Mandibular Distraction Osteogenesis for Pierre Robin Sequence in Early Infancy: A Systematic Review.

BACKGROUND

Pierre Robin Sequence (PRS) is defined by micrognathia, glossoptosis, and upper airway obstruction. Although neonatal management is well established, surgical indications and protocols beyond this period remain variable due to the absence of standardized guidelines. This review evaluates published treatment algorithms for mandibular distraction osteogenesis (MDO), identifies common decision-making criteria, and proposes a unifying algorithm informed by existing literature. We hypothesize that MDO is preferred following failed conservative management of feeding and airway function.

METHODS

A literature search was conducted following PRISMA guidelines. Inclusion criteria required studies to describe treatment algorithms, decision-making criteria, or institutional protocols for MDO. Of 275 studies identified, 23 met inclusion criteria.

RESULTS

Airway management strategies prioritized non-invasive approaches, including prone/lateral positioning, supplemental oxygen, positive airway pressure, and nasopharyngeal airway (NPA) use. Polysomnography (PSG) was utilized in 12 studies to guide decision-making, with an Apnea-Hypopnea Index (AHI) > 20 frequently cited as the threshold for surgical escalation. 16 studies favored MDO over tongue-lip adhesion (TLA) due to its lower risk of long-term tracheostomy and feeding impairment, though three studies preferred TLA, and four did not specify a preferred approach. The earliest reported MDO occurred at 11 days; however, the mean age across studies was 100 days, indicating most interventions occurred in early infancy beyond the neonatal period (<28 days).

CONCLUSIONS

Algorithm-based airway management in PRS emphasizes early PSG assessment, conservative trialing, and MDO as the preferred surgical intervention. Further studies should refine predictive criteria for MDO success and optimize management strategies beyond the neonatal period.