Sections View Full Chapter Figures Tables Videos Annotate Full Chapter Figures Tables Videos Supplementary Content ++ Pierre Robin syndromePierre Robin Malformation SequencePierre Robin complexPierre Robin anomalyPierre Robin triadRobin AnomaladCleft Palate, Micrognathia and Glossoptosis ++ 756.0 Anomalies of skull and face bones1 ++ Q87.0 Congenital malformation syndromes predominantly affecting facial appearance2 ++ 5B Impaired neuromotor development +++ Description ++ Group of congenital malformations usually a triad combinationCleft palate (92%)3MicrognathiaSmall lower jawGlossoptosis (70 to 85%)3 at birthTongue tends to fall back toward the throat +++ Essentials of Diagnosis ++ May have failure to thrive or severe respiratory distress3 +++ General Considerations ++ Presents as emergencies in the newborn period because of airway restrictionMay have clubfeet or hearing loss (60%)3CNS defects (50%)3Developmental delayHypotoniaHydrocephalusInfants with Pierre Robin may have Strickler Syndrome and Velocardiofascial SyndromeCongenital heart disease in 15%4 +++ Demographics ++ Affects males and females equally unless X-linked31 in 8,500 births6 +++ Signs and Symptoms ++ Ear infectionsBreathing problemsCleft soft palateHigh arched palateSmall, retracted lower jawNatal teethLarge tongue5 +++ Functional Implications ++ Frequent ear infections6Difficulty feedingDecreased hearingDelayed speech +++ Possible Contributing Causes ++ UnknownMay be a result of positioning of the fetus in the beginning of pregnancyMay be autosomal recessive3 +++ Differential Diagnosis3 ++ Stickler syndromeTrisomy 11qTrisomy 18Moebius syndromeCHARGE syndrome +++ Laboratory Tests ++ Genetic testingDNA methylation analysis to assess if genetic link +++ Imaging ++ Radiography to assess craniofacial anatomy ++ Organ anomalies +++ Medical Procedures ++ Surgery to repair the cleft palate ++ Admittance or continued hospitalization after birth for surgical repair, tracheostomy, gastrostomy, or tympanostomy tubes3OtolaryngologistGeneticistAudiologistOccupational therapistSpeech therapist ++ Inability to achieve or sustain prone position while on elbowsInability to rollInability to sitNon-ambulatory ++ Bayley Scales of Motor Development or Bayley IIPeabody Scales of Motor DevelopmentMovement Assessment of InfantsNeonatal Behavioral Assessment ScaleEarly Intervention Developmental ProfileTest of Infant Motor PerformanceAlberta Infant Motor Scale ++ Prevent asphyxia until the mandible becomes large enough to accommodate the tongue7Supine positioning contraindicated until jaw grows or until surgical repair5Prone positioning is preferred for infants3, 5Therapeutic exerciseStrengtheningStretchingFunctional activitiesTransfersGait trainingStair climbingNeurodevelopmental treatmentInhibition of spasticityFacilitation of normal movement patternsAcquisition of motor milestones ++ Sustain prone position on ... Your Access profile is currently affiliated with [InstitutionA] and is in the process of switching affiliations to [InstitutionB]. Please select how you would like to proceed. Keep the current affiliation with [InstitutionA] and continue with the Access profile sign in process Switch affiliation to [InstitutionB] and continue with the Access profile sign in process Get Free Access Through Your Institution Learn how to see if your library subscribes to McGraw Hill Medical products. Subscribe: Institutional or Individual Sign In Error: Incorrect UserName or Password Username Error: Please enter User Name Password Error: Please enter Password Sign in Forgot Password? Forgot Username? Sign in via OpenAthens Sign in via Shibboleth You already have access! Please proceed to your institution's subscription. Create a free profile for additional features.