Pierre Robin Sequence: Challenges in the Evaluation, Management and the Role of Early Distraction Osteogenesis
Bowornsilp Chowchuen MD, MBA*, Kamonwan Jenwitheesuk MD*, Prathana Chowchuen MD**, Benjamas Prathanee PhD***
Affiliation : * Division of Plastic Surgery, Department of Surgery, Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand ** Department of Radiology, Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand *** Department of Otorhinolaryngology, Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand
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
