Prevalence and Management of Laryngomalacia in Patients With Pierre Robin Sequence

Abstract

Objective To characterize the prevalence and presentation of laryngomalacia and efficacy of supraglottoplasty (SGP) in a cohort of patients with Pierre Robin Sequence (PRS). Design Retrospective cohort study. Setting Tertiary-care children's hospital. Patients, Participants Consecutive patients with PRS born between January 2010 and June 2018. Main Outcome Measures Chart review included demographics, comorbid airway obstruction including laryngomalacia, timing of surgical interventions, clinical symptoms, sleep study data, and modified barium swallow study data. 126 patients with PRS were included; 54% had an associated syndrome, 64% had an overt cleft palate, and 22% had a submucous cleft palate. 64/126 were noted to have laryngomalacia (51%). Patients with concurrent PRS and laryngomalacia were significantly more likely to have submucous cleft palate ( P = .005) and present with aspiration with cough ( P = .01) compared to patients with PRS without laryngomalacia. Patients with concurrent laryngomalacia and PRS showed a significant decrease in apnea–hypopnea index (AHI) and obstructive AHI (OAHI) after mandibular distraction, with a median AHI and OAHI improvement of 22.3 ( P = .001) and 19.8 ( P = .002), respectively. Patients who underwent only SGP did not show significant improvement in these parameters ( P = .112 for AHI, P = .064 for OAHI). The prevalence of laryngomalacia in our PRS cohort was 51%. Patients with PRS and laryngomalacia are more likely to present with overt aspiration compared to patients with PRS without laryngomalacia. These data support that laryngomalacia does not appear to be a contraindication to pursuing MDO.