Atlantoaxial subluxation as an early manifestation in an adolescent with undifferentiated spondyloarthritis: a case report and review of the literature.

INTRODUCTION: Atlantoaxial instability has been described as a manifestation of ankylosing spondylitis (juvenile and adult onset), reactive arthritis, juvenile idiopathic arthritis, and rheumatoid arthritis; however, it has rarely been reported as an early manifestation of these disorders. We present this case report to increase awareness of the condition in the hope that earlier recognition of this disease may prevent further serious injury. CASE PRESENTATION: We report the case of a 17-year-old Hispanic adolescent woman who was initially diagnosed with undifferentiated spondyloarthritis due to peripheral arthritis, enthesitis, a positive human leukocyte antigen B27 result, and inflammatory spinal pain lasting two months. Our patient experienced persistent and worsening occipitocervical pain and signs of myelopathy three months after diagnosis; consequently, we found atlantoaxial instability along with cervical spine bone erosion and pannus formation. She was treated surgically with a C1-2 posterior instrumented fusion and at six weeks post-operatively was started on tumor necrosis factor α blockade. Her occipitocervical symptoms subsided following surgery and initiation of immunomodulation. CONCLUSIONS: Our report serves to emphasize to pediatric and adult general practitioners, pediatricians, internists, family physicians, pediatric and adult rheumatologists and spine surgeons that atlantoaxial subluxation may be an early manifestation of spondyloarthritis, and that the condition is treatable by surgical intervention and immunomodulation.

Intraoperative monitoring of motor evoked potentials in very young children.

OBJECT: Neurophysiological monitoring of motor evoked potentials (MEPs) during complex spine procedures may reduce the risk of injury by providing feedback to the operating surgeon. While this tool is a well-established surgical adjunct in adults, clinical data in children are sparse. The purpose of this study was to determine the reliability and safety of MEP monitoring in a group of children younger than 3 years of age undergoing neurosurgical spine procedures. METHODS: A total of 10 consecutive spinal procedures in 10 children younger than 3 years of age (range 5-31 months, mean 16.8 months) were analyzed between January 1, 2008, and May 1, 2010. Motor evoked potentials were elicited by transcranial electric stimulation. A standardized anesthesia protocol for monitoring consisted of a titrated propofol drip combined with bolus dosing of fentanyl or sufentanil. RESULTS: Motor evoked potentials were documented at the beginning and end of the procedure in all 10 patients. A mean baseline stimulation threshold of 533 ± 124 V (range 321-746 V) was used. Six patients maintained MEP signals ≥ 50% of baseline amplitude throughout the surgery. There was a greater than 50% decrease in intraoperative MEP amplitude in at least 1 extremity in 4 patients. Two of these patients returned to baseline status by the end of the case. Two patients had a persistent decrement or variability in MEP signals at the end of the procedure; this correlated with postoperative weakness. There were no complications related to the technique of monitoring MEPs. CONCLUSIONS: A transcranial electric stimulation protocol monitoring corticospinal motor pathways during neurosurgical procedures in children younger than 3 years of age was reliably and safely implemented. A persistent intraoperative decrease of greater than 50% in this small series of 10 pediatric patients younger than 3 years of age predicted a postoperative neurological deficit. The authors advocate routine monitoring of MEPs in this pediatric age group undergoing neurosurgical spine procedures.

Low-grade myofibroblastic sarcoma of the sacrum.

Myofibroblastic tumors are soft-tissue neoplasms arising from myofibroblasts, ubiquitous cells sharing ultrastructural features of muscular and fibroblastic cells. Vasudev and Harris described a malignant counterpart of these benign tumors in 1978. Most reported cases of myofibroblastic sarcoma have arisen in the head and neck region and the soft tissues of the extremities. To the best of the authors' knowledge, there have been only 8 previous reports on primary myofibroblastic sarcoma of the bone. The authors report a new case of this rare tumor affecting the sacrum and ilium of a 15-year-old girl and discuss the role of total sacrectomy and lumbopelvic reconstruction for treatment of this disease.

Video-assisted thorascopic repair of a subarachnoid-pleural fistula in a child after thoracic tumor resection: technical note.

STUDY DESIGN: Technical report. OBJECTIVE: To describe a minimally invasive surgical approach for the obliteration of a subarachnoid-pleural fistula in a 4-year-old child after resection of an intrathoracic ganglioneuroma. SUMMARY OF BACKGROUND DATA: Development of a subarachnoid-pleural fistula has been reported after thoracotomy for lung, chest wall, and spinal tumors, when an iatrogenic meningeal laceration results in establishing communication between the spinal subarachnoid space and the pleural cavity. METHODS: Review of a single case in which video-assisted thorascopic surgery (VATS) was used to deposit fibrin glue and to suture a pleural allograft. Literature review was performed to document other options to treat subarachnoid-pleural cerebrospinal fluid (CSF) fistula. RESULTS: At 10 months after VATS repair, the CSF fistula has remained closed. CONCLUSION: VATS technique should be considered for a safe, efficacious, and durable CSF leak repair and as an alternative to open thoracotomy in the pediatric age group.