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Pierre Robin Sequence: Stratifying Feeding Dysfunction and Variables Affecting Outcome
Lee W, Chen W, Barnett JM, Ford MD, Steinbach CV, Pang JH, Losee JE, Goldstein JA.
University of Pittsburgh Medical Center
2017-02-15
Presenter: Wei-Wei Lee
Affidavit:
75% of the work on this project represents the original work of the presenting author.
Director Name: Vu Nguyen
Author Category: Medical Student
Presentation Category: Clinical
Abstract Category: Craniomaxillofacial
Background: Feeding dysfunction in patients with Pierre Robin Sequence (PRS) can be a significant challenge. We present feeding and swallowing outcomes in our PRS patients.
Methods: A retrospective review of all PRS patients at our institution (2010-2016) gathered medical/surgical histories, detailed clinical/radiographic speech/swallow evaluations, and polysomnography data. Data was analyzed using SPSS (significance p<0.05).
Results: 105 PRS patients (37 female, 43 male) were included. Of the 80 patients who had complete clinical/radiographic evaluations, 24 patients (28%) were safe for oral intake at initial evaluation.
Fifty-six patients were unsafe for PO: 10 underwent tracheostomy (18%), 31 MDO (55%), 4 TLA (7%), 47 endoscopic otolaryngological airway procedures (84%), 3 avoided airway-related surgeries (5%). Pre-operative MBS demonstrated 12 patients (60%) with laryngeal penetration, 16 (55%) with aspiration. Twenty-five (45%) were gastrostomy-tube dependent at some point in their treatment. Post-operatively, 14/56 (25%) remained gastrostomy-tube dependent and 34/55 (62%) demonstrated improved feeding status. Swallow studies showed improved rates of laryngeal penetration (n=10, 37%) and aspiration (n=13, 32%).
Patients requiring otolaryngological airway procedures had the highest incidence of feeding improvement (38%, n=17), however there was no statistically significant difference between intervention groups (p=0.913). Medical history and syndromic status did not correlate with incidence of improvement in any group.
Conclusions: Patients with PRS have a high rate of pre-operative feeding/swallowing dysfunction. Detailed multimodal clinical evaluation is crucial to identify these patients and prevent aspiration. Many patients' feeding and swallowing improved post-operatively, supporting patient-tailored interventions.
