Mandibular Distraction Osteogenesis as a Primary Intervention in Infants With Pierre Robin Sequence

Area of Science:

• Craniofacial Surgery
• Pediatric Surgery
• Genetics

Background:

• Pierre Robin sequence (PRS) is a congenital condition characterized by a triad of facial abnormalities.
• PRS can manifest as syndromic (sPRS) or isolated (iPRS), often leading to severe airway and feeding difficulties.
• Mandibular distraction osteogenesis (MDO) is a surgical intervention to address these functional deficits.

Purpose of the Study:

• To evaluate and compare the outcomes of MDO in patients with syndromic PRS versus isolated PRS.
• To assess the efficacy of MDO in improving airway and feeding parameters in PRS patients.
• To identify potential differences in MDO outcomes between sPRS and iPRS cohorts.

Main Methods:

• Retrospective review of 29 PRS patients treated with MDO between 2015 and 2019.
• Patients were stratified into iPRS or sPRS groups based on genetic testing.
• Airway (tracheostomy avoidance, sleep apnea, oxygen saturation) and feeding (oral feeds, weight gain) outcomes were analyzed.

Main Results:

• No significant differences in pre-MDO characteristics, apnea-hypoxia index, or laryngeal view between iPRS and sPRS groups.
• Overall positive feeding outcome in 83% and positive airway outcome in 86% of patients post-MDO.
• Statistically significant improvements in apnea-hypoxia index and laryngeal view were observed post-MDO, with no significant difference between sPRS and iPRS groups.

Conclusions:

• MDO is an effective treatment for both syndromic and isolated Pierre Robin sequence, improving airway and feeding issues.
• While sPRS patients may present with greater morbidities, MDO can successfully manage their challenging clinical developments.
• MDO offers a potential alternative to traditional surgical interventions for respiratory and feeding problems in PRS patients.