Symptomatic Outcome after Bone-only Suboccipital Decompression in Adult Patients with Chiari Type I Malformations in the Absence of Hydromyelia or Hydrocephalus

2017 ◽  
Vol 78 (04) ◽  
pp. 344-349 ◽  
Author(s):  
Hazem Abuhusain ◽  
Manuri Gunawardena ◽  
Tyler Auschwitz ◽  
Charles Teo ◽  
Nicolas Koechlin
Keyword(s):  
Chiari Malformation ◽  
Length Of Hospital Stay ◽  
Adult Patients ◽  
Common Symptom ◽  
Type I ◽  
Tonsillar Herniation ◽  
Adult Type ◽  
Duration Of Symptoms ◽  
Suboccipital Decompression ◽  
Pain Symptoms

Background Type I Chiari malformation presents without an associated hydromyelia in 30 to 70% of cases, yet there is no agreement regarding the optimal surgical treatment for these patients. We review our experience for treating symptomatic adult type I Chiari malformation without hydromyelia using a suboccipital bone decompression of the hindbrain and no duraplasty in 12 adult patients. Outcome was measured according to the Chicago Chiari Outcome Scale (CCOS). Results Nine of 12 patients were female; average age at surgery was 34.4 years (range: 17–67 years). Average duration of symptoms prior to surgery was 9.6 years (2 months–29 years). The most common symptom was head and/or neck pain (11/12 patients). All patients additionally presented with at least one non-pain symptom. Mean degree of tonsillar herniation on magnetic resonance imaging was 6.8 mm (range: 5–12 mm) below McRae's line. Operative time was on average 68 minutes (range: 47–120 minutes). No surgical complications were noted in any patient. Length of hospital stay was 2 days (1 overnight) for all patients. Mean follow-up was 167 weeks (range: 13–378 weeks). CCOS for all patients on average was 14.50 (range: 12–16). Pain symptoms underwent improvement (7/11 [63.6%]) or complete resolution (4/11 [36.4%]) in all affected patients. A shorter duration of preoperative symptoms significantly correlated with a better CCOS (p = 0.03). Degree of tonsillar herniation had no significant effect on CCOS (p = 0.67). Of non-pain symptoms, paresthesias/dysesthesias and visual symptoms improved or resolved completely in all affected patients. No patient experienced a worsening of either pain or non-pain symptoms. Conclusion In the subset of adult patients with a type I Chiari malformation and no associated hydromyelia, a craniectomy without an additional opening of the dura may achieve good overall results according to the CCOS.

Intraoperative ultrasonography as a guide to patient selection for duraplasty after suboccipital decompression in children with Chiari malformation Type I

2008 ◽  
Vol 2 (1) ◽  
pp. 52-57 ◽  
Author(s):  
Matthew J. McGirt ◽  
Frank J. Attenello ◽  
Ghazala Datoo ◽  
Muraya Gathinji ◽  
April Atiba ◽  
...  
Keyword(s):  
Chiari Malformation ◽  
Intraoperative Ultrasonography ◽  
Type I ◽  
Tonsillar Herniation ◽  
Chiari Malformation Type I ◽  
Lower Cranial Nerve ◽  
Cerebrospinal Fluid Flow ◽  
Subarachnoid Spaces ◽  
Suboccipital Decompression ◽  
Symptom Recurrence

