Related Experiment Videos
Spinal cord abnormalities in caudal regression syndrome
1Department of Pathology, Milton S. Hershey Medical Center, Pennsylvania State University, Hershey 17033.
Acta Neuropathologica
|January 1, 1991
Summary
Caudal regression syndrome involves spinal cord malformations, with severity correlating to vertebral anomalies. Neuropathologic findings in infants with sirenomelia and lumbosacral agenesis highlight caudal spinal cord abnormalities.
Area of Science:
- Neuropathology
- Developmental biology
- Pediatric neurology
Background:
- Caudal regression syndrome (CRS) encompasses a spectrum of congenital anomalies, from sacral agenesis to sirenomelia.
- Sirenomelia, a severe form of CRS, presents with lower limb malformations and agenesis of the rectum and genitourinary tracts.
Observation:
- This study details the neuropathologic examination of four infants diagnosed with CRS: three with sirenomelia and one with lumbosacral agenesis.
- Cerebral and upper spinal cord structures were normal; however, all cases exhibited abnormalities in the caudal spinal cord region.
Findings:
- The severity of caudal spinal cord anomalies, including sacral root/ganglia fusion, heterotopias, filar lipomas, and spinal cord tethering, was found to be proportional to the extent of vertebral anomalies.
- In severe lumbosacral agenesis, there was a complete absence of the lumbosacral spinal cord and dysplasia of thoracic cord segments.
Implications:
- These neuropathologic findings suggest that caudal spinal cord malformations are integral components of Caudal Regression Syndrome.
- Understanding the correlation between vertebral and spinal cord anomalies can aid in diagnosing and managing CRS.