The Pierre Robin Mandible is Hypoplastic and Morphologically Abnormal

J Craniofac Surg. 2017 Nov;28(8):1946-1949. doi: 10.1097/SCS.0000000000003974.


Background: For Pierre Robin sequence (PRS) patients, there is incomplete characterization of 3D differences and effects of mandibular distraction osteogenesis (MDO) on the mandible compared to normal controls.

Methods: PRS infants who underwent MDO at 2 craniofacial referral centerals with pre- and postoperative computed tomography (CT) scans were identified. A group of age-matched control patients with CTs were identified in the PACS database. Demographic and perioperative data were recorded. Mandibular lengths, angles, and volumes were measured. Morphologic and outcomes data were analyzed in a case-control comparison.

Results: Sixty-three CT scans were analyzed. Fifteen pre-op PRS patient and 15 control CTs were well matched in terms of age and sex. Mandibular volume (78%), ramus length (87%), and body length (95%) were all decreased in the PRS patients. Anterior symphyseal angle (84%) was significantly reduced in PRS patients while mandibular angle (102%) was maintained. Eighteen post-op PRS patient and 15 control CTs were well matched in terms of age and gender. Mandibular volumes (106%) were normalized following distraction with shorter mandibular rami (88%) and longer mandibular bodies (109%). Postoperatively, mandibular angle (100%) and anterior symphyseal angle (99%) were ultimately indistinguishable from controls.

Conclusions: The mandible in PRS is dysmorphic compared to age-matched controls. Overall, they have a smaller volume, shorter ramus, and an obtuse symphyseal angle. MDO improves mandibular volume and normalizes the symphyseal angle, but results in a longer mandibular body and shorter mandibular ramus.

MeSH terms

  • Airway Obstruction / diagnosis
  • Airway Obstruction / etiology
  • Case-Control Studies
  • Cephalometry / methods
  • Female
  • Humans
  • Infant
  • Male
  • Mandible* / abnormalities
  • Mandible* / diagnostic imaging
  • Mandible* / pathology
  • Mandible* / surgery
  • Micrognathism / diagnosis*
  • Micrognathism / etiology
  • Osteogenesis, Distraction / methods*
  • Outcome and Process Assessment, Health Care
  • Pierre Robin Syndrome* / diagnosis
  • Pierre Robin Syndrome* / surgery
  • Polysomnography / methods
  • Retrospective Studies
  • Tomography, X-Ray Computed / methods