Object Indications for duraplasty in treatment of Chiari malformation Type I (CM-I) remain unclear. In the present study, the authors evaluate their surgical experience to determine whether intraoperative ultrasonography is effective in the selection of patients with CM-I who can be adequately treated with craniectomy alone without duraplasty. Methods The authors reviewed the records of 256 children who underwent first-time hindbrain decompression for CM-I. Craniectomy alone (without duraplasty) was performed when intraoperative ultrasonography suggested adequate decompression of the subarachnoid spaces ventral and dorsal to the tonsils after suboccipital craniectomy alone. Duraplasty was performed if intraoperative ultrasonography demonstrated persistent dural compression of the tonsils following craniectomy. Symptom recurrence as a function of time was compared between cases of duraplasty versus suboccipital decompression alone stratified by extent of tonsillar herniation. Results Duraplasty was performed in 140 patients (55%), and suboccipital decompression alone was performed in 116 patients (45%). Patients underwent follow-up for 29 ± 15 months. Symptoms included headache in 192 patients (75%) and lower cranial nerve and brainstem dysfunction in 68 (27%). In 38 patients (15%) there was tonsillar herniation rostral to the C-1 lamina, in 195 (76%) it extended between the C-1 and C-2 lamina, and in 23 patients (9%) there was herniation caudal to the lower border of the C-2 lamina. In children with tonsillar herniation caudal to C-1, ultrasonography-guided suboccipital decompression alone was associated with a 2-fold increase in the risk of symptom recurrence compared with those who also underwent duraplasty (p = 0.01). In children with tonsillar herniation rostral to C-1, outcome was equivalent between suboccipital decompression alone and duraplasty (p = 0.41). Conclusions In the setting of moderate-to-severe tonsillar CM-I, intraoperative ultrasonography demonstrating decompression of the subarachnoid spaces ventral and dorsal to the tonsils may not effectively select patients in whom bone decompression alone is sufficient. Duraplasty may be warranted in cases of tonsillar herniation that extends below the C-1 lamina regardless of intraoperative ultrasonography findings. More objective cerebrospinal fluid flow or volumetric measures may be needed intraoperatively to guide duraplasty in patients with more pronounced tonsillar herniation.

scholarly journals External validation of current prediction systems of improvement after decompression surgery in Chiari malformation type I patients: can we do better?

2020 ◽  
pp. 1-6 ◽  
Author(s):  
James Feghali ◽  
Yangyiran Xie ◽  
Yuxi Chen ◽  
Sean Li ◽  
Judy Huang
Keyword(s):  
Chiari Malformation ◽  
Receiver Operating Curve ◽  
Symptom Improvement ◽  
Type I ◽  
Chiari Malformation Type I ◽  
Meaningful Improvement ◽  
Point Score ◽  
Significant Difference ◽  
Suboccipital Decompression ◽  
Prediction Systems

OBJECTIVEThe 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.METHODSA 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.RESULTSThe 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).CONCLUSIONSPreviously 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.

Effect of symptomatic pseudomeningocele on improvement in pain, disability, and quality of life following suboccipital decompression for adult Chiari malformation Type I

2013 ◽  
Vol 119 (5) ◽  
pp. 1159-1165 ◽  
Author(s):  
Scott L. Parker ◽  
Saniya S. Godil ◽  
Scott L. Zuckerman ◽  
Stephen K. Mendenhall ◽  
Noel B. Tulipan ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Chiari Malformation ◽  
Type I ◽  
Complication Rates ◽  
Chiari Malformation Type I ◽  
Long Term Outcome ◽  
Pain Disability ◽  
Patient Reported ◽  
Suboccipital Decompression

Object Suboccipital decompression is a common procedure for patients with Chiari malformation Type I (CMI). Published studies have reported complication rates ranging from 3% to 40%, with pseudomeningocele being one of the most common complications. To date, there are no studies assessing the effect of this complication on long-term outcome. Therefore, the authors set out to assess the effect of symptomatic pseudomeningocele on patient outcomes following suboccipital decompression for CM-I. Methods The study comprised 50 adult patients with CM-I who underwent suboccipital craniectomy and C-1 laminectomy with or without duraplasty. Clinical presentation, radiological studies, operative variables, and complications were assessed for each case. Baseline and 1-year postoperative patient-reported outcomes were assessed to determine improvement in pain, disability, and quality of life. The extent of improvement was compared for patients with and without development of a postoperative symptomatic pseudomeningocele. Results A symptomatic pseudomeningocele developed postoperatively in 9 patients (18%). There was no difference with regard to clinical, radiological, or operative variables for patients with or without a postoperative pseudomeningocele. Patients without a pseudomeningocele had significant improvement in all 9 patient-reported outcome measures assessed. On the other hand, patients with pseudomeningocele only had significant improvement in headache (as measured on the Numeric Rating Scale) and headache-related disability (as measured on the Headache Disability Index) but no improvement in quality of life. Twenty-nine (71%) of 41 patients without a pseudomeningocele reported improvement in health status postoperatively compared with only 3 (33%) of 9 patients with a postoperative pseudomeningocele (p = 0.05). Conclusions Surgical management of CM-I in adults provides significant and sustained improvement in pain, disability, general health, and quality of life. Development of a postoperative symptomatic pseudomeningocele has lingering effects at 1 year, and it significantly diminishes the overall benefit of suboccipital decompression for CM-related symptoms. Further research is needed to accurately predict which patients may benefit from decompression alone without duraplasty.

