Longitudinal Outcomes of a Multimodal Treatment Approach Including Mandibular Distraction Osteogenesis and Continuous Positive Airway Pressure for Pierre Robin Sequence

BACKGROUND: Newborns with Pierre Robin sequence (PRS) often experience chronic intermittent hypoxemia/hypoventilation associated with airway obstruction. The heterogeneity of the severity of upper airway obstruction makes management a challenge; the optimal intervention in individual cases is not clear.OBJECTIVE: To investigate the prevalence of surgical/nonsurgical interventions for PRS at two children’s hospitals. Patient characteristics and outcomes were examined.METHODS: The present retrospective chart review identified 139 patients with PRS born between 2000 and 2010. Demographic information, mode of airway management, associated anomalies and syndromes, polysomnography results, length of intensive care unit and hospital stay, complications and deaths were extracted.RESULTS: Interventions included prone positioning (alone [61%]), tongue-lip adhesion (45%), nasopharyngeal intubation (28%), continuous positive airway pressure (20%), tracheostomy (19%) and mandibular distraction osteogenesis (5%). Tracheostomies were more prevalent in syndromic patients (P=0.03). Patients who underwent tracheostomy had a lower birth weight (P=0.03) compared with newborns with other interventions. Patients who underwent surgical interventions had longer intensive care unit stays (P<0.001). No intervention was associated with a statistically significant likelihood of requiring a subsequent intervention. Thirty percent of patients underwent polysomnography, with a higher proportion of these using continuous positive airway pressure (n=15) (P<0.01).CONCLUSIONS: In the present descriptive study, patients with syndromic PRS or low birth weight underwent early intervention, which included a tracheostomy. Objective measures of airway obstruction were underutilized. Decision making regarding evaluation and management of upper airway obstruction in this population remains clinician and resource dependent. Reporting data obtained from a large cohort of PRS patients is important to compare experiences and motivate future studies investigating this complex condition.

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Continuous Positive Airway Pressure (CPAP) Therapy for the Treatment of Hypernasality: A Single Case Study

Perspectives on Speech Science and Orofacial Disorders ◽

10.1044/ssod24.2.48 ◽

2014 ◽

Vol 24 (2) ◽

pp. 48-58 ◽

Cited By ~ 3

Author(s):

Lakshmi Kollara ◽

Graham Schenck ◽

Jamie Perry

Keyword(s):

Continuous Positive Airway Pressure ◽

Airway Pressure ◽

Positive Airway Pressure ◽

Single Case ◽

Treatment Method ◽

Post Treatment ◽

Subjective Perception ◽

Cpap Therapy ◽

Long Term Follow Up

Studies have investigated the applications of Continuous Positive Airway Pressure (CPAP) therapy in the treatment of hypernasality due to velopharyngeal dysfunction (VPD; Cahill et al., 2004; Kuehn, 1991; Kuehn, Moon, & Folkins, 1993; Kuehn et al., 2002). The purpose of this study was to examine the effectiveness of CPAP therapy to reduce hypernasality in a female subject, post-traumatic brain injury (TBI) and pharyngeal flap, who presented with signs of VPD including persistent hypernasality. Improvements in mean velopharyngeal orifice size, subjective perception of hypernasality, and overall intelligibility were observed from the baseline to 8-week post-treatment assessment intervals. Additional long-term assessments completed at 2, 3, and 4 months post-treatment indicated decreases in immediate post-treatment improvements. Results from the present study suggest that CPAP is a safe, non-invasive, and relatively conservative treatment method for reduction of hypernasality in selected patients with TBI. More stringent long-term follow up may indicate the need for repeated CPAP treatment to maintain results.

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