OBJECTIVE: Pediatric cervical spine injuries (CSI) can be devastating, and children < 8 years are particularly at risk for upper CSI given unique anatomical differences. Diagnosis of these injuries can be delayed due to variable clinical presentations and a paucity of existing literature. The authors aimed to characterize the spectrum of pediatric upper CSI.
METHODS: This was a retrospective, single-center case series of trauma patients aged < 16 years who were assessed at a level I pediatric trauma center and diagnosed with upper CSI between 2000 and 2020. Patients were included if they had evidence of bony or ligamentous injury from the occiput to C2 on imaging or autopsy. Data were obtained from manual chart review and analyzed using descriptive statistics.
RESULTS: In total, 502 patients were screened and 202 met inclusion criteria. Of these, 31 (15%) had atlanto-occipital (AO) joint distractions, 10 (5%) had atlanto-axial (AA) joint distractions, 31 (15%) had fractures of C1-2, and 130 (64%) had ligamentous injury without joint distraction. Of the patients with AO injury, 15 patients had complete dislocation. They presented as hemodynamically unstable with signs of herniation and 14 died (93%). In contrast, 16 had incomplete dislocation (subluxation). They usually had stable presentations and survived with good outcomes. Of the patients with AA injury, 2 had complete dislocation, presented with arrest and signs of herniation, and died. In contrast, 8 patients with subluxation mostly presented as clinically stable and all survived with little residual disability. The most common fractures of C1 were linear fractures of the lateral masses and of the anterior and posterior arches. The most common fractures of C2 were synchondrosis, hangman, and odontoid fractures. Overall, these patients had excellent outcomes. Ligamentous injuries frequently accompanied other brain or spine injuries. When these injuries were isolated, patients recovered well.
CONCLUSIONS: Among upper CSI, AO and AA joint injuries emerged as particularly severe with high mortality rates. Both could be divided into complete dislocations or incomplete subluxations, with clear clinical differences and the former presenting with much more severe injuries. Lateral cervical spine radiography should be considered during resuscitation of unstable trauma patients to assess for these CSI subtypes. Fractures and ligamentous injuries were clinically heterogeneous, with presentations and outcomes depending on severity and associated injuries.

Septic arthritis of the atlanto-occipital joint caused by Streptococcus intermedius is extremely rare. We present the first case report of this entity in a fully immunocompetent 5-year-old girl. The magnetic resonance imaging and blood tests were consistent with septic arthritis, so she started empirical antibiotic therapy. Septic arthritis should be excluded in children with torticollis, fever and neck pain.

The management of atlas fractures is controversial and hinges on the integrity of transverse atlantal ligament (TAL).
To identify risk factors for atlas fracture nonunion, with and without TAL injury.
All isolated, traumatic atlas fractures treated at our institution between 1999 and 2016 were analyzed. Multivariable logistic regression was used to identify variables associated with TAL injury confirmed on MRI, occult TAL injury seen on MRI but not suspected on computed tomography (CT), and with fracture nonunion on follow-up CT at 12 weeks.
Lateral mass displacement (LMD) ≥ 7 mm had a 48.2% sensitivity, 98.3% specificity, and 82.6% accuracy for identifying TAL injury. MRI-confirmed TAL injury was independently associated with LMD > 7 mm ( P = .004) and atlanto-dental interval ( P = .039), and occult TAL injury was associated with atlanto-dental interval ( P = .019). Halo immobilization was associated with having a Gehweiler type 3 fracture ( P = .020), a high-risk injury mechanism ( P = .023), and an 18.1% complication rate. Thirteen patients with TAL injury on MRI and/or LMD ≥ 7 mm were treated with a cervical collar only, and 11 patients (84.6%) healed at 12 weeks. Nonunion rates at 12 weeks were equivalent between halo (11.1%) and cervical collar (12.5%). Only age independently predicted nonunion at 12 weeks ( P = .026).
LMD > 7 mm on CT is not sensitive for TAL injury. Some atlas fractures with TAL injury can be managed with a cervical collar. Nonunion rates are not different between halo immobilization and cervical collar, but a strong selection bias precludes directly comparing the efficacy of these modalities. Age independently predicts nonunion.