Time to Resolution of Symptoms After Suboccipital Decompression with Duraplasty in Children with Chiari Malformation Type I

2018 ◽  
Vol 117 ◽  
pp. e544-e551 ◽  
Author(s):  
Eveline Teresa Hidalgo ◽  
Yosef Dastagirzada ◽  
Cordelia Orillac ◽  
Svetlana Kvint ◽  
Emily North ◽  
...  
Keyword(s):  
Chiari Malformation ◽  
Type I ◽  
Chiari Malformation Type I ◽  
Suboccipital Decompression

Suboccipital decompression for Chiari malformation–associated scoliosis: risk factors and time course of deformity progression

2008 ◽  
Vol 1 (6) ◽  
pp. 456-460 ◽  
Author(s):  
Frank J. Attenello ◽  
Matthew J. McGirt ◽  
April Atiba ◽  
Muraya Gathinji ◽  
Ghazala Datoo ◽  
...  
Keyword(s):  
Cobb Angle ◽  
Spinal Deformity ◽  
Chiari Malformation ◽  
Time Course ◽  
Thoracolumbar Junction ◽  
Type I ◽  
Chiari Malformation Type I ◽  
Severe Scoliosis ◽  
Suboccipital Decompression ◽  
Deformity Progression

Object Chiari malformation Type I (CM-I) is often associated with scoliosis. It remains unclear which subgroups of patients are most likely to experience progression of spinal deformity after cervicomedullary decompression. The authors' goal was to determine the time frame of curvature progression and assess which patient subgroups are at greatest risk for progression of spinal deformity after surgery. Methods The authors retrospectively reviewed the records of all pediatric patients with significant scoliosis in whom suboccipital decompression was performed to treat for CM-I during a 10-year period at a single academic institution. Clinical, radiological, and operative variables were assessed as independent factors for failure (worsening of scoliosis) by using a univariate regression analysis. Results Twenty-one children (mean age 9 ± 3 years; 4 male) underwent hindbrain decompression for CM-I–associated scoliosis and were followed for a mean of 39 months. All patients harbored a syrinx. Eight patients (38%) experienced improvement in scoliosis curvature, whereas 10 (48%) suffered a progression. Thoracolumbar junction scoliosis (p = 0.04) and failure of the syrinx to improve (p = 0.05) were associated with 5- and 4-fold respective increases in the likelihood of deformity progression. Each increasing degree of preoperative Cobb angle was associated with an 11% increase in the likelihood of scoliotic curve progression (p < 0.05). Conclusions Over one third of patients with CM-I–associated scoliosis will improve after cervicomedullary decompression alone. Cervicomedullary decompression is a good first-line option, particularly in children with concordant posterior fossa symptoms. Patients presenting with more severe scoliosis (increasing Cobb angle) or scoliosis that crosses the thoracolumbar junction may benefit from earlier orthopedic involvement and should be monitored regularly for curvature progression after cervicomedullary decompression. In cases in which there is a failure of the syrinx to show improvement after suboccipital decompression, the patients are also more likely to develop curvature progression.

scholarly journals Diffusion tensor imaging assessment of microstructural brainstem integrity in Chiari malformation Type I

2016 ◽  
Vol 125 (5) ◽  
pp. 1112-1119 ◽  
Author(s):  
Vibhor Krishna ◽  
Francesco Sammartino ◽  
Philip Yee ◽  
David Mikulis ◽  
Matthew Walker ◽  
...  
Keyword(s):  
Diffusion Tensor Imaging ◽  
Posterior Fossa ◽  
Chiari Malformation ◽  
Diffusion Tensor ◽  
Structural Parameters ◽  
Ventricular Volume ◽  
Neurological Dysfunction ◽  
Type I ◽  
Tonsillar Herniation ◽  
Chiari Malformation Type I

