Absent pedicles in campomelic dysplasia

作者：McDowell Michael M; Dede Ozgur; Bosch Patrick; Tyler Kabara Elizabeth C^*

来源：Child's Nervous System, 2017, 33(6): 987-992.

DOI：10.1007/s00381-017-3375-4

• Skeletal dyplasia
• Dysplastic
• Pediatric
• Kyphoscoliosis
• Campomelic
• Camptomelic

Objectives The objective of the present study is to report a case of campomelic dysplasia illustrating the absence of cervical and thoracic pedicles. This report reiterates the importance of this clinical peculiarity in the setting of spine instrumentation.
Materials and methods A 10-year-old female patient with campomelic dysplasia presented with progressive kyphoscoliosis and signs of neural compromise. Imaging studies confirmed thoracic level stenosis and demonstrated absence of multiple pedicles in cervical and thoracic spine. The patient underwent decompression and instrumentation/fusion for her spinal deformity.
Results The patient was instrumented between C2 and L4 with pedicle screws and sublaminar cables. However, pedicle fixation was not possible for the lower cervical and upper-mid thoracic spine. Also, floating posterior elements precluded the use of laminar fixation in the lower cervical spine. Cervicothoracic lumbosacral orthosis (CTLSO) was used for external immobilization to supplement the tenuous fixation in the cervicothoracic area. The patient improved neurologically with no signs of implant failure at the 2-year follow-up.
Conclusions Absence of pedicles and floating posterior elements present a challenge during spine surgery in campomelic dysplasia. Surgeons should prepare for alternative fixation methods and external immobilization when planning on spinal instrumentation in affected patients.
Level of Evidence: Level IV Case Report

• 出版日期2017-6