Winner of CoW 11 September 2016
Truncated / wedge shaped high termination of spinal cord with partial sacral agenesis - Caudal regression syndrome - Group 1
Findings
Spinal cord ends at a higher level at D12 with 'truncated' or 'wedge' shaped termination. Hypoplastic S2 vertebrae with failure of formation of S3 and onwards.
Discussion
Caudal regression is a relatively rare congenital anomaly characterized by caudal vertebral agenesis or dysgenesis (including hemisacral anomalies), most often in combination with spinal cord malformations .The vast majority of cases are sporadic, however familial cases occasionally occur. An association with VACTERL and Currarino triad syndromic complexes has been reported.In an antenatal setting there is also associations with maternal diabetes. Staging, Grading or Classification Criteria: Group 1: More severe caudal dysgenesis with high-lying, ‘’club or wedge shaped ‘’ terminating cord. This group is associated with severe sacral osseous anomalies. +/- dilated central canal, conus CSF cyst. Group 2: Less severe dysgenesis with low-lying, tapered, distal cord tethered by tight filum. This group is associated with milder sacral dysgenesis with tethered cord +/- Lipoma, lipomyelomeningocele, or terminal myelocystocele.
Findings
Spinal cord ends at a higher level at D12 with 'truncated' or 'wedge' shaped termination. Hypoplastic S2 vertebrae with failure of formation of S3 and onwards.
Discussion
Caudal regression is a relatively rare congenital anomaly characterized by caudal vertebral agenesis or dysgenesis (including hemisacral anomalies), most often in combination with spinal cord malformations .The vast majority of cases are sporadic, however familial cases occasionally occur. An association with VACTERL and Currarino triad syndromic complexes has been reported.In an antenatal setting there is also associations with maternal diabetes. Staging, Grading or Classification Criteria: Group 1: More severe caudal dysgenesis with high-lying, ‘’club or wedge shaped ‘’ terminating cord. This group is associated with severe sacral osseous anomalies. +/- dilated central canal, conus CSF cyst. Group 2: Less severe dysgenesis with low-lying, tapered, distal cord tethered by tight filum. This group is associated with milder sacral dysgenesis with tethered cord +/- Lipoma, lipomyelomeningocele, or terminal myelocystocele.
Note:
We do not discourage differential diagnosis. But all the differentials must satisfy the findings noted in the case.
If you feel you have answered rightly but cannot find your name in the above list, please call 09551942599.
Did you Know?
The order in which the names appear in this winner's list is based on the time of submission. The first person to send the correct answer gets his/her name on top of the list!
We do not discourage differential diagnosis. But all the differentials must satisfy the findings noted in the case.
If you feel you have answered rightly but cannot find your name in the above list, please call 09551942599.
Did you Know?
The order in which the names appear in this winner's list is based on the time of submission. The first person to send the correct answer gets his/her name on top of the list!