Mandibular Distraction Osteogenesis forthe Treatment of Pierre Robin Sequence: The University of Pittsburgh Experience
Zoe M. MacIsaac, MD, Irene Ma, MD, Ian Chow, MD, Adam Henrie, BA, Wendy Chen, MD, Joseph E. Losee, MD, Jesse A. Goldstein, MD.
University of Pittsburgh, Pittsburgh, PA, USA.
PURPOSE: Multiple approaches exist to managing patients with Pierre Robin sequence (PRS), however, protocols for treatment differ between institutions, and the long-term follow up has been reported from few institutions. Here we report experience and objective outcomes of patients with PRS managed with mandibular distraction osteogenesis (MDO).
METHODS: A retrospective cohort study of symptomatic PRS patients treated with MDO was performed using clinical data to determine demographic, operative, and postoperative data, including tracheostomy status, method of nutrition, and polysomnographic data, pre- and post-operatively.
RESULTS: Between 2013 and 2016, 30 patients were operatively managed for PRS with MDO. Following identification of PRS, patients with airway distress underwent evaluation by plastic surgery, otolaryngology, speech pathology, and pulmonology. Patients underwent flexible endoscopy, diagnostic laryngobronchoscopy, and polysomnography. Average gestational age was 37.8 weeks. While average APGAR was 8.1, 3 patients (10%) were intubated at birth. Average GILLS Score was 2.3. Patients underwent MDO at an average of 44 days of age [range 11-395], and were distracted an average of 17.3 mm [range 12-20.] Most patients were extubated in the operating room, and all but two patients (6%) were on room air at most recent follow up. Degree of advancement was positively associated with postoperative oxygen saturation (R 0.543, p<0.03.) The two patients receiving supplement oxygenation at follow up carried syndromic diagnosis and had undergone tracheostomy.
Three patients were taking oral nutrition preoperatively (10% taking oral nutrition), while all but three patients were taking oral nutrition at most recent follow up (90% taking oral nutrition.) Preoperatively, apnea-hypopnea-index (AHI) was 23.7; obstructive-apnea-hypopnea-index (OAHI) was 21.3; average oxygen saturation was 94.5% with a nadir averaging 77.7%. Postoperatively this improved to AHI of 8.6, OAHI of 6.0, average oxygen saturation of 96.1% and nadir of 83.7% (p<0.05 pre- versus postoperatively.) Average follow up was 18 months following MDO.
CONCLUSION: When managed with a rigorous protocol for preoperative workup and selection, management of patients with symptomatic PRS with MDO demonstrates great success in improving airway status. Most patients required no further airway intervention, and transferred to oral feeding. Future studies will include multi-institutional investigation to increase power, and long-term investigation of mandibular growth and morphology for these patients.
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