Pierre Robin Sequence: A Critical Evaluation of Feeding Outcomes
Wei-Wei Lee, BS1, Wendy Chen, MD2, Joshua Barnett, BS1, Matthew Ford, MS, CCC-SLP3, Christal Steinbach, CCC-SLP3, John H. Pang, MD2, Joseph E. Losee, MD3, Jesse A. Goldstein, MD3.
1University of Pittsburgh School of Medicine, Pittsburgh, PA, USA, 2University of Pittsburgh Department of Plastic Surgery, Pittsburgh, PA, USA, 3Children's Hospital of Pittsburgh of UPMC, Pittsburgh, PA, USA.
Background: A major contributor to morbidity in Pierre Robin Sequence (PRS) is feeding/swallowing dysfunction. We present a critical evaluation of feeding/swallowing outcomes in a large cohort of PRS patients.
Methods: A retrospective review of all PRS patients at our institution (2010-2016) gathered medical/surgical histories, detailed clinical/radiographic evaluations, speech/swallow studies, and polysomnography data. Data was analyzed using SPSS (significance p<0.05).
Results: Eighty-five of 111 PRS patients (40 female, 45 male) met inclusion criteria. Based on initial feeding/swallowing evaluations, 72% (n=61) patients were found unsafe for oral intake. Mean initial total apnea-hypopnea index (AHI) was significantly higher in patients deemed unsafe, compared to those safe for oral intake (mean AHI 26.06 vs. 17.17, p=0.039). In patients unsafe for oral intake who had pre-operative modified barium swallow studies (MBS), 52% (n=12) demonstrated laryngeal penetration and 50% (n=16) frank aspiration.
Of patients unsafe for PO, 5% (n=3) were treated only with tracheostomy, 26% (n=16) MDO, 2% (n=1) TLA, while 15% (n=9) underwent endoscopic otolaryngological airway procedures, with the remainder undergoing a combination of these treatments. 16% (n=10) avoided any airway-related intervention. The rates of interventions were similar to those of patients initially deemed safe for oral intake: 4% (n=1) tracheostomy, 25% (n=6) MDO, 0% (n=0) TLA, 25% (n=6) endoscopic ENT, 25% (n=6) avoided airway-related surgeries, with the rest requiring a combination of interventions (p=0.641).
Across the intervention groups in patients unsafe for PO, there was no significant difference in incidence of pre-operative aspiration (p=0.856) or laryngeal penetration (p=0.599) on swallow study. Forty-eight percent (n=29) were gastrostomy-tube dependent at some point in their treatment. Post-operatively, 28% (n=17) remained gastrostomy-tube dependent and while 72% (n=36) demonstrated improved feeding status. Swallow studies showed similar rates of aspiration (50% pre-operatively, n=16 vs. 32% post-operatively, n=14, p=0.151) and laryngeal penetration (52% pre-operatively, n=12 vs. 33% post-operatively, n=10, p=0.261) after intervention in patients unsafe for oral intake.
Among patients unsafe for PO, those requiring tracheostomy had the lowest incidence of feeding improvement (0%, p=0.011). All other groups had greater than 75% rates of improvement. Medical history and syndromic status did not correlate with incidence of improvement in any group.
Of all PRS patients meeting inclusion criteria, patients undergoing MDO only (26%, n=22) demonstrated statistically significant improvement between pre- and post-operative AHI (p=0.011) when compared to those requiring endoscopic otolaryngological intervention either alone or in combination with MDO. Importantly, this group demonstrated the lowest incidence for G-tube requirement during any point of their treatment course (18%, n=4, p=0.048) and the highest incidence of feeding improvement (93%, n=13, p=0.044).
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. Patients undergoing MDO had the highest rate of feeding improvement compared to other treatment modalities.
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