BACKGROUND: Occipitocervical fixation (OCF) can provide good fusion rate to treat various craniovertebral junction (CVJ) pathologies. Biomechanically it gives rigid fixation, good fusion rate, and allows for effective decompression. However, rigid fixation on the mobile occipitocervical junction has shortcomings that affect the post-operative clinical functional outcomes and range of motion. This study aimed to evaluate and elaborate the functional outcomes, range of motions, and radiographic findings in our patients underwent OCF.
METHODS: We presented a report of 3 patients underwent posterior decompression procedure followed by occipitocervical fixation. All three patients\' clinical outcome was assessed clinically by, Japanese Orthopaedic Association (JOA) score and grading, Karnofsky, range of motion and radiographic cervical alignment evaluation parameters.
RESULTS: All patients have seen improvement (minimal 1 grade in JOA and >30 points of Karnofsky score) in 3 months after the procedure, had a tolerable range of motion limitation, normal range of cervical lordotic and cervical brow vertebral angle (CBVA). Unfortunately, one patient with loss of cranial fixation may be related to history of infection and lack of post-operative wound care.
CONCLUSIONS: Our cases conclude that Occipitocervical fixation is a safe technique that provides excellent fusion rate with good functional outcome and tolerable range of motion limitation. Due to its unique anatomy and technically demanding, serial post-operative monitoring evaluation of this procedure is paramount.

Occipitocervical fusion is an important surgical procedure to treat instability of the upper cervical and craniocervical junction. Fixation to the dense cortical bone of the occiput, contemporaneously typically accomplished with a plate and screws, is known to be strong and durable, but there are many competing methods used to secure an adequate number of fixation points of sufficient strength at the cervical end. Extension of hardware to the midcervical region to acquire additional fixation points, however, results in loss of subaxial motion segments and additional potential morbidity. The C2 vertebra is unique in that its morphology and dimensions permit fixation with longer screws than are typically possible to place in the midcervical lateral masses. Translaminar and pars screw techniques, both commonly used to achieve C2 fixation, are not mutually exclusive, as their respective trajectories are considerably different and engage different portions of the bony anatomy.
We describe a novel, 4-point C2 fixation technique for OC fusion that may avoid the need to extend fusion to the subaxial spine.
This technical note illustrates how 4-point C2 fixation can be employed in occiptocervical fusion.
4-point fixation of C2 combining translaminar and pars screw placement is technically feasible and may be a suitable strategy to spare subaxial motion segments in OC fusion procedures. Futher investigation may establish its applicability to additional surgical procedures.

Malformations in the craniocervical junction (CCJ) are rare in the pediatric population but often need surgical treatment. We present a pediatric case series of patients treated with a 2-stage surgical approach with a halo vest and occipitocervical fusion and review complications and outcomes.
A retrospective analysis of a single-center case series was performed. Pediatric patients affected by congenital craniocervical junction anomalies and treated with a 2-stage approach were included. A halo vest was implanted in the first surgery, and ambulatory progressive reduction was performed. When a favorable anatomic situation was observed, arthrodesis was performed. Safety analysis was undertaken by analyzing the incidence of complications in both procedures. Effectivity analysis was carried out analyzing radiologic and clinical outcome (Goel grade and modified Japanese Orthopaedic Association score). Student t test was used for statistical analysis.
Sixteen cases were included. Mean age of patients was 9.38 years. Safety analysis showed 2 halo loosenings, 1 pin infection, 2 wound infections, 1 cerebrospinal fluid leak, and 2 delayed broken rods. No major complications were observed. Radiologic analysis showed an improvement in the tip of the odontoid process to the McRae line distance (from -3.26 mm to -6.16 mm), atlantodental interval (from 3.05 mm to 1.88 mm), clival-canal angle (from 134.61° to 144.38°), and cervical kyphosis (from 6.39° to 1.54°). Clinical analysis also showed improvement in mean Goel grade (from 1.75 to 1.44) and modified Japanese Orthopaedic Association score (from 15.12 to 16.41).
The 2-stage approach was a suitable and effective treatment for craniocervical junction anomalies in pediatric patients.

The paracondylar process is an exostosis, situated lateral to the occipital condyles, which expands towards the transverse process of the atlas. The epitransverse process of the atlas is a bony outgrowth that extends from the transverse process towards the occiput. Ponticulus posterior is a bony bridge that spans from the lateral mass of the atlas towards the posterior extremity of the vertebral artery groove. They are important anatomical variations. In this article, we analyze a rare situation of concomitant presence of ponticulus posterior, foramen arcuate, paracondylar process and epitransverse process, all of them situated on the right side of an individual with artificial cranial deformation from the fifth century AD. The paracondylar process and the epitransverse process form an accessory atlantooccipital joint. The expression of these variations, though under genetic influence, might have also been influenced by artificial cranial deformation. To our knowledge, this association has not been reported. The epitransverse process and the ponticulus posterior are important because of the positional relationship with the vertebral artery on which they may exert compression effects generating blood flow disorders. The paracondylar process is located at the insertion of rectus capitis lateralis, an important surgical landmark, which could be affected by the presence of the paracondylar process, thus leading to possible difficulties in orientation and iatrogenic trauma. This case contributes to extending the knowledge regarding anatomical variations, being of great use to the contemporary medical field, especially surgery.

Nonmissile penetrating injuries to the craniocervical junction caused by a glass fragment are rare, and a standard management strategy has not been established.
A 75-year-old Japanese man was brought into our emergency department after receiving a left retroauricular stab wound by broken glass fragments. After spinal immobilization, a computed tomography (CT) scan revealed glass fragments penetrating at the right craniocervical junction to the interatlantooccipital subarachnoid space. CT angiography showed that both vertebral arteries were not injured. Magnetic resonance imaging demonstrated that the glass fragments did not penetrate the cervical cord or medulla oblongata. These glass fragments were removed via a midline incision from the external occipital protuberance to the C7 and with laminectomy without suboccipital craniectomy. Five of the glass fragments were found and removed in total. The dural defect was patched with a free fascia autograft. His postoperative course was uneventful. Postoperative CT angiography showed that both vertebral arteries were intact and the glass fragments had been removed completely.
CT graphical diagnosis is useful for the management of penetrating craniocervical junction trauma, and it should be considered in the evaluation of patients who have suffered craniocervical penetrating injury even in the absence of major wounds or bleeding. Spinal immobilization of patients with craniocervical penetrating injuries is crucial to avoid not only secondary neurologic damage but also secondary critical vascular damage. Incomplete or inadequate assessment of craniocervical stab wounds results in unexpected hazards that are preventable.

Spontaneous atlantooccipital dislocation is a rare clinical entity. Patients may present with neck pain and restriction of movements. Rarely does a patient present with hypoglossal nerve palsy. We report 1 such case, whose 12th nerve palsy promptly recovered after corrective surgery.

BACKGROUND: Although instrumented stabilization of pediatric atlanto-occipital dislocation (AOD) has been described in the literature, there is little evidence regarding instrumentation techniques in pediatric patients presenting with both AOD and a cervical fracture. We present a case of a 2-year-old male involved in a motor vehicle collision with an unstable C2 fracture and AOD, treated with an occiput-C4 posterior arthrodesis using a rod, crosslink, and cable construct.
METHODS: This patient suffered a type III C2 fracture and AOD with 4 mm craniocaudal and 3 mm anterior displacement. In the operating room, 2 cobalt chrome connecting rods (3.5 mm) were connected to 1 another with crosslinks at C2 and C4. These were affixed with suboccipital and sublaminar cables at C1, C2, and C4. At 14 months postoperatively, his spine is clinically and radiographically stable. He has spontaneous movement in all 4 extremities, and remains in a persistent vegetative state because of his underlying central nervous system injury.
CONCLUSIONS: Although there is a breadth of literature investigating instrumentation approaches to pediatric AOD, there is minimal evidence on outcomes of patients presenting with both AOD and cervical fracture. The technique we describe has proven safe and effective for this patient.