Mandibular Distraction Osteogenesis for the Treatment of Pierre Robin Sequence: The University of Pittsburgh Experience
Zoe M. MacIsaac, MD, Irene Ma, MD, Ian Chow, MD, Wendy Chen, MD, Joseph E. Losee, MD, Jesse Goldstein, MD.
UPMC, Pittsburgh, PA, USA.
BACKGROUND: Multiple approaches exist to managing patients with Pierre Robin Sequence (PRS). Mandibular distraction osteogenesis (MDO) has become a widely accept4d treatment modality. Protocols for MDO differ between institutions, with few reports of long-term follow. Here we report experience and outcomes of patients with PRS managed with mandibular distraction osteogenesis (MDO). METHODS: A retrospective cohort study of symptomatic PRS patients treated with MDO between 2011-2018 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, as well as patient comorbidities and treatment details. GILLS analysis (gastroesophageal reflux disease, intubation preoperatively, late operation, low birth weight, and syndromic diagnosis) was performed to assess the severity of airway obstruction. RESULTS: Between 2011-2018, 65 patients were managed for PRS with MDO. Patients with airway distress underwent evaluation by plastic surgery, otolaryngology, speech pathology, and pulmonology, within the first 1-2 days of hospitalization. Workup included flexible endoscopy, diagnostic laryngobronchoscopy, and polysomnography. Average gestational age was 37.8 weeks. 4 patients were intubated at birth. Average GILLS Score was 2.3. Patients underwent MDO at an average of 44 days of age [range 11-395], averaging 17.6 days into their hospitalization, and were distracted an average of 16.7 mm [range 12-20.] Most were extubated in the operating room, with a trend towards extubation in the intensive care unit. Three patients were taking oral nutrition preoperatively, and all but three patients were taking oral nutrition at most recent follow up. Patients were discharged on average of 31.7 days following MDO.All but two patients were on room air at most recent follow up. Degree of advancement was positively associated with greater postoperative oxygen saturation (R 0.543, p<0.05.) The two patients receiving supplement oxygenation at follow up carried syndromic diagnosis.Preoperatively, apnea-hypopnea-index (AHI) was 23.7; obstructive-apnea-hypopnea-index (OAHI) was 21.3; average oxygen saturation was 94.5%, and nadir averaged 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. There were no major complications
CONCLUSIONS: 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.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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