Abstract

Background: Resection of intramedullary spinal cord tumors (IMSCTs) in pediatric patients results in a high incidence of spinal deformity (i.e., kyphoscoliosis often requiring fusion). Here, a 6-year-old male underwent a spinal fusion to correct postlaminectomy thoracic kyphosis following resection of an IMSCT.

Case Description: A 6-year-old male initially underwent multilevel thoracic laminectomies for resection of an IMSCT. Six months later, he presented with the onset of kyphoscoliosis. During adolescence he became increasingly paraparetic due to a thoracic kyphosis that had now progressed to 118°. He underwent a 360 decompression/fusion that included a T1-T9 laminectomy, a T5 to T11 anterior interbody arthrodesis/rib autograft, and posterolateral T2-T12 fusion/iliac crest autograft with Harrington rods placed from T5 to T12. Postoperative radiographs showed the thoracic kyphosis improved to 62°. However, 4.5 years later, X-rays showed the thoracic kyphosis newly progressed to 90° (i.e., from T3 to T12). Thirty-four years after this corrective surgery, he remained neurologically intact with only mild complaints of balance changes, and bladder/bowel urgency while radiographs confirmed continued stability. Further, the thoracic magnetic resonance imaging showed only chronic thoracic spine/cord changes.

Conclusion: A 6-year-old child originally underwent a thoracic laminectomy for an IMSCT. As an adolescent, due to progressive postlaminectomy kyphosis, he underwent a successful secondary thoracic 360° decompression/ fusion. Notably, 34 years later, he did not require any surgical revision.

Keywords: Cerebrospinal fluid, Intramedullary spinal cord tumor, Postlaminectomy kyphosis, Scoliosis, Kyphosis

INTRODUCTION

Intramedullary spinal cord tumors (IMSCTs) make up 2–5% of all primary spinal cord tumors; gliomas accounting for 80% of these lesions.[ 11 ] In children, astrocytomas make up nearly 60% of the World Health Organization (WHO) grades I/II cervical/ thoracic (C/T) IMSCTs tumors, while with remaining 40% include WHO grade II C/T ependymomas.[ 6 , 11 ] Patients typically present with back or neck pain, and myelopathy/paraparesis attributed to progressive kyphosis/deformity often warranting 360° decompressions/fusions.[ 6 ] Here, a 6-year-old male originally underwent a laminectomy for a IMSCT. However, by age 13, he required an extensive circumferential decompression/fusion for postlaminectomy kyphoscoliosis. Notably, 34 years later, he remained neurologically and radiographically stable.

CASE REPORT

Initial presentation

A 6-year-old male, diagnosed with a IMSCT, underwent an initial thoracic laminectomy and radiation therapy for partial tumor resection (i.e., “glioma”). Postoperatively, he was fully plegic with a T6 sensory level and thoracic kyphosis from T1 to T12 (45°). By 6.5 years of age, the scoliosis progressed [ Table 1 ] for which he received a Milwaukee brace. He was neurologically intact until age 13 when the thoracic kyphosis progressed and he developed new bilateral lower extremity weakness (i.e., 4/5 proximal/ distal) [ Table 1 ].

Table 1:

Scoliosis progression.

Corrective surgery

After traction in a halo in a wheelchair, he underwent an anterior fusion (i.e. a thoracotomy with discectomies and interbody rib graft fusions from T5 to T11). Notably, 39 days later he underwent a circumferential decompression/fusion; T1 to T9 laminectomy/posterolateral T2-T12 fusion with iliac crest autograft, and placement of T5 to T12 Harrington rods (i.e., hooks of placed on the transverse processes of T5 through T7 and T10 through 12 bilaterally). A dural tear at the T6 level was immediately repaired intraoperatively. On postoperative day 8, the patient’s cerebrospinal fluid leak resolved with conservative treatment and he was placed in a halo cast for several months. Postoperative radiographs showed continued improvement after both surgeries [ Table 1 ].

Postoperative course

Nine months later, (i.e., age 13 years 9 mos.), he developed a wound dehiscence and required removal of the right-sided instrumentation with debridement followed by placement of a Milwaukee brace. At ages 18 and 23, he remained neurologically normal and the thoracic kyphosis had largely stabilized [ Table 1 ]. At age 47, he presented with mild myelopathy and issues, bowel and bladder urgency and stable scoliosis [ Table 1 and Figure 1 ]. The cervical and lumbar MR studies were negative. Notably, the thoracic magnetic resonance imaging showed an atrophic upper thoracic spinal cord, while the lower thoracic cord revealed an increased intramedullary T2 signal that extended to the conus [ Figures 2 and 3 ]. As he was just mildly symptomatic, no further surgery was offered.

Figure 1:

Standing posteroanterior (left) and lateral (right) scoliosis plain films. There is a right-side scoliotic curve from T8 to L3 (apex at T12) of 31° and thoracic kyphosis from T3 to T12 of 82°. The size of the thoracic vertebral bodies appears small given the size of the lumbar vertebral bodies, and the thorax is proportionally small.

Figure 2:

A T2-weighted sagittal magnetic resonance imaging of cervical and upper thoracic spine demonstrating dural ectasia in the upper thoracic spine associated with an apparent extensive laminectomy defect that begins at T2 and extends distally. The visualized thoracic spinal cord is markedly atretic and adherent to the posterior aspect of the thecal sac. There is considerable artifact from instrumentation at T6.

Figure 3:

A T2-weighted sagittal magnetic resonance imaging of the thoracic spine demonstrating increased intramedullary T2 signal that extends to the conus medullaris. There is considerable artifact from instrumentation that obscures the mid- and most of the upper thoracic spine.

DISCUSSION

Pediatric surgery for IMSCT

Surgical resection is the mainstay of IMSCT treatment in the pediatric age group [ Table 2 ]. However, this carries the risk for developing postlaminectomy spinal deformity/scoliosis (incidence 24–100%).[ 1 , 3 , 5 , 8 , 9 ] Children like the 6-year-old presented are at higher risk for spinal deformity largely due to; underlying ligamentous laxity, cartilage content, reduced paraspinal musculature support, horizontal orientation of facet joints, radiation therapy to an underdeveloped spine, and disproportionate load bearing due to the relative cranium to body mass ratio.[ 2 , 5 , 10 ]

Table 2:

Literature review of IMSCTs.

MSCT resection in the pediatric population

Pediatric patients undergoing initial IMSCT resection for tumor recurrence/progression will typically demonstrate progressive postoperative kyphosis warranting posterolateral fusion. One study evaluated kyphoscoliosis resulting in paraplegia 42 years after a childhood laminectomy for a IMSCT.[ 4 ] Kelley et al.[ 7 ] recently reported success at 18 months following a resection of a IMSCT with multilevel thoracic pedicle subtraction osteotomy/fusion performed in a pediatric patient with post laminectomy kyphotic deformity.

CONCLUSION

A 6-year-old child underwent a thoracic laminectomy for an IMSCT requiring subsequent revision circumferential decompression/fusion. Notably, 34 years later, the patient presented with mild hyperreflexia and did not warrant repeated surgery.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

Disclaimer

The views and opinions expressed in this article are those of the authors and do not necessarily reflect the official policy or position of the Journal or its management. The information contained in this article should not be considered to be medical advice; patients should consult their own physicians for advice as to their specific medical needs.

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