Surgical Management Options for Infants with Pierre Robin Sequence: Establishing a Standard

There is no gold standard for the surgical management of Pierre Robin sequence (PRS). This review aimed to comprehensively evaluate the most recent data regarding airway and feeding outcomes for infants with PRS undergoing MDO or TLA.

A literature review in April 2023 searched PubMed, Scopus, and Cochrane for English articles published since 2018 on MDO and/or TLA efficacy in infants with PRS. Studies with <10 participants, non-primary procedures, <1 year follow-up, and irrelevant outcomes were excluded. Primary outcomes included polysomnography metrics (AHI, O 2 nadir) and achievement of full oral feeds. Secondary outcomes included tracheostomy avoidance, growth/weight assessments, reoperations, gastrostomy avoidance, and complications.

A total of 22 studies were analyzed. All MDO cohorts demonstrated significant improvement in AHI and O 2 nadir following surgery, whereas none of the TLA cohorts exhibited significant differences. Multiple MDO studies showed 100% achievement of oral feeds 1 month after surgery, while the latest TLA cohort study noted 42.5% achievement at 9 months. There were no differences in growth/weight assessments between MDO and TLA patients. Both MDO and TLA groups exhibited >95% tracheostomy avoidance. Reoperation and gastrostomy rates were markedly lower following MDO.

With routinely documented polysomnography data, MDO consistently exhibited superior resolution of upper airway obstruction compared with TLA; moreover, the markedly lower rate of reoperations suggests that the airway resolution provided by MDO is more stably maintained. In addition, our data reinforces previous notions suggesting MDO superiority in the achievement of full oral feeds as well as in gastrostomy avoidance.