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SPINE IMAGE
Year : 2021  |  Volume : 8  |  Issue : 4  |  Page : 63-64

Caudal Regression Syndrome with Craniovertebral Junction Anomaly


Department of Neurosurgery, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India

Date of Submission27-Oct-2021
Date of Acceptance27-Oct-2021
Date of Web Publication24-Dec-2021

Correspondence Address:
Jayesh Sardhara
Department of Neurosurgery, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/joss.joss_22_21

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How to cite this article:
Kumar A, Sardhara J. Caudal Regression Syndrome with Craniovertebral Junction Anomaly. J Spinal Surg 2021;8:63-4

How to cite this URL:
Kumar A, Sardhara J. Caudal Regression Syndrome with Craniovertebral Junction Anomaly. J Spinal Surg [serial online] 2021 [cited 2022 Jan 21];8:63-4. Available from: http://www.jossworld.org/text.asp?2021/8/4/63/333618

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.
Figure 1: Spine image

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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.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.




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