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.
MeSH terms
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Airway Obstruction / diagnostic imaging
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Airway Obstruction / surgery*
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Atlanto-Axial Joint / diagnostic imaging
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Atlanto-Axial Joint / injuries*
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Humans
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Infant, Newborn
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Joint Instability / diagnostic imaging
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Joint Instability / etiology*
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Joint Instability / surgery
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Magnetic Resonance Imaging
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Male
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Odontoid Process / diagnostic imaging
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Odontoid Process / injuries*
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Odontoid Process / surgery
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Osteochondrodysplasias / diagnostic imaging
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Osteochondrodysplasias / etiology*
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Osteochondrodysplasias / surgery
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Patient Positioning / adverse effects*
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Pierre Robin Syndrome / complications*
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Pierre Robin Syndrome / diagnostic imaging
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Pierre Robin Syndrome / surgery*
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Plastic Surgery Procedures / adverse effects
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Plastic Surgery Procedures / methods*
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Spinal Cord Compression / diagnostic imaging
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Spinal Cord Compression / etiology
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Spinal Cord Compression / surgery
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Tomography, X-Ray Computed
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Treatment Outcome