Operative findings and surgical outcomes in patients undergoing Chiari 1 malformation decompression: relationship to the extent of tonsillar ectopia

Acta Neurochirurgica (2020) 162:1539–1547

The diagnosis of Chiari 1 malformation is based on the extent of tonsillar ectopia.

Objective To examine the relationship between the extent of tonsillar ectopia and the intra-operative findings and clinical outcome following Chiari decompression surgery.

Methods Patients were divided into four groups depending on the position of the cerebellar tonsil (T): group 1: 0 < T < 3; group 2: 3 ≤ T ≤ 5; group 3: 5 < T ≤ 10; and group 4: T > 10. Intra-operative observations were recorded with regard to compression of the brain stem by posterior inferior cerebellar artery (pica), neuroma formation along the first cervical (C1), and accessory spinal nerves (XI), and pallor of the cerebellar tonsils. Brain stem auditory evoked potentials, (BAEP), were monitored in each case. One hundred sixty-eight patients accrued between 2009 and 2013 agreed to participate in an outcome study to determine the effectiveness of foramen magnum decompression. Findings across the four groups were compared using one-way ANOVA. Observed differences were further subjected to paired analysis. Intra-group comparisons were made using the paired t test. A P value less than 0.05 was considered statistically significant.

Results There were 98 patients in group 1, 147 patients in group 2, 180 patients in group 3, and 63 patients in group 4. The mean extent of tonsillar ectopia was 0.4, 4.0, 7.1, and 14.3 mm in the four groups respectively. The prevalence of tonsillar pallor was greatest in group 4. Otherwise, there was no difference observed in the operative findings. A reduction of > 0.1 msec in the wave III–wave V latency of the BAEP was noted in all four groups with equal frequency. One hundred ten patients complied with at least 6 months follow-up. There was no difference in the prevalence of symptoms between the four groups at the time of initial evaluation and at 6 weeks and 6 months following surgery. There was a statistically significant reduction in the intensity of individual symptoms 6 months following surgery regardless of the extent of tonsil ectopia.

Conclusion Other than the finding of tonsillar pallor, there was no relationship between the extent of tonsillar ectopia and the intraoperative anatomical and physiological observations, nor was there any relationship to the likelihood of symptomatic improvement following surgery. These observations call into question the focus on the extent of tonsillar of ectopia in assessing the patient who presents with symptoms of the Chiari malformation.

The value of multimodality intraoperative neurophysiological monitoring in treating pediatric Chiari malformation type I


Acta Neurochir (2016) 158:335–340

Chiari malformation type I is defined as a descent of cerebellar tonsils below the level of the foramen magnum. The traditional treatment for symptomatic patients is foramen magnum decompression (FMD) surgery. Intraoperative neurophysiological monitoring (INM) is an established surgical adjunct, which is proposed to reduce the potential risk of various surgical procedures. Though INM has been suggested as being helpful in patient positioning and in determining the optimal surgical extent of FMD (i.e., duroplasty, laminectomy, tonsillectomy), its shortcomings include prolongation of anesthesia and surgery as well as monetary costs. Multimodality INM including transcranial-electric motor evoked potential (TcMEP) is not routinely employed in most practices. This study evaluates efficacy of multimodality INM during FMD.

Methods This work is a retrospective analysis of prospectively collected data. Twenty-two FMD surgeries in 21 pediatric patients (aged 1–18 years) were performed at our center utilizing multimodality INM. All patients presented Chiari malformation type I, 18 of which had presented with syringomyelia, underwent posterior fossa decompression (FMD+ C1 laminectomy), accompanied in some with additional cervical laminectomies, duroplasty, and partial tonsillectomies. TcMEP and somatosensory evoked potentials (SSEP) were monitored throughout the procedure including before and after positioning. INM alarms were correlated with perioperative and long-term patient outcomes.

Results INM data remained stable during 19 operations. Three cases displayed significant attenuation in the monitoring signals, all concomitant with patient positioning on the surgical table. One case showed attenuation in SSEP data only, which remained attenuated following repositioning. Another displayed altered TcMEP concomitant with positioning which partially stabilized following repositioning and resolved following bony decompression. The third case showed unilateral attenuation of both TcMEP and SSEP data, which did not rectify until closure. In each of these three cases, no new neurological deficits were observed post operatively.

Conclusions Multimodality INM can be useful in FMD surgery, particularly during patient positioning. TcMEP attenuations may occur independent of SSEPs. The clinical implications of these monitoring alerts have yet to be defined. There is a need to establish an optimal, cost-effective monitoring protocol for FMD.