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Outcomes analysis of neonates treated for Pierre Robin Sequence: An 18-year experience.

Runyan CM, Tork S, Chen W, Gordon CB, and BS Pan
University of Cincinnati
2014-03-15

Presenter: Chris Runyan

Affidavit:
50% of the data collection was performed by Dr. Runyan. The abstract and oral presentation are also the work of Dr. Runyan. Research design and planning are a combination of Dr. Pan and Runyan

Director Name: W. John Kitzmiller

Author Category: Resident Plastic Surgery
Presentation Category: Clinical
Abstract Category: Craniomaxillofacial

Background: Pierre Robin Sequence (PRS) is caused by micrognathia, which leads to glossoptosis and airway obstruction. Treatment for PRS includes conservative measures, bypass of the obstruction, airway repositioning and correction of the anatomic deficiency. This study presents treatment outcomes of the largest series of neonates with PRS.

Methods: An IRB approved, 18-year retrospective review of all neonates treated with PRS was performed. Examined variables included demographics, syndromic and neurologic status, feeding outcomes, and polysomnography and microlaryngoscopy data.

Results: 140 neonates with PRS were divided into three cohorts based upon initial treatment: conservative management (n=22), external mandibular distraction (MD, n=68) and tracheostomy (n=50). There were no significant differences in rates of prematurity (24%) or cleft palate (70%) between the groups. Those receiving tracheostomy were more likely to have neurologic impairment (OR=2.85, p=0.02), be syndromic (OR=2.62, p=0.01), have GE reflux (OR=2.4, p=0.02) and require intervention within 5 days of birth (OR=60.65, p=0.005) compared with the MD group. Of patients receiving MD, 8.8% (6/68) failed treatment requiring subsequent tracheostomy. Four factors were associated with failure of MD: low birth weight, syndromic status, neurologic impairment and poor post-intervention polysomnogram. These parameters may be used to identify those at risk for MD failure with high sensitivity and specificity. The presence of multilevel obstruction on microlaryngoscopy (23.7% of MD group) wasn't associated with higher failure rates.

Conclusions: Mandibular distraction is an efficacious treatment modality for neonates with micrognathia and tongue based airway obstruction, and should be considered the first line intervention to avoid tracheostomy.

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