Douglas L. Brockmeyer
✓ A new technique for performing a posterior rib and multistranded cable atlantoaxial fusion in children is described. The technique has been used successfully, in two patients 22 and 18 months of age, respectively. In both cases, fusion was used to augment C1–2 transarticular screw fixation, and solid arthrodesis was achieved without a halo orthosis.
Douglas L. Brockmeyer, Meghan M. Brockmeyer, and Taryn Bragg
Congenital craniovertebral anomalies are relatively common, but anomalies leading to overt craniocervical instability may be difficult to recognize and treat. The authors present a series of patients with atlantal hemi-rings, a disorder resulting in congenital craniovertebral instability. Presentation, treatment, imaging, and follow-up data obtained in patients with atlantal hemi-rings were assessed to identify factors relevant to craniocervical instability.
Nineteen patients were identified with atlantal hemi-rings, defined as a bony discontinuity of the C-1 ring in conjunction with lateral displacement of the C-1 lateral masses (as seen on coronal CT scans). Clinical and radiological characteristics were analyzed, including patient age at presentation, extent of occipitocervical motion, amount of C-1 lateral mass displacement, associated craniocervical anomalies, integrity of the transverse ligament, and neurological status.
The mean patient age at presentation was 22 months (range birth to 9 years). The mean amount of occipitocervical translation seen on dynamic imaging was 9 mm (range 2–20 mm). Four patients required occipitocervical fusion at presentation. The remaining 15 patients were monitored for a mean of 20 months, and 9 ultimately underwent fusion. Surgery was also recommended for 4 of the remaining 6 children.
This report describes the radiological and clinical characteristics of patients with atlantal hemirings and craniocervical instability. The authors believe that this anomaly is the underlying cause of progressive instability in a significant proportion of patients with craniocervical abnormalities. The presence of atlantal hemi-rings should prompt immediate and thorough neurosurgical evaluation.
Sarah T. Garber and Douglas L. Brockmeyer
Subaxial cervical instability in very young or small-for-age children is uncommon and typically arises from trauma or skeletal dysplasia. Various operative techniques have been used to achieve stabilization in pediatric patients with evidence of instability, including anterior, posterior, and combined approaches. In this study, the authors report their results with subaxial cervical instability in this patient population treated using a static single-screw anterior cervical plate (ACP) system and allograft fusion.
In a retrospective chart review, the authors identified all patients 6 years of age or younger who underwent an anterior cervical fusion procedure using a static single-screw ACP system either as a stand-alone construct or as part of an anterior-posterior stabilization procedure. Reasons for fusion included trauma, tumor, and congenital anomalies.
Five patients 6 years of age or younger underwent anterior cervical fusion using a static single-screw system during the 19-year study period. Follow-up ranged from 12 to 51 months (mean 26.8 months). Two patients underwent repeat surgery, one 7 days after and the other 21 months after their initial procedure. At last follow-up, a mean vertical growth of 22.8% was seen across the fused segments, with no evidence of kyphotic or lordotic abnormalities.
In very young or small-for-age children, the use of a static single-screw ACP system appears to be a safe and effective option to manage subaxial cervical instability. Bony fusion and continued longitudinal growth occur within the fused segments, with no evidence of long-term cervical malalignment.
Wayne M. Gluf and Douglas L. Brockmeyer
Object. In this, the second of two articles regarding C1–2 transarticular screw fixation, the authors discuss their surgical experience in treating patients 16 years of age and younger, detailing the rate of fusion, complication avoidance, and lessons learned in the pediatric population.
Methods. The authors retrospectively reviewed 67 consecutive patients (23 girls and 44 boys) younger than 16 years of age in whom at least one C1–2 transarticular screw fixation procedure was performed. A total of 127 transarticular screws were placed in these 67 patients whose mean age at time of surgery was 9 years (range 1.7–16 years). The indications for surgery were trauma in 24 patients, os odontoideum in 22 patients, and congenital anomaly in 17 patients. Forty-four patients underwent atlantoaxial fusion and 23 patients underwent occipitocervical fusion. Two of the 67 patients underwent halo therapy postoperatively.
All patients were followed for a minimum of 3 months. In all 67 patients successful fusion was achieved.Complications occurred in seven patients (10.4%), including two vertebral artery injuries.
Conclusions. The use of C1–2 transarticular screw fixation, combined with appropriate atlantoaxial and craniovertebral bone/graft constructs, resulted in a 100% fusion rate in a large consecutive series of pediatric patients. The risks of C1–2 transarticular screw fixation can be minimized in this population by undertaking careful patient selection and meticulous preoperative planning.
Douglas L. Brockmeyer and Ronald I. Apfelbaum
✓ Posterior occipitocervical stabilization procedures were successfully performed in 10 patients (nine boys and one girl) 16 years of age or younger by using C1–2 transarticular screws coupled with a rigid occipitocervical construct. The average length of follow-up evaluation was 18.8 months (range 5–37 months). No implant failed and all fusions were successful without the use of an external orthotic halo device.
Rajiv R. Iyer and Douglas L. Brockmeyer
This case involved a 6-year-old boy with Down syndrome, left C1 lateral mass hypertrophy, C1–2 rotatory subluxation, and spinal cord compression. He presented after falling down some stairs at his home. Torticollis, dysphagia, and speech delay were noted on examination. Vascular imaging showed impingement on the left vertebral artery by the anomalous C1 lateral mass. Through a posterior approach, the hypertrophic C1 lateral mass was resected, and an occiput–C2 fusion was performed. Postoperatively, his torticollis and brainstem symptoms were resolved.
The video can be found here: https://youtu.be/1U0GLdw6c70
Robert J. Bollo, Jay Riva-Cambrin, Meghan M. Brockmeyer, and Douglas L. Brockmeyer
Chiari malformation Type I (CM-I) is a congenital anomaly often treated by decompressive surgery. Patients who fail to respond to standard surgical management often have complex anomalies of the craniovertebral junction and brainstem compression, requiring reduction and occipitocervical fusion. The authors hypothesized that a subgroup of “complex” patients defined by specific radiographic risk factors may have a higher rate of requiring occipitocervical fusion.
A retrospective review was conducted of clinical and radiographic data in pediatric patients undergoing surgery for CM-I between 1995 and 2010. The following radiographic criteria were identified: scoliosis, syringomyelia, CM Type 1.5, medullary kinking, basilar invagination, tonsillar descent, craniocervical angulation (clivoaxial angle [CXA] < 125°), and ventral brainstem compression (pB–C2 ≥ 9 mm). A multivariate Cox regression analysis was used to determine the independent association between occipitocervical fusion and each variable.
Of the 206 patients who underwent CM decompression with or without occipitocervical fusion during the study period, 101 had preoperative imaging available for review and formed the study population. Mean age at surgery was 9.1 years, and mean follow-up was 2.3 years. Eighty-two patients underwent suboccipital decompression alone (mean age 8.7 years). Nineteen patients underwent occipitocervical fusion (mean age 11.1 years), either as part of the initial surgical procedure or in a delayed fashion. Factors demonstrating a significantly increased risk of requiring fusion were basilar invagination (HR 9.8, 95% CI 2.2–44.2), CM 1.5 (HR 14.7, 95% CI 1.8–122.5), and CXA < 125° (HR 3.9, 95% CI 1.2–12.6).
Patients presenting with basilar invagination, CM 1.5, and CXA < 125° are at increased risk of requiring an occipitocervical fusion procedure either as an adjunct to initial surgical decompression or in a delayed fashion. Patients and their families should be counseled in regard to these findings as part of a preoperative CM evaluation.
Paul Klimo Jr., Valerie Coon, and Douglas Brockmeyer
Os odontoideum was first described in the late 1880s and still remains a mystery in many respects. The genesis of os odontoideum is thought to be prior bone injury to the odontoid, but a developmental cause probably also exists. The spectrum of presentation is striking and ranges from patients who are asymptomatic or have only neck pain to those with acute quadriplegia, chronic myelopathy, or even sudden death. By definition, the presence of an os odontoideum renders the C1–2 region unstable, even under physiological loads in some patients. The consequences of this instability are exemplified by numerous cases in the literature in which a patient with os odontoideum has suffered a spinal cord injury after minor trauma. Although there is little debate that patients with os odontoideum and clinical or radiographic evidence of neurological injury or spinal cord compression should undergo surgery, the dispute continues regarding the care of asymptomatic patients whose os odontoideum is discovered incidentally. The authors' clinical experience leads them to believe that certain subgroups of asymptomatic patients should be strongly considered for surgery. These subgroups include those who are young, have anatomy favorable for surgical intervention, and show evidence of instability on flexion-extension cervical spine x-rays. This recommendation is bolstered by the fact that surgical fusion of the C1–2 region has evolved greatly and can now be done with considerable safety and success. When atlantoaxial instrumentation is used, fusion rates for os odontoideum should approach 100%.
Jian Guan, Jay Riva-Cambrin, and Douglas L. Brockmeyer
Patients treated for Chiari I malformation (CM-I) with posterior fossa decompression (PFD) may occasionally and unpredictably develop postoperative hydrocephalus. The clinical risk factors predictive of this type of Chiari-related hydrocephalus (CRH) are unknown. The authors' objective was to evaluate their experience to identify risk factors that may predict which of these patients undergoing PFD will develop CRH after surgery.
The authors performed a retrospective clinical chart review of all patients who underwent PFD surgery and duraplasty for CM-I at the Primary Children's Hospital in Utah from June 1, 2005, through May 31, 2015. Patients were dichotomized based on the need for long-term CSF diversion after PFD. Analysis included both univariate and multivariable logistic regression analyses.
The authors identified 297 decompressive surgeries over the period of the study, 22 of which required long-term postoperative CSF diversion. On multivariable analysis, age < 6 years old (OR 3.342, 95% CI 1.282–8.713), higher intraoperative blood loss (OR 1.003, 95% CI 1.001–1.006), and the presence of a fourth ventricular web (OR 3.752, 95% CI 1.306–10.783) were significantly associated with the need for long-term CSF diversion after decompressive surgery.
Younger patients, those with extensive intraoperative blood loss, and those found during surgery to have a fourth ventricular web were at higher risk for the development of CRH. Clinicians should be alert to evidence of CRH in this patient population after PFD surgery.