Neonatal Mandibular Distraction Osteogenesis in Infants With Pierre Robin Sequence

Abstract

Pierre Robin sequence is the constellation of micrognathia, glossoptosis, and tongue-based airway obstruction. When airway obstruction is severe, feeding, growth, and respiratory demise are at risk. Neonatal mandibular distraction osteogenesis is a technique which improves tongue-based airway obstruction and avoids tracheostomy in patients with severe expressions of Pierre Robin sequence. Its efficacy in relieving airway obstruction is well documented, and it has become the surgical intervention of choice at many craniofacial centers. However, this is an uncommon procedure which can be performed within the first weeks of life, offering little space for a learning curve. The success of neonatal distraction and avoidance of complications is highly dependent on proper surgical technique. This report provides a brief overview of the disease, details the technique of the senior surgeon with captioned videos, describes the protocol used at our institution and reports long-term outcomes with a case description.

Polysomnography in the Early Consolidation Phase of Mandibular Distraction as a Tool to Identify Residual Tongue-Based Airway Obstruction and Need for Further Distraction: A Follow-Up Study

“…While several other studies have reported the use of PSG pre- and post-operatively in their management of patients undergoing MDO, few have identified using the study results to affect decisions regarding distraction phase. 13,14,25 Repeat distraction rates have been reported in 6% of cases, while relapse rates are cited as high as 64%. 7,26-28 However, some argue that relapse is an inevitable consequence of operating on the skeletally immature patient who may continue to have asynchronous growth of the mandible and maxilla even after distraction.…”

An Ode to Gillies and Millard: “The Ten Commandments Applied to Self-Inflicted Head and Neck Gunshot Wounds”

“…Though distractors are not typically used for this type of defect rather for congenital conditions such as Pierre-Robin Sequence or hemifacial microsomia, we adapted these techniques to this patient’s condition. 23,25-27 For direct visualization and to maintain intra-oral mucosal integrity we used an extra-oral approach and we applied a linear distraction movement rather than a curve. Once placed, there was a latency period of 5 days and then 0.5 mm of distraction twice daily until approximately 40 mm was reached.…”

Laryngoscopy Grade Improvement and Difficult Airway Resolution in Infants With Robin Sequence Undergoing Mandibular Distraction Osteogenesis: A Multi-Institutional Study

“…For JHH and JH-ACH patients not requiring intubation or tracheostomy prior to surgery, polysomnography was obtained to evaluate the severity of obstructive sleep apnea (OSA; typically obstructive apnea-hypopnea index [AHI] ! 20 for patients proceeding to MDO) and rule out a significant central component (Diep et al, 2020). Bedside flexible laryngoscopy was generally performed prior to surgery.…”

“…The small, posteriorly positioned mandible is inadequate in supporting the soft tissues of the oropharynx and tongue, causing the tongue to fall back into the pharynx and occlude the upper airway (Evans et al, 2011). Mandibular distraction osteogenesis (MDO) has now been adopted as the first-line surgical therapy at many centers and aims to reposition the tongue anteriorly through its attachments to the mandible, effectively increasing the anteroposterior diameter of the infant's airway (Bangiyev et al, 2016;Khansa et al, 2017;Susarla et al, 2018;Bekisz et al, 2020;Diep et al, 2020).…”