: 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.
Objective: To present the clinical findings, management, outcome and the role of early distraction osteogenesis in patients with PRS.
Material and Method: The medical records were reviewed of patients with PRS seen and managed by the authors at Srinagarind Hospital, Khon Kaen University between 2001 and 2011.
Results: Fifteen patients with PRS were seen and managed. The female-to male ratio was 1.8 to 1 (9 girls, 5 boys). All of the patients presented with a small mandible, retrodisplaced tongue and upper airway difficulty. One patient had cleft lip only and one patient had cleft lip with cleft palate. Patients were primarily from the provinces of Khon Kaen and Mahasarakham. Conservative management was successful in 12 patients while the 3 with tracheostomy required distraction osteogenesis and the tracheostomy was subsequently successfully decanualated. At the last follow-up, most of the patients had proper catch-up and mandibular growth.
Conclusion: 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.

Keywords: Pierre Robin Sequence, Airway and feeding difficulty, Mandibular distraction osteogenesis, Interdisciplinary management


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