|Year : 2021 | Volume
| Issue : 4 | Page : 63-64
Caudal Regression Syndrome with Craniovertebral Junction Anomaly
Ashutosh Kumar, Jayesh Sardhara
Department of Neurosurgery, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India
|Date of Submission||27-Oct-2021|
|Date of Acceptance||27-Oct-2021|
|Date of Web Publication||24-Dec-2021|
Department of Neurosurgery, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Kumar A, Sardhara J. Caudal Regression Syndrome with Craniovertebral Junction Anomaly. J Spinal Surg 2021;8:63-4
A 9-year-old male child, born of the nonconsanguineous marriage, presented with gradually progressive spastic quadriparesis and paresthesia for 1 year, bladder and bowel incontinence for 1½ years, and paraparesis noted at 3 months of age. The developmental milestones were achieved, and the higher mental functions were appropriate for age. The power in bilateral upper limbs was 3/5, and 1–2/5 in bilateral lower limbs. 60%–70% sensory loss (to all the modalities) was noted from C2 below.
CT-CVJ was suggestive of partial assimilation of the atlas with an incompletely formed posterior arch. Complex cervical deformity involved scoliosis toward the left and an exaggerated cervical lordosis, with C2-C3 retrolisthesis [Figure 1]a, [Figure 1]b, [Figure 1]c. Significant spinal canal narrowing (7.5 mm) was present at C2, causing spinal cord compression [Figure 1]d and [Figure 1]f. Sacral agenesis was noted with posterior tethering of the cord [Figure 1]d. Due to C1/C2 gross rotational deformity, the V3 segment of the vertebral artery becomes stretched and lies in the midline (anomalous medial deviation of a vertebral artery), posing extreme challenges of landmark identification and risk of injury during ventral decompression procedure [Figure 1]e.
Transoral odontoidectomy with C3 corpectomy followed by posterior occipital cervical (O-C3-C4-C5) fusion was planned. Due to the anatomical complexity of the deformity and the pediatric age group, the surgical management of this kind of syndromic craniovertebral junction anomalies is very challenging. To dealt with this bony anomalies, use of intraoperative neuronavigation, and three-dimensional computed tomography reconstruction images were essential requisites and were found to be immensely helpful for the identification of midline landmarks during ventral decompression. 16 mm × 3.5 mm screws were used for posterior cervical fixation [Figure 1]g and [Figure 1]h.
At 1 year of follow-up, adequate boney fusion was seen. Clinically, the patient had improvement in both power and spasticity. He was able to stand with support at 18 months of follow-up.
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Conflicts of interest
There are no conflicts of interest.