Pierre Robin Syndrome: A Review
European Journal of Molecular & Clinical Medicine,
2020, Volume 7, Issue 10, Pages 572-575
AbstractPierre Robin syndrome (PRS) is defined as a triad of small jaw, tongue retraction, and air passage hindrance, wherein infants often exhibit an immature mandible and respiration difficulties at birth. The small mandible drives the tongue backward, leading to PRS. In general, a cleft palate which is broad and U shaped is additionally related with this anomaly. PRS is not a syndrome alone, though instead various disorders, with single anomaly leading to another. Nevertheless, it is related to many other craniofacial abnormalities and may emerge together with a syndromic diagnosis, such as velocardiofacial and Stickler syndromes. Infants with PRS should be evaluated by a multidisciplinary team to assess the anatomic findings, delineate the source of airway obstruction, and address airway and feeding issues. Positioning will resolve the airway obstruction in 70% of cases. In the correct position, most children will also be able to feed normally. If the infant continues to show evidence of desaturation, then placement of a nasopharyngeal tube is indicated. Early feeding via a nasogastric tube may also reduce the amount of energy needed and allow for early weight gain. Prior to considering any surgical procedure, the clinician should first rule out any sources of obstruction below the base of the tongue that would necessitate a tracheostomy.
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