Related Experiment Video
Updated: Jan 15, 2026
06:45
Modified Posterior Vertebral Column Resection for Patients with Thoracolumbar Kyphotic Deformity
Published on: September 16, 2022
2.8K
Cervicothoracic Kyphosis and Spinal Cord Compression in Hurler Syndrome
Saral Patel1, Rajul Gupta, Alvin Jones
1Cincinnati Children's Hospital and Medical Center, Cincinnati, OH.
Journal of Pediatric Orthopedics
|October 9, 2025
Summary
Hurler syndrome patients often develop progressive cervicothoracic (C-T) kyphosis and spinal cord compression. Early detection and evaluation of C-T kyphosis are crucial for surgical planning and preventing spinal cord injury in these patients.
Area of Science:
- Pediatric Orthopedics
- Medical Genetics
- Neurology
Background:
- Hurler syndrome frequently presents with spinal abnormalities, including cervical instability and spinal cord compression.
- Cervicothoracic (C-T) kyphosis and associated spinal cord compression are less commonly reported but significant concerns in Hurler syndrome patients.
Purpose of the Study:
- To determine the prevalence of C-T kyphosis and spinal cord compression in Hurler syndrome.
- To evaluate the clinical significance of C-T kyphosis and spinal cord compression through clinical symptoms and neuromonitoring.
Main Methods:
- Retrospective case series of 47 Hurler syndrome patients (2009-2023) with spinal MRIs.
- Radiographic assessment of C-T kyphosis and spinal cord compression (Kang grading); ROC analysis for C-T kyphosis significance.
- Review of intraoperative neuromonitoring (IONM) data (SSEP, TcMEP) during non-spine surgeries.
Main Results:
- Mean C-T kyphosis was 17 degrees, progressing over time.
- C-T kyphosis >20 degrees correlated with spinal cord compression (Kang grade 2).
- Significant IONM changes occurred in 3 patients; one developed paraplegia after surgery without IONM.
Conclusions:
- C-T kyphosis is a progressive finding in Hurler syndrome, often associated with spinal cord compression.
- Evaluating C-T kyphosis is vital for surgical planning and mitigating the risk of spinal cord injury.
- Further research is needed to establish optimal management strategies for C-T kyphosis in Hurler syndrome.
Related Concept Videos
01:16Vertebral Column: Regions and Curvature
5.9K
The vertebral column or spine is a flexible column that supports the head, neck, and body and allows for their movements. It also protects the spinal cord.
Regions of the Vertebral Column
In an adult, the spine is subdivided into five regions: the cervical, the thoracic, the lumbar, the sacral, and the coccygeal region. The spine initially develops as a series of 33 vertebrae; after 20 years of age, the nine bones in the sacral region, five sacral, and four coccygeal bones fuse to form...
Regions of the Vertebral Column
In an adult, the spine is subdivided into five regions: the cervical, the thoracic, the lumbar, the sacral, and the coccygeal region. The spine initially develops as a series of 33 vertebrae; after 20 years of age, the nine bones in the sacral region, five sacral, and four coccygeal bones fuse to form...
5.9K
01:29Cardiomyopathy III: Hypertrophic Cardiomyopathy
401
Hypertrophic cardiomyopathy, or HCM, is an autosomal dominant genetic disorder characterized by asymmetric left ventricular hypertrophy without ventricular dilation. It is more common in men and is typically diagnosed in young, athletic adults.EtiologyHCM is primarily genetic and is caused by mutations in genes encoding sarcomeric proteins. Researchers have identified over 1400 mutations across at least 11 different genes. Among these, the most frequently occurring mutations are found in the...
401
01:21Aneurysm II: Clinical Manifestations and Diagnostic Studies
293
Thoracic, aortic arch and abdominal aneurysms are significant vascular conditions that can present with various clinical manifestations and lead to serious complications. Understanding these manifestations and the appropriate diagnostic studies is essential for effective management and treatment.Thoracic Aortic AneurysmsThoracic aortic aneurysms often remain asymptomatic until they reach a size that impinges on adjacent structures. They typically cause deep, diffuse chest pain that radiates to...
293
01:30General Structure of a Vertebra
6.0K
A typical vertebra, with the exception of the sacrum and coccyx, consists of a body, a vertebral arch, and seven different projections termed processes. The anterior portion of the vertebrae, the body, supports about half the body’s weight. The vertebral bodies progressively increase in size and thickness from the cervical region to the lumbar region of the vertebral column. The intervertebral discs present between the bodies of adjacent vertebrae firmly unites them, forming a continuous...
6.0K
01:20The Thoracic Cage: Ribs
8.0K
Ribs are curved, flattened bones forming the thoracic cavity wall with the thoracic muscles. There are 12 pairs of thoracic ribs. The posterior ends of all the ribs articulate with the T1–T12 thoracic vertebrae. In contrast,the anterior ends of most ribs attach to the sternum via their costal cartilages.
Parts of a Typical Rib
A typical rib has a head, neck, and body. The posterior end of the rib is called the head, followed by a narrow neck. The head articulates primarily with the costal...
Parts of a Typical Rib
A typical rib has a head, neck, and body. The posterior end of the rib is called the head, followed by a narrow neck. The head articulates primarily with the costal...
8.0K
01:15Spinal Cord: Gross Anatomy
5.3K
The spinal cord resides within the protective confines of the vertebral column. It is the main pathway for information traveling between the brain and the body. It plays a fundamental role in nearly all bodily functions, from simple reflexes to complex motor movements. The spinal cord begins at the medulla oblongata at the base of the brainstem and extends downward, terminating at the conus medullaris near the first and second lumbar vertebrae. The spinal cord's length in adults is...
5.3K