OBJECTIVE The diagnosis of Chiari malformation Type I (CM-I) is primarily based on the degree of cerebellar tonsillar herniation even though it does not always correlate with symptoms. Neurological dysfunction in CM-I presumably results from brainstem compression. With the premise that conventional MRI does not reveal brain microstructural changes, this study examined both structural and microstructural neuroimaging metrics to distinguish patients with CM-I from age- and sex-matched healthy control subjects. METHODS Eight patients with CM-I and 16 controls were analyzed. Image postprocessing involved coregistration of anatomical T1-weighted with diffusion tensor images using 3D Slicer software. The structural parameters included volumes of the posterior fossa, fourth ventricle, and tentorial angle. Fractional anisotropy (FA) was calculated separately in the anterior and posterior compartments of the lower brainstem. RESULTS The mean age of patients in the CM-I cohort was 42.6 ± 10.4 years with mean tonsillar herniation of 12 mm (SD 0.7 mm). There were no significant differences in the posterior fossa volume (p = 0.06) or fourth ventricular volume between the 2 groups (p = 0.11). However, the FA in the anterior brainstem compartment was significantly higher in patients with CM-I preoperatively (p = 0.001). The FA values normalized after Chiari decompression except for persistently elevated FA in the posterior brainstem compartment in patients with CM-I and syrinx. CONCLUSIONS In this case-control study, microstructural alterations appear to be reliably associated with the diagnosis of CM-I, with a significantly elevated FA in the lower brainstem in patients with CM-I compared with controls. More importantly, the FA values normalized after decompressive surgery. These findings should be validated in future studies to determine the significance of diffusion tensor imaging–based assessment of brainstem microstructural integrity as an adjunct to the clinical assessment in patients with CM-I.

scholarly journals Outcomes after suboccipital decompression without dural opening in children with Chiari malformation Type I

2015 ◽  
Vol 16 (2) ◽  
pp. 150-158 ◽  
Author(s):  
Benjamin C. Kennedy ◽  
Kathleen M. Kelly ◽  
Michelle Q. Phan ◽  
Samuel S. Bruce ◽  
Michael M. McDowell ◽  
...  
Keyword(s):  
Risk Factors ◽  
Chiari Malformation ◽  
Multivariate Regression ◽  
Type I ◽  
Tonsillar Herniation ◽  
Chiari Malformation Type I ◽  
Radiographic Findings ◽  
Major Complications ◽  
Risk Factors For Recurrence ◽  
Dural Opening

OBJECT Symptomatic pediatric Chiari malformation Type I (CM-I) is most often treated with posterior fossa decompression (PFD), but controversy exists over whether the dura needs to be opened during PFD. While dural opening as a part of PFD has been suggested to result in a higher rate of resolution of CM symptoms, it has also been shown to lead to more frequent complications. In this paper, the authors present the largest reported series of outcomes after PFD without dural opening surgery, as well as identify risk factors for recurrence. METHODS The authors performed a retrospective review of 156 consecutive pediatric patients in whom the senior authors performed PFD without dural opening from 2003 to 2013. Patient demographics, clinical symptoms and signs, radiographic findings, intraoperative ultrasound results, and neuromonitoring findings were reviewed. Univariate and multivariate regression analyses were performed to determine risk factors for recurrence of symptoms and the need for reoperation. RESULTS Over 90% of patients had a good clinical outcome, with improvement or resolution of their symptoms at last follow-up (mean 32 months). There were no major complications. The mean length of hospital stay was 2.0 days. In a multivariate regression model, partial C-2 laminectomy was an independent risk factor associated with reoperation (p = 0.037). Motor weakness on presentation was also associated with reoperation but only with trend-level significance (p = 0.075). No patient with < 8 mm of tonsillar herniation required reoperation. CONCLUSIONS The vast majority (> 90%) of children with symptomatic CM-I will have improvement or resolution of symptoms after a PFD without dural opening. A non–dural opening approach avoids major complications. While no patient with tonsillar herniation < 8 mm required reoperation, children with tonsillar herniation at or below C-2 have a higher risk for failure when this approach is used.

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