Pediatric cervical Hemivertebrae - A Rare Case Presentation in a 3-Year-Old Child.

INTRODUCTION: Hemi vertebrae are very rarely seen at a cervical level which results in kyphotic deformity without coronal tilt. Vertebral formation defects have been the basis of congenital kyphoscoliosis deformity in the pediatric age group. Cervical spine kyphosis more than 10° along with kinking of cord at a single level results in neurodeficits which require urgent management on the lines of decompression, realignment, and bony fusion to prevent recurrence and failure and to achieve superior outcomes. However, in pediatric age group, spine surgeons face a lot challenges with respect to surgical anatomy, body landmarks, and bone anchors. CASE REPORT: A 3-year-old male patient presented to the outpatient department with complain of progressive bilateral upper and lower limb weakness and progressive deformity of the cervical spine which increased in the past 2 months. The patient earlier used to walk with support. However, for 2 months, there was progressive decrease in motor function. The clinical course, radiologic features, pathology, and treatment outcome of the patient were documented. C3 hemivertebrectomy and stabilization from C2 to C4 with fibular strut grafting and anterior cervical plating were done under neuromonitoring guidance. The neurologic symptoms of the patient were markedly improved after surgery. CONCLUSION: We have reported the first case of the management of pediatric cervical spine hemivertebrae with neurodeficit in a 3-year-old child, with anterior hemivertebrectomy, strut grafting, and plating which improved the patient neurologically and functionally.

Management of Acquired Compressive Myelopathy due to Spinal Fluorosis.

BACKGROUND: Fluorosis is an endemic disease of India which causes compressive cervical and/or dorsal myelopathy. This study aims to evaluate the role of surgical management in the crippling fluorosis along with evaluation of radiological imaging as screening/diagnosing tool for the disease. MATERIALS AND METHODS: This is a prospective cohort study of 33 patients operated at tertiary care center having nontraumatic involvement of spinal cord affecting neurology with history, clinical and radiological features (Ossified Posterior Longitudinal Ligament-, Ossified Ligamentum flavum) suggesting fluorosis as the cause of compression. Outcomes were measured in terms of improvement in Nurick grading, Rankins scale, spasticity, Oswestry Disability Index, modified Japanese Orthopaedic Association scores. RESULTS: Spinal fluorosis is a male predominant disease affecting the elderly after years of fluorine intake. Cervical and/or dorsal spine are predominantly involved at multiple levels (>=2). Diagnosis of the disease poses difficulty due to lack of established laboratory parameters with high sensitivity, availability, and lack of awareness among surgeons. Skeletal survey alone has >90% sensitivity for diagnosing the disease. Once evaluated properly, decompression at correctly identified levels invariably improves the spasticity and quality of life immediately post-surgery. At final followup, there was on average improvement of 2 scales in nurick grade, rankins scale and ashworth grading whereas average improvement in ODI, mJOA and dorsal specific mJOA were 52%, 3.17 points and 2.7 points respectively. However, preoperative counselling for "apparent neurological deterioration" in immediate postoperative period is very important. Complications like infection and dural tear have to be prevented with special surgical tactics. CONCLUSION: Skeletal survey along with computed tomography and magnetic resonance imaging is cost-effective modality for the screening/diagnosis for fluorosis. Once developed, surgery, either curative or palliative, is the best treatment at crippling stage of the disease.

Are Two Surgeries Necessary for Correction of 1 Deformity- Cervical Kyphosis in Neurofibromatosis?- A Rare Case Report.

INTRODUCTION: Dystrophic neurofibromatosis type I, involving upper cervical spines, is rare, which can cause serious complications. Myelopathy develops due to compression of the cord posteriorly. Surgical correction has its inherent risks and difficulties because of poor bone quality, difficult anterior approach because of bizarre deformities, and the necessary manipulation, which might cause more cord damage and ischemia. Anterior decompression with alignment correction was an early popular choice. But without posterior shortening, the technique proved unsatisfactory in restoring normal alignment. It tended to expose the graft bone at increased risk of insufficient union or extruding. So a combination of anterior decompression and posterior correction was generally recommended. This report describes surgical technique applicable to cases of severe cervical kyphosis using only anterior approach. CASE REPORT: A 13-year-old boy, a case of neurofibromatosis type 1 presented with neck pain, and bilateral upper limb radiculopathy for 2 months with affected daily living, with intact neurology. Imaging demonstrated 46° kyphosis (C3-C5), marked dystrophic changes of the C4 and C5, extreme dorsal angulation indenting the cord. Anterior surgery was planned for the patient with neuromonitoring. Through left anterior approach, total C3, C4 and C5 corpectomy was accomplished, keeping the PLL intact. The defect was filled with a fibular cortical graft which was stabilized with 48 mm cervical screw plate, showing full correction of kyphosis postoperatively. At the follow-up 6 months postoperatively, lateral plain radiograph showed stability of the cervical spine fusion without correction loss. The patient had full relief from tingling and numbness with no neurological deficit and could resume his daily duties. Patient is asymptomatic at 2 years follow up. CONCLUSION: Only anterior decompression and fusion are effective in satisfactorily correcting cervical kyphosis in cases of neurofibromatosis without neurologic compromise and avoids the risk of damage to vital neurovascular structures because of pedicle or lateral mass screw fixation.