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Cervical Spine Injury From Unrecognized Craniocervical Instability in Severe Pierre Robin Sequence Associated With

Xiao Zhu1, Kelly N Evans2, Areeg El-Gharbawy3

  • 11 Division of Pediatric Plastic Surgery, University of Pittsburgh School of Medicine, Pennsylvania, PA, USA.

The Cleft Palate-Craniofacial Journal : Official Publication of the American Cleft Palate-Craniofacial Association
|March 1, 2018
PubMed

Insights

Infants with Pierre Robin Sequence and skeletal dysplasia require early cervical spine imaging. Unrecognized craniocervical instability can lead to severe spinal cord injury, emphasizing cautious manipulation.

Area of Science:

  • Pediatric Orthopedics
  • Genetics
  • Neurology

Background:

  • Pierre Robin Sequence (PRS) is often associated with skeletal dysplasias.
  • Craniocervical instability is a significant risk in these patients.
  • Early identification of spinal issues is crucial for preventing severe injury.

Observation:

  • A case study of an infant with PRS and type II collagenopathy is presented.
  • The infant underwent multiple airway procedures involving spinal manipulation.
  • Craniocervical instability was identified late, after significant manipulation.

Findings:

  • The infant developed severe cervical cord compression.
  • This was due to an odontoid fracture and occipitoatlantoaxial instability.
  • Delayed diagnosis exacerbated the spinal injury.

Implications:

  • Highlights the critical need for early cervical spine imaging in infants with PRS and suspected skeletal dysplasia.
  • Underscores the importance of cautious handling and minimal spinal manipulation in these vulnerable patients.
  • Emphasizes prompt recognition and management of craniocervical instability to prevent devastating neurological outcomes.

Keywords:
Pierre Robin sequencecraniocervical instabilityodontoid fractureskeletal dysplasiaspinal cord compressiontype II collagenopathy

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