Pierre Robin Sequence: An Updated Evidence-Based Treatment Proposal

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.