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James Feghali, Yangyiran Xie, Yuxi Chen, Sean Li and Judy Huang

OBJECTIVE

The Chiari Severity Index (CSI) and points-based algorithm of Thakar et al. are two prognostic tools that have been developed to predict the likelihood of improvement after suboccipital decompression in adult patients with Chiari malformation type I (CM-I). This study aimed to externally validate and critically evaluate these algorithms in the interest of guiding the development of improved prediction systems.

METHODS

A consecutive cohort of CM-I patients undergoing suboccipital decompression between September 2006 and September 2018 were included. The CSI and Thakar point score were computed for all patients, and associations with improvement were analyzed. The ability of both prediction systems to predict improvement as measured by different Chicago Chiari Outcome Scale (CCOS) cutoffs was assessed using receiver operating curve analysis. Post hoc correlations between the algorithms and different CCOS subcomponents were also assessed.

RESULTS

The surgical cohort was composed of 149 adult CM-I patients, of whom 39 (26%) had a syrinx. Most patients experienced improvement after surgery (80% CCOS ≥ 13; 96% CCOS ≥ 11). The proportion of patients improving decreased with increasing CSI, but the results were not statistically significant (p = 0.246). No statistically significant difference in the mean Thakar point score was identified between improved and nonimproved patients using both CCOS cutoffs (p = 0.246 for a cutoff of 13 and p = 0.480 for a cutoff of 11). The CSI had a poor ability in identifying improved patients at a CCOS cutoff of 13 (area under the curve [AUC] 0.582) and 11 (AUC 0.646). The Thakar point score similarly had poor discrimination at a cutoff of 13 (AUC 0.467) and 11 (AUC 0.646). Neither algorithm had significant correlation with any of the CCOS subcomponents except for CSI and nonpain symptom improvement (coefficient = −0.273, p = 0.001).

CONCLUSIONS

Previously published algorithms failed to provide prediction value with regard to clinically meaningful improvement following suboccipital decompression in adult CM-I patients. Future models and practical scoring systems are still required to improve the decision-making process.

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Luyuan Li, John N. Jensen, Sara Szabo, Peter VanTuinen and Sean M. Lew

Desmoid tumors, also known as aggressive fibromatosis, are locally infiltrating musculoaponeurotic neoplasms arising in connective tissues. Desmoid tumors may be associated with familial adenomatous polyposis (FAP), a genetic disorder that presents with hundreds to thousands of precancerous colorectal polyps. The authors report the case of an 18-month-old boy who underwent resection of a right temporal desmoid tumor (initially diagnosed as cranial fasciitis) and developed a bilateral frontoparietal calvarial desmoid tumor 2 years later. The patient underwent gross-total resection of the tumor that required a large cranioplasty. He was subsequently diagnosed with FAP. The patient has been without tumor recurrence for 9 years afterwards and has not required revision of his cranioplasty. This is the first report describing a recurrent cranial desmoid tumor in a pediatric patient with FAP. The authors believe, however, that some of the cases previously reported as cranial fasciitis are likely desmoid tumors pathobiologically and genetically.

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Joshua L. Golubovsky, Hong Li, Arbaz Momin, Jianning Shao, Maxwell Y. Lee, Leonardo A. Frizon, Olivia Hogue, Benjamin Walter, André G. Machado and Sean J. Nagel

OBJECTIVE

Parkinson’s disease (PD) is a progressive neurological movement disorder that is commonly treated with deep brain stimulation (DBS) surgery in advanced stages. The purpose of this study was to investigate factors that affect time to placement of a second-sided DBS lead for PD when a unilateral lead is initially placed for asymmetrical presentation. The decision whether to initially perform unilateral or bilateral DBS is largely based on physician and/or patient preference.

METHODS

This study was a retrospective cohort analysis of patients with PD undergoing initial unilateral DBS for asymmetrical disease between January 1999 and December 2017 at the authors’ institution. Patients treated with DBS for essential tremor or other conditions were excluded. Variables collected included demographics at surgery, time since diagnosis, Unified Parkinson’s Disease Rating Scale motor scores (UPDRS-III), patient-reported quality-of-life outcomes, side of operation, DBS target, intraoperative complications, and date of follow-up. Paired t-tests were used to assess mean changes in UPDRS-III. Cox proportional hazards analysis and the Kaplan-Meier method were used to determine factors associated with time to second lead insertion over 5 years.

RESULTS

The final cohort included 105 patients who underwent initial unilateral DBS for asymmetrical PD; 59% of patients had a second-sided lead placed within 5 years with a median time of 34 months. Factors found to be significantly associated with early second-sided DBS included patient age 65 years or younger, globus pallidus internus (GPi) target, and greater off-medication reduction in UPDRS-III score following initial surgery. Older age was also found to be associated with a smaller preoperative UPDRS-III levodopa responsiveness score and with a smaller preoperative to postoperative medication-off UPDRS-III change.

CONCLUSIONS

Younger patients, those undergoing GPi-targeted unilateral DBS, and patients who responded better to the initial DBS were more likely to undergo early second-sided lead placement. Therefore, these patients, and patients who are more responsive to medication preoperatively (as a proxy for DBS responsiveness), may benefit from consideration of initial bilateral DBS.

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Oral Presentations

2010 AANS Annual Meeting Philadelphia, Pennsylvania May 1–5, 2010