Spinal Deformities after Childhood Tumors

Abstract

Childhood tumors of the central nervous system (CNS) and other entities affecting the spine are rare. Treatment options vary from surgical biopsy to partial, subtotal, and total resection, to radiation, to chemotherapy. The aim of this study is to investigate spinal deformity and subsequent surgical interventions in this patient cohort. A retrospective review at our institution identified children with CNS tumors, spinal tumors, and juxta-spinal tumors, as well as spinal deformities. Tumor entity, treatment, mobilization, and radiographic images were analyzed relative to the spinal deformity, using curve angles in two planes. Conservative or surgical interventions such as orthotic braces, growth-friendly spinal implants, and spinal fusions were evaluated and analyzed with respect to treatment results. Tumor entities in the 76 patients of this study included CNS tumors (n = 41), neurofibromatosis with spinal or paraspinal tumors (n = 14), bone tumors (n = 12), embryonal tumors (n = 7), and others (n = 2). The initial treatment consisted of surgical biopsy (n = 5), partial, subtotal, or total surgical resection (n = 59), or none (n = 12), followed by chemotherapy, radiotherapy, or both (n = 40). Out of 65 evaluated patients, 25 revealed a moderate or severe scoliotic deformity of 71° (range 21-116°), pathological thoracic kyphosis of 66° (range 50-130°), and lordosis of 61° (range 41-97°). Surgical treatment was performed on 21 patients with implantation of growth-friendly spinal implants (n = 9) as well as twelve dorsal spinal fusions (two with prior halo distraction). Surgical interventions significantly improved spinal deformities without additional neurological impairment. With the increasing number of children surviving rare tumors, attention should be focused on long-term problems such as spinal deformities and consequent disabilities. A significant number of children with CNS tumors, spinal tumors or juxta-spinal tumors required surgical intervention. Early information about spinal deformities and a close follow-up are mandatory for this patient group.

Keywords: central nervous system; children; intramedullary spinal cord tumor; spinal deformity; treatment; tumor.

Figure 1

Tumor entities of the studied pediatric population (n = 76).

Figure 2

Seven-year old girl with neurofibromatosis type I and scoliosis of 101° (a,b). Bilateral rib-to-pelvis vertical expandable prosthetic titanium rib (VEPTR) devices were able to correct the scoliotic deformity to 43° (c,d). VEPTR implants are surgically lengthened every six months (e,f) to maintain the correction and to permit growth.

Figure 3

Fifteen-year old boy who was diagnosed with intraspinal astrocytoma at age ten. He received chemotherapy and radiotherapy. Five years later, severe thoracic spinal deformity had developed (a–c). Halo traction was applied for gradual scoliosis and kyphosis correction as well as reduction of the surgical risk due to neurological deterioration (d,e). Surgery was performed using spinal cord monitoring. When erecting the spine, motor evoked potentials (MEP) disappeared. After controlling correct screw placement by computed tomography, spinal deformity correction could only be performed until MEP weakened, thus leaving considerate kyphosis as a residual deformity (f,g).

Figure 4

MRI presentation of a cervical pilocytic astrocytoma grade I of the World Health Organization (WHO) grading system (yellow arrow) before (a) and after surgical resection (b) in a 14-year old girl. Surgical situs shows opening of the dura (c), CUSA (cavitronic ultrasonic surgical aspirator) de-bulking (d), opened cavitying of the spinal cord with partially removed tumor (e), closure of the spinal cord (f) and dural closure (g).

Figure 5

Fifteen-year old girl with juxta-spinal neurofibromatosis and severe three-dimensional upper thoracic spinal deformity (a–c). MRI prior to surgical treatment revealed severe neurofibromatosis with thoracic obstruction, which was incompletely removed.

Figure 6

Girl with intramedullary glioma WHO I° from T8 to L3, which was initially diagnosed at age two and partially removed using laminoplasty. At age 12, the patient had mild scoliosis and hyperkyphosis as well as severe lordosis (a,b). At age 14, deformity had progressed to 53° scoliosis, 60° kyphosis and 97° lordosis (c,d). Brace therapy did not correct the deformity sufficiently (e,f). Clinical presentation (g–j) reveals hip flexion contractures and enhanced lumbar lordosis. The patient needed trunk motion for walking due to a neurological impairment after treatment.