Kyphosis Correction In A Patient With Spondyloepiphyseal Dysplasia

14-year old Sheetal [name changed] presented to the OPD of the author with features of spondyloepiphyseal dysplasia. She had kyphosis at the thoracolumbar junction with features of progression associated with pain [figure 1]. In view of this, she was offered surgical intervention in the form of PVCR [posterior vertebral column resection] at the apex of deformity.

MRI revealed developmental stenosis of the canal which necessitated that the spinal cord had ‘no reserve’ space in the canal [figure 2]. Shortening or lengthening the vertebral column would likely compromise cord space. Hence, care was taken to reconstitute the anterior column with a cage, to an extent that did not alter the spinal cord length [figure 3]. The surgery was carried out through a single approach from the posterior [backside] of the patient. This obviated the need for a second surgery from the anterior aspect [front side]. The surgical strategy involved working from around the spinal cord to remove the deformed bone anteriorly.
This led to a safe, satisfactory & effective outcome. Her kyphosis improved from 570 to 140 [75% correction].
This case highlights the meticulous care necessary to maintain neurological safety in such high risk surgeries.

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