Chordomas are histologically benign tumors with local aggressive behavior. They arise from embryological remnants of the notochord at the clivus, mobile spine, and sacrum. Chordomas are rare tumors in the pediatric age group. Their surgical management is difficult, given their propensity for inaccessible anatomical regions, and proximity to critical neurovascular structures. While en bloc resection with surgical margins has been advocated as the preferred approach for chordomas, tumor characteristics and violation of adjacent anatomical boundaries may not allow for safe en bloc resection of the tumor. Here, the authors present the case of a C1 chordoma in a 5-year-old boy with epidural and prevertebral extension. The patient’s treatment consisted of a far-lateral approach for resection of the tumor and C1 arch, followed by circumferential reconstruction of the craniocervical junction with an expandable cage spanning the skull base to C2, and posterior occipitocervical spinal instrumentation. At 42 months after surgery, the patient remains neurologically intact with stable oncological status, and no evidence of craniocervical junction instrumentation failure.
Lourdes C. Eco, Alison Brayton, William E. Whitehead, and Andrew Jea
Rob Dickerman and Ashley Reynolds
Andrew Jea, Keyne K. Johnson, William E. Whitehead, and Thomas G. Luerssen
The use of spinal instrumentation to stabilize the occipitocervical junction in pediatric patients has increased and evolved in recent years. Wiring techniques have now given way to screw-rod or screw-plate techniques with or without postoperative external immobilization. Although C-2 translaminar screws have been used in these constructs, subaxial translaminar screws have not, to date, been described in either the pediatric or adult patient populations.
The authors describe the feasibility of translaminar screw placement in the C-3 lamina. Rigid fixation with translaminar screws offers an alternative to subaxial fixation with lateral mass screws, allowing for formation of biomechanically sound spinal constructs and minimizing potential neurovascular morbidity. Their use requires careful analysis of preoperative imaging studies, intact posterior elements, and avoidance of violation of the inner laminar wall.
Ashwin Viswanathan, Katherine Relyea, William E. Whitehead, Daniel J. Curry, Thomas G. Luerssen, and Andrew Jea
The authors describe a rare case of pneumothorax as a complication of thoracic pedicle screw placement in an 11-year-old girl undergoing posterior segmental instrumentation for a kyphotic deformity. Spontaneous pneumothorax after posterior fusion for adolescent idiopathic scoliosis has been reported in the orthopedic literature; however, to the best of the authors' knowledge, pneumothorax directly related to pedicle screw placement for spinal deformity has not been previously described. The authors discuss the anatomical and technical aspects leading to this complication and the lessons learned from it.
Robert H. Rosenwasser
James M. Drake
Sandi Lam, Thomas G. Luerssen, William E. Whitehead, Andrew Jea, and I-Wen Pan
Roukoz B. Chamoun, William E. Whitehead, Daniel J. Curry, Thomas G. Luerssen, and Andrew Jea
The use of C-1 lateral mass screws provides an alternative to C1–2 transarticular screws in the pediatric population. However, the confined space of the local anatomy and unfamiliarity with the technique may make the placement of a C-1 lateral mass screw more challenging, especially in the juvenile or growing spine.
A CT morphometric analysis was performed in 76 pediatric atlases imaged at Texas Children's Hospital from October 1, 2007 until April 30, 2008. Critical measurements were determined for potential screw entry points, trajectories, and lengths, with the goal of replicating the operative technique described by Harms and Melcher for adult patients.
The mean height and width for screw entry on the posterior surface of the lateral mass were 2.6 and 8.5 mm, respectively. The mean medially angled screw trajectory from an idealized entry point on the lateral mass was 16° (range 4 to 27°). The mean maximal screw depth from this same ideal entry point was 20.3 mm. The overhang of the posterior arch averaged 6.3 mm (range 2.1–12.4 mm). The measurement between the left- and right-side lateral masses was significantly different for the maximum medially angled screw trajectory (p = 0.003) and the maximum inferiorly directed angle (p = 0.045). Those measurements in children < 8 years of age were statistically significant for the entry point height (p = 0.038) and maximum laterally angled screw trajectory (p = 0.025) compared with older children. The differences between boys and girls were statistically significant for the minimum screw length (p = 0.04) and the anterior lateral mass height (p < 0.001).
A significant variation in the morphological features of C-1 exists, especially between the left and right sides and in younger children. The differences between boys and girls are clinically insignificant. The critical measurement of whether the C-1 lateral mass in a child could accommodate a 3.5-mm-diameter screw is the width of the lateral mass and its proximity to the vertebral artery. Only 1 of 152 lateral masses studied would not have been able to accommodate a lateral mass screw. This study reemphasizes the importance of a preoperative CT scan of the upper cervical spine to assure safe and effective placement of the instrumentation at this level.
Roukoz B. Chamoun, Michel E. Mawad, William E. Whitehead, Thomas G. Luerssen, and Andrew Jea
Currently, no diagnostic or treatment standards exist for extracranial carotid artery dissection (CAD) in children after trauma. The purpose of this study was to review and describe the characteristics, diagnosis, and treatment of this rather uncommon sequelae of pediatric trauma.
A systematic review of the literature was performed to examine the pertinent studies of traumatic extracranial carotid artery (CA) injuries in children.
No randomized trials were identified; however, 19 case reports or small case series consisting of 34 pediatric patients were found in the literature. The diagnosis of CAD was made in 33 of 34 patients only after the onset of ischemic symptomatology. Twenty-four of 34 patients underwent cerebral angiography to confirm diagnosis; MR angiography affirmed the diagnosis in 6 of 34 patients. There was little published experience with CA ultrasonography or CT angiography for diagnosis. Thirty of 34 patients were treated with medical therapy or observation; 2 of 4 patients treated with observation alone died. There was little experience with open surgical treatment of CAD in the pediatric population, and there were no studies on the endovascular treatment of traumatic CAD in children. The literature does not support anticoagulation therapy over antiplatelet therapy.
As a result of this review of the literature, the authors propose the algorithms for the evaluation and treatment of traumatic extracranial CADs in children. These recommendations include utilizing MR angiography as a screening tool in cases in which the clinical suspicion of CAD is high, using conventional cerebral angiography to confirm the diagnosis, implementing antiplatelet therapy as initial medical management, and reserving endovascular stenting in cases of failed medical treatment.
Leonardo Rangel-Castilla, Steven W. Hwang, William E. Whitehead, Daniel J. Curry, Thomas G. Luerssen, and Andrew Jea
Tuberculosis (TB) is a common disease worldwide that is caused by Mycobacterium tuberculosis. Tuberculosis of the spine, also called Pott disease, is the most common site of bony dissemination. Although children are disproportionately affected, spinal TB is nonetheless rare in very young children. Cases involving infants requiring surgical intervention have been previously reported, and they are often associated with greater management challenges given the technical difficulty with instrumentation in very young children.
This case involved a 3-year-old girl with TB centered at T-6, who presented with myelopathy from spinal cord compression and a severe kyphotic deformity (> 60°). She underwent a single-stage costotransversectomy for vertebral column resection, followed by reconstruction with an anterior expandable titanium cage and posterior pedicle screw instrumentation. At last follow-up, the patient was clinically and radiographically stable.
The authors report on the youngest patient with spinal TB treated surgically with this strategy and review the literature regarding prior cases involving young children. Although limited by the paucity of cases in the literature, surgical debridement and spinal fusion appear to provide a safe alternative to prolonged bed rest or casting and may offer additional benefits of a faster recovery and ambulation.