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Pierre robin sequence.

Noopur Gangopadhyay1, Derick A Mendonca, Albert S Woo

  • 1Plastic and Reconstructive Surgery, Washington University School of Medicine, Saint Louis, Missouri.

Seminars in Plastic Surgery
|May 2, 2013
PubMed
Summary
This summary is machine-generated.

Pierre Robin sequence (PRS) involves micrognathia and glossoptosis causing airway obstruction in infants. Conservative positioning resolves obstruction in most cases, but some require further intervention.

Keywords:
Pierre Robin sequenceairway obstructiondistraction osteogenesisglossoptosismicrognathia

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Area of Science:

  • Craniofacial anomalies
  • Pediatric respiratory distress
  • Congenital disorders

Background:

  • Pierre Robin sequence (PRS) is a triad of micrognathia, glossoptosis, and airway obstruction.
  • It often presents with a hypoplastic mandible, leading to posterior tongue displacement and airway compromise.
  • PRS is frequently associated with a U-shaped cleft palate and can occur with syndromic diagnoses.

Purpose of the Study:

  • To review the presentation and management of Pierre Robin sequence.
  • To outline diagnostic and therapeutic strategies for airway and feeding issues in infants with PRS.
  • To discuss common surgical interventions for refractory cases.

Main Methods:

  • Literature review of Pierre Robin sequence.
  • Clinical assessment of anatomical findings and airway obstruction.
  • Discussion of conservative and surgical management options.

Main Results:

  • Conservative positioning resolves airway obstruction in approximately 70% of infants with PRS.
  • Nasopharyngeal tubes and nasogastric feeding tubes are indicated for persistent desaturation or feeding difficulties.
  • Surgical options like tongue-lip adhesion and mandibular distraction osteogenesis are considered for non-responsive cases.

Conclusions:

  • Multidisciplinary evaluation is crucial for infants with PRS to address airway and feeding challenges.
  • Conservative management is effective for the majority of cases.
  • Surgical intervention should be considered after ruling out lower airway obstruction and failure of conservative measures.