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Cleft Palate Craniofac J. 2018 May;55(5):773-777. doi: 10.1177/1055665618758102. Epub 2018 Feb 28.

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

Author information

1
1 Division of Pediatric Plastic Surgery, University of Pittsburgh School of Medicine, Pennsylvania, PA, USA.
2
2 Division of Craniofacial Medicine, University of Washington School of Medicine, Washington, DC, USA.
3
3 Division of Medical Genetics, University of Pittsburgh School of Medicine, Pennsylvania, PA, USA.
4
4 Division of Pediatric Otolaryngology, University of Pittsburgh School of Medicine, Pennsylvania, PA, USA.
5
5 Division of Pediatric Neurological Surgery, University of Pittsburgh School of Medicine, Pennsylvania, PA, USA.

Abstract

Pierre Robin Sequence (PRS) can be associated with skeletal dysplasias, presenting with craniocervical instability and devastating spinal injury if unrecognized. The authors present the case of an infant with PRS and a type II collagenopathy who underwent multiple airway-securing procedures requiring spinal manipulation before craniocervical instability was identified. This resulted in severe cervical cord compression due to odontoid fracture and occipitoatlantoaxial instability. This case highlights the importance of early cervical spine imaging and cautious manipulation in infants with PRS and suspected skeletal dysplasia.

KEYWORDS:

Pierre Robin sequence; craniocervical instability; odontoid fracture; skeletal dysplasia; spinal cord compression; type II collagenopathy

PMID:
29489401
DOI:
10.1177/1055665618758102
[Indexed for MEDLINE]

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