Prevalence and Management of Laryngomalacia in Patients With Pierre Robin Sequence.

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.