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Modified Posterior Vertebral Column Resection for Patients with Thoracolumbar Kyphotic Deformity
Published on: September 16, 2022
Congenital Cervical Spinal Deformities
1Centre Hospitalo-Universitaire Grenoble Alpes, Hôpital Couple-Enfant, Centre Alpin de la Scoliose, Service d'orthopédie Pédiatrique, CS 10217, 38043 Grenoble Cedex 09, France.
Insights
Pediatric cervical spine malformations present surgical challenges due to limited treatment options. Understanding their embryological origins and associated instability is key for effective management and prognosis.
Area of Science:
- Pediatric Orthopedic Surgery
- Developmental Biology
- Embryology
Background:
- Cervical spine malformations pose significant challenges in pediatric orthopedic surgery, often linked to genetic syndromes and impacting spinal function.
- These congenital anomalies arise from disruptions in embryonic somite segmentation during the third week of development, affecting somitogenesis.
- Current classifications for vertebral malformations are general, complicating the characterization and prognosis of cervical spine deformities, including Klippel-Feil syndrome.
Purpose of the Study:
- To highlight the complexities in diagnosing and managing pediatric cervical spine malformations.
- To emphasize the embryological basis of these anomalies and their impact on spinal stability.
- To discuss the role of advanced surgical techniques and the importance of identifying instability.
Main Methods:
- Review of embryological development of the cervical spine and somitogenesis.
- Analysis of classifications for vertebral malformations and their application to cervical anomalies.
- Discussion of surgical advancements, including navigation, robotics, and 3D printed models for rigid cervical spine deformities.
- Evaluation of occipitocervical transitional anomalies and their association with instability.
Main Results:
- Cervical spine malformations have complex embryological origins, affecting spinal statics and function in multiple planes.
- Klippel-Feil syndrome exemplifies continuous or discontinuous vertebral involvement, complicating prognosis.
- Surgical treatment for rigid deformities, while advanced, remains rare; however, technical aids improve safety and accuracy.
- Occipitocervical anomalies require careful assessment for instability, which is a primary surgical concern.
Conclusions:
- Accurate prognosis for cervical spine malformations is challenging due to varied presentations and lack of specific classifications.
- Identifying instability is crucial in cases of cervical spine anomalies and occipitocervical transitional anomalies.
- Advanced surgical technologies enhance the treatment of rigid cervical spine deformities, though indications remain limited.
Abstract:
Malformations of the cervical spine are a challenge in pediatric orthopedic surgery since the treatment options are limited. These congenital anomalies are often syndrome-related and have multiple repercussions on the function and statics of the cervical spine in all three planes. They are related to developmental abnormalities during the somite segmentation that occurs during the third week of embryonic development. Successful somitogenesis requires proper functioning of a clock regulated by complex signaling pathways that guide the steps needed to form the future spine. There is no specific classification for vertebral malformations at the cervical level. To characterize the progressive nature of a malformation, one must use general classifications. In the specific case of Klippel-Feil syndrome, these malformations can affect several vertebral levels in a continuous or discontinuous manner, but also the vertebral body and vertebral arch in a variable way. Thus, establishing a reliable prognosis in the coronal and sagittal planes is a complex undertaking. While technical mastery of certain osteotomy procedures has led to advances in the surgical treatment of rigid deformities of the cervical spine, the indications are still very rare. Nevertheless, the procedure has become safer and more accurate because of technical aids such as surgical navigation, robotics and 3D printed models or patient-specific guides. Occipitocervical transitional anomalies have embryological specificities that can explain the bony malformations seen at this level. However, most are rare, and the main concern is identifying any instability that justifies surgical stabilization. The presence of a cervical spine anomaly should trigger the search for occipitocervical instability and vice-versa.
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