Pierre Robin Sequence: An Evidence-Based Treatment Proposal

J Craniofac Surg. 2018 Mar;29(2):332-338. doi: 10.1097/SCS.0000000000004178.

Abstract

Background: The Pierre Robin sequence (PRS) has been defined as the presence of micrognathia, glossoptosis, and respiratory obstruction in the neonatal period. Since its original description, different therapeutic approaches have been proposed obtaining different success rates, but there is no consensus about its management.

Methods: A literature review was conducted in PubMed, Embase, and Cochrane databases, for the period of January,1985 to November, 2016. A number of 23 articles resulting from clinical studies, discussing diagnostic tests or therapeutic approaches, and directly or indirectly comparing diagnostic or treatment modalities were selected and assessed using the GRADE methodology.

Results: After reviewing and analyzing the selected articles, an evidence-based algorithm for diagnosis and integral management of PRS patients was designed.

Conclusion: Based on the anatomical principles and natural evolution of PRS, the clinical scenario must be evaluated thoroughly as a dynamic event to develop a management sequence that minimizes morbidity and mortality and accelerates patients' reinsertion to normal life.

Publication types

  • Review

MeSH terms

  • Airway Obstruction
  • Glossoptosis
  • Humans
  • Micrognathism
  • Pierre Robin Syndrome* / diagnosis
  • Pierre Robin Syndrome* / pathology
  • Pierre Robin Syndrome* / physiopathology
  • Pierre Robin Syndrome* / therapy