Individualized surgical treatment of Chiari 1 malformation: A single-center experience.

OBJECTIVE: To present our experience with an individualized surgical approach to treat Chiari malformation type 1. METHODS: Based on (1) neurological symptoms, (2) the existence and extent of a syrinx and (3) the degree of the tonsillar descent we performed four types of approaches on a case-by-case basis in 81 patients: (1) foramen magnum decompression (FMD) with dura splitting (FMDds); (2) FMD with duraplasty (FMDdp); (3) FMD with duraplasty and tonsillar manipulation (FMDao); and (4) tonsillar resection/reduction (TR). Patient characteristics, Chiari Severity Index (CSI), fourth ventricular roof angle (FVRA) and Chicago Chiari Outcome Scale (CCOS) were analyzed. RESULTS: CCOS was between 13 and 16 points in 8/11 (73 %) patients after FMDds, 38/45 (84 %) patients after FMDdp, and 24/24 (100 %, one patient lost to follow-up) patients after TR. We experienced an overall complication rate of 13.6 % (11/81) in this series, whereas seven of these eleven complications (64 %) occurred in the FMDao group and the complication rate increased with the invasiveness of the approach (0 % FMDds; 4 % FMDdp; 12 % TR). CONCLUSION: Given the clear correlation between the extend of the approach and the complication rate the least invasive approach necessary to achieve clinical improvement should be selected. Due to the high complication rates, FMDao should not be used as a treatment option. The severity of the tonsillar descent, basilar invagination and current CM1 scores could be used to aid in the approach selection.

Cerebellar tonsil reduction for surgical treatment of Chiari malformation type I in children.

OBJECTIVE: The goal of this study was to review the efficacy and safety of different surgical techniques used for treatment of Chiari malformation type I (CM-I) in children. METHODS: The authors retrospectively reviewed 437 consecutive children surgically treated for CM-I. Procedures were classified into four groups: bone decompression (posterior fossa decompression [PFD]) and duraplasty (PFD with duraplasty [PFDD]), PFDD with arachnoid dissection (PFDD+AD), PFDD with tonsil coagulation of at least one cerebellar tonsil (PFDD+TC), and PFDD with subpial tonsil resection of at least one tonsil (PFDD+TR). Efficacy was measured as a greater than 50% reduction in the syrinx by length or anteroposterior width, patient-reported improvement in symptoms, and rate of reoperation. Safety was measured as the rate of postoperative complications. RESULTS: The mean patient age was 8.4 years (range 3 months to 18 years). In total, 221 (50.6%) patients had syringomyelia. The mean follow-up was 31.1 months (range 3-199 months), and there was no statistically significant difference between groups (p = 0.474). Preoperatively, univariate analysis showed that non-Chiari headache, hydrocephalus, tonsil length, and distance from the opisthion to brainstem were associated with the surgical technique used. Multivariate analysis demonstrated that hydrocephalus was independently associated with PFD+AD (p = 0.028), tonsil length was independently associated with PFD+TC (p = 0.001) and PFD+TR (p = 0.044), and non-Chiari headache was inversely associated with PFD+TR (p = 0.001). In the treatment groups postoperatively, symptoms improved in 57/69 (82.6%) PFDD patients, 20/21 (95.2%) PFDD+AD patients, 79/90 (87.8%) PFDD+TC patients, and 231/257 (89.9%) PFDD+TR patients, and differences between groups were not statistically significant. Similarly, there was no statistically significant difference in postoperative Chicago Chiari Outcome Scale scores between groups (p = 0.174). Syringomyelia improved in 79.8% of PFDD+TC/TR patients versus only 58.7% of PFDD+AD patients (p = 0.003). PFDD+TC/TR remained independently associated with improved syrinx outcomes (p = 0.005) after controlling for which surgeon performed the operation. For those patients whose syrinx did not resolve, no statistically significant differences between surgery groups were observed in the length of follow-up or time to reoperation. Overall, there was no statistically significant difference between groups in postoperative complication rates, including aseptic meningitis and CSF- and wound-related issues, or reoperation rates. CONCLUSIONS: In this single-center retrospective series, cerebellar tonsil reduction, by either coagulation or subpial resection, resulted in superior reduction of syringomyelia in pediatric CM-I patients, without increased complications.

Outcomes in children undergoing posterior fossa decompression and duraplasty with and without tonsillar reduction for Chiari malformation type I and syringomyelia: a pilot prospective multicenter cohort study.

OBJECTIVE: Despite significant advances in diagnostic and surgical techniques, the surgical management of Chiari malformation type I (CM-I) with associated syringomyelia remains controversial, and the type of surgery performed is surgeon dependent. This study's goal was to determine the feasibility of a prospective, multicenter, cohort study for CM-I/syringomyelia patients and to provide pilot data that compare posterior fossa decompression and duraplasty (PFDD) with and without tonsillar reduction. METHODS: Participating centers prospectively enrolled children suffering from both CM-I and syringomyelia who were scheduled to undergo surgical decompression. Clinical data were entered into a database preoperatively and at 1-2 weeks, 3-6 months, and 1 year postoperatively. MR images were evaluated by 3 independent, blinded teams of neurosurgeons and neuroradiologists. The primary endpoint was improvement or resolution of the syrinx. RESULTS: Eight clinical sites were chosen based on the results of a published questionnaire intended to remove geographic and surgeon bias. Data from 68 patients were analyzed after exclusions, and complete clinical and imaging records were obtained for 55 and 58 individuals, respectively. There was strong agreement among the 3 radiology teams, and there was no difference in patient demographics among sites, surgeons, or surgery types. Tonsillar reduction was not associated with > 50% syrinx improvement (RR = 1.22, p = 0.39) or any syrinx improvement (RR = 1.00, p = 0.99). There were no surgical complications. CONCLUSIONS: This study demonstrated the feasibility of a prospective, multicenter surgical trial in CM-I/syringomyelia and provides pilot data indicating no discernible difference in 1-year outcomes between PFDD with and without tonsillar reduction, with power calculations for larger future studies. In addition, the study revealed important technical factors to consider when setting up future trials. The long-term sequelae of tonsillar reduction have not been addressed and would be an important consideration in future investigations.

Posterior reversible encephalopathy syndrome in a patient with a Chiari I malformation.

BACKGROUND: The authors describe a unique case of a patient who developed posterior reversible encephalopathy syndrome (PRES) following postoperative treatment of a Chiari I malformation. CASE DECSRIPTION: A 25-year-old female presented with complaints of left upper and lower extremity paresthesias and gait disturbances. A magnetic resonance imaging (MRI) of the brain and cervical spine showed a Chiari I malformation with tonsillar descent beyond the level of the C1 lamina. She underwent a suboccipital craniectomy and C1 laminectomy with cerebellar tonsillar cauterization and duraplasty. Postoperatively, an MRI showed bilateral acute infarcts of the cerebellar vermis. She was initially treated for cerebellar ischemia with hypertensive therapy with a subsequent decline in her neurologic status and generalized tonic-clonic seizure. Further workup showed evidence of PRES. After weaning pressors, the patient had a significant progressive improvement in her mental status. CONCLUSION: Although the mechanism of PRES remains controversial given its diverse clinical presentation, several theories implicate hypertension and steroid use as causative agents.