Abstract

Background contextSurgical indications and procedures for spinal Langerhans cell histiocytosis (LCH) in children are still controversial. Reports containing large samples of surgically treated patients are few in the currently available literature, and the reported operative procedures were also somewhat obsolete. So, further investigation based on large-sample cases and using improved surgical techniques is beneficial and helpful to refine the treatment strategy. PurposeTo recommend a reasonable treatment strategy for thoracic or lumbar spine LCH in children complicated with neurologic deficit. Study design/settingRetrospective/academic medical center. Patient sampleTwelve children aged from 2 to 16 years old with the diagnosis of thoracic or lumbar spinal LCH accompanied by neurologic deficit received surgical treatment from January 2005 to January 2010. Outcome measuresFrankel scale for neurologic function, fusion of the mass, and recurrence of the lesion. MethodsAll 12 patients presented initially with local pain and progressive neurologic detriment. Neurologic evaluation revealed two patients with Frankel Grade B, eight with Grade C, and two with Grade D. Radiographic features were positive for typical vertebra plana, a space-occupying mass in the spinal canal compressing neural elements, and a spinal canal encroachment rate more than 50%. Posterior instrumentation with pedicle screw combined with anterior corpectomy, decompression, and support bone graft was performed in the first seven patients as a one-stage procedure. In the remaining five patients, posterior pedicle screw fixation, laminectomy for decompression (via excision of the tumor-like mass), and repair of laminae with allograft bone block were performed. The collapsed vertebral body was left untouched. No chemotherapy or radiotherapy was administrated postoperatively in any of the cases. ResultsThe mean follow-up duration was 43.3 months. The mean operation time was 330 minutes with combined procedure and 142 minutes with single posterior approach (p=.000). The average blood loss was 933 mL with combined procedure and 497 mL with single posterior approach (p=.039). Three of seven patients who received combined surgery encountered approach-related complications, that is, one with intercostal neuralgia and two with pleural effusion. No severe neurologic deteriorate, instrumentation failure, or disease recurrence was detected at follow-up. Neurologic function completely recovered in all 12 patients from 2 to 12 weeks after surgery. The anterior bone graft fused and shaped well in all seven patients, and allograft bone block for lamina repair also achieved complete fusion in the remaining five patients. The internal fixator was removed at 3 to 5 years (average 4.1 years) after initial operation in six patients. No deformity, including scoliosis and kyphosis, has been identified during follow-up period in both procedures. ConclusionsFor spinal LCH patients, neurologic deficit is a main indication for operative treatment to prevent permanent and serious consequences. Surgery provides an opportunity for rapid recovery of neurologic function. Both combined and single-stage posterior approaches based on pedicle screw instrumentation techniques are similarly effective in relieving neurologic compression. However, single-stage posterior approach is more favorable with less complications, and preserving involved vertebral body is not a latent hazard of recurrence.

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