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Pierre Robin Sequence: An Updated Evidence-Based Treatment Proposal.

Oswaldo J Gomez1, Andrea Lopez, Esteban Gutierrez

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Summary

This study updates the diagnosis and management algorithm for Pierre Robin sequence (PRS), a condition affecting newborns. It emphasizes nutritional monitoring and oral feeding, with surgical strategies remaining unchanged.

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Area of Science:

  • Pediatric Medicine
  • Genetics
  • Neonatology

Background:

  • Pierre Robin sequence (PRS) is a congenital condition characterized by micrognathia, glossoptosis, and respiratory obstruction.
  • Early identification of PRS is crucial for reducing infant morbidity and mortality.
  • A prior diagnostic and treatment algorithm for PRS requires updating based on new evidence.

Purpose of the Study:

  • To update the existing diagnostic and treatment algorithm for Pierre Robin sequence (PRS).
  • To incorporate the latest evidence regarding the diagnosis and management of PRS patients.

Main Methods:

  • A comprehensive literature review was performed across PubMed, Embase, and Cochrane databases.
  • The review covered studies published between November 2016 and September 2021.
  • Evidence was evaluated using the GRADE methodology, selecting 38 relevant clinical studies.

Main Results:

  • An updated algorithm for the diagnosis and comprehensive management of PRS was developed.
  • New information was integrated into the algorithm based on recent scientific evidence.
  • The updated algorithm guides the diagnosis and management of patients with PRS.

Conclusions:

  • There is no current consensus on PRS treatment or comparative multicenter studies.
  • Surgical indications for PRS management remain consistent with previous recommendations.
  • Nutritional monitoring and assessment of oral feeding are paramount in all PRS cases.