PMC8422436_01
T1 postcontrast thoracic (a) and lumbar (b) and T2 noncontrast thoracic (c) and lumbar (d) sagittal midline views of patient's postoperative MRI demonstrate an expansile, intramedullary astrocytoma extending from T7 to L3 is demonstrated with associated syringohydromyelia extending from C2 to T7 with a remodeled and expanded spinal canal after decompressive laminectomies were performed from T7 to L3.
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F2
In 2007, at the age of 2, the patient underwent thoracic six (T6) to lumbar two (L2) laminectomies, for subtotal resection (STR) of a pilocytic astrocytoma with expansion duraplasty [Figure 2] followed by adjuvant chemotherapy.
PMC8422436_02
Serial standing scoliosis radiographs. (a,d) Preoperative AP and lateral radiographs before laminectomy and tumor resection. (b,e) First postoperative standing long-cassette thoracolumbar radiographs, AP and lateral, obtained in 2010, treated with external bracing. (c,f) Interval AP and lateral standing long-cassette thoracolumbar radiographs obtained in 2015 demonstrating a progressive kyphoscoliosis. (g) Continued progression of scoliosis seen on subsequent AP long-cassette thoracolumbar radiographs obtained in 2016. (h) Hand radiographs obtained to evaluate patient's bone age in 2015, which was determined to be advanced of her chronologic age.
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F3
Over the next few years, the patient's kyphoscoliosis continued to progress [Figure 3], while interval MRIs demonstrated gross stability of her IMSCT [Figure 4]. Having reached skeletal maturity [Figure 3e], the patient underwent surgical correction of her progressive kyphoscoliosis, in December 2016.
Interval postoperative MRI obtained to evaluate for tumor recurrence or progression revealed new enhancing 6 mm nodule at level of T11/12, deemed clinically insignificant in context of progressive kyphoscoliosis and stable in appearance on subsequent MRIs. (a) T2 noncontrast image of thoracic spine in sagittal plane, with evidence of significant coronal deformity as well as T2 hyperintense cystic lesions in the lower thoracic spine. (b) T1 postcontrast image of the thoracic spine at the inferior aspect of T11 with evidence of enhancing nodule (blue arrow).
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F4
Over the next few years, the patient's kyphoscoliosis continued to progress [Figure 3], while interval MRIs demonstrated gross stability of her IMSCT [Figure 4].
Preoperative standing scoliosis radiographs including right lateral bending (a), upright AP (b), left lateral bending (c), and upright lateral (d) views. These films show a Lenke type 3+ scoliotic curve - levoscoliosis of the thoracic spine with cobb angle 46  and apex at T8, as well as a dextroscoliosis of the lumbar spine with a Cobb angle of 41  and apex at L2. There was minimal improvement of the patient's coronal deformity on lateral bending. The patient also has a thoracic kyphosis of 80 degrees. The optimal lowest instrumented vertebra was determined by the neutralization of the L2-3 disc space on left-sided bending radiographs (c).
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F5
Preoperative standing scoliosis and lateral bending X-rays were used to plan the extent of her fusion [Figure 5], extending posteriorly from T3-L2.
PMC8422436_03
(a) Typical distraction and compressive forces (black arrows) placed along apex of coronal deformity in the absence of intraspinal pathology. (b) Utilization of primarily compressive forces (red arrows) along convexity of the curve to prevent over lengthening the spinal canal in the presence of intraspinal pathology, as was the case in our patient. Select axial T2-weighted noncontrast and one T1-weighted postcontrast MRI cross-sections of her spine highlight progressive intradural tumor (blue circles), cysts, and syringomyelia.
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F6
T3 to L2. Correction of the patient's coronal and sagittal deformity was accomplished utilizing a combination of cantilever and in situ compression maneuvers. The patient's coronal deformity was primarily fixed through compression of the convex aspect of her curve [Figure 6].
PMC8422436_04
Standing thoracolumbar radiographs immediately (a) and 2-year postoperative (b) demonstrating significant improvement in patient's coronal deformity as well as restoration of a physiologic sagittal alignment.
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F7
The patient's postoperative standing scoliosis radiographs demonstrated significant reduction of her kyphoscoliosis [Figure